Part 2

Hematology, Cardiology, Rheumatology & Emergencies

 ‫بسم هللا الرحمن الرحيم‬

University of Khartoum
Faculty of Medicine
Awasir Batch (91)

Academic Secretary

❖ This is a complementary part to the 2nd Edition of MOUSAB NOTES OF INTERNAL

MEDICINE (2020).
❖ The content of this note is text transcription from audio recordings of Dr Mousab
Mohammed Ibrahim (Kibreet) Batch 88 Qasawir Reviwes of Internal Medicine for
Batch 91 Awasir (Recorded in June 2021) at Faculty of Medicine, University of
Khartoum.
❖ This edition is designed as a hard copy; a soft copy will be available in the Telegram
channel https://t.me/Kibreet which also contains all Dr.Mousab Ibrahim (Kibreet)
audio records.

Participants in this work are members of Awasir Batch (91):

Writing Team:

▪ Hanaa Abdulrahman Fadil

▪ Hadeel Mohammed Moaid
▪ Twasol Adam Omer Ahmed
▪ Eman Mohamed Abdallah Ali
▪ Alaa Tajeldeen Habeeb
▪ Shiraz Faisal Mohamed Bushra

Final Layout & Coordination:

▪ Hajer Ismat Alameen

▪ Hanaa Abdulrahman Fadil

i|Page
 ‫تنبيهات مهمة‪:‬‬

‫❖ هذا الشيت هو تفريغ لمراجعات د‪ .‬مصعب محمد إبراهيم‪ ،‬كتبه مجموعة من خريجي‬
‫الدفعة اواسر‪ -‬طب الخرطوم‪ -‬ال تنسوهم من صالح دعائكم‬
‫❖ في حالة مالحظة أي خطأ في الطباعة او المعلومات الرجاء التواصل معنا على هذا البريد‬
‫االلكتروني‪[email protected] :‬‬
‫❖ بأي حال ال يعتبر هذا الشيت بديل عن المراجع األساسية للمادة والمحاضرات‬
‫❖ الشكر موصول أيضا لكل من ساهم في مراجعته من طالب الدفعة ‪ )91‬اواسر)‪:‬‬
‫‪ -‬روان عبد الواحد عبد المنعم‬
‫‪ -‬محمد عبد الحفيظ صالح‬
‫‪ -‬سجى صالح محمد‬
‫‪ -‬نشوى عوض التوم‬
‫‪ -‬رزاز مهدي‬
‫❖ وطالب الدفعة ‪( 92‬بيارق)‪:‬‬
‫‪ -‬فاتن محمد‬
‫‪ -‬آيات ابراهيم‬

‫‪ii | P a g e‬‬
Table of Contents

Hematology ..................................................................................... 1
Cardiology .................................................................................... 31
Rheumatology ............................................................................... 42
NOTES in Emergencies ............................................................... 54

iii | P a g e
 Hematology

RBCs Disorders

Anemia: Hb <13.5 in males, < 11 or 12.5 in females

▪ Symptoms: fatigability, dyspnea, palpitations
▪ Sign: pallor
▪ Precipitates Acute coronary syndrome “ACS”.

• Microcytic anemia: IDA, Thalassemia, Sideroblastic

MCV<80

• Normocytic anemia: Hemolytic anemia, Anemia of chronic

MCV 80-100:
diseases (CKD)

• Macrocytic anemia
MCV>100 • Megaloblastic anemia

MCV>100 indicates:
1. Macrocytic anemia: Macrocytic & hyper-segmented neutrophils more than 5
lobes in blood film but no megaloblasts in bone marrow
- Example: Liver disease, Hypothyroidism, pregnancy, alcohol, cytotoxic drugs
2. Megaloblastic anemia: [Macrocytic & hyper-segmented neutrophils more than 5
lobes in blood film + megaloblasts in bone marrow
- Example: B12 deficiency, folic acid deficiency.

1|Page
 Microcytic Anemia

1. Iron deficiency anemia (IDA):

- Specific sign: Angular stomatitis, spooning nails.

Causes:
✓ Blood loss (GI or MC).
✓ Increase demand with pregnancy, infancy, prematurity.
✓ Infection (hook worms: ankylestoma)
The most common form of anemia in pregnancy is IDA.
IDA:
Iron studies:
▪ serum iron: decreased
▪ ferritin: decreased
▪ Transferrin: decreased
▪ TIBC: increased
▪ RDW: increased in IDA. (RBCs have different volumes).

Investigations for anemia:

- CBC, MCV, MCH, MCHC, PBP
- Iron studies:
Serum iron, serum ferritin, transferrin saturation, TIBC
• RDW

MCQs:
✓ Young female young in reproductive age with IDA: treat empirically
✓ Male OR postmenopausal female with unexplained IDA: need investigation by
upper and lower GI endoscopy to exclude cancer
Management: Iron supplement (Oral / IM / IV) same efficacy” IM: not usually used”
- Oral, feruss sulphate, fumerate, gluconate
- IV: Dextran / Sucrose

2|Page
IV iron indications:
✓ Intolerance to oral form because it causes, nausea vomiting diarrhea and
constipation
✓ Malabsorption i.e celiac disease
✓ Dialysis patient’s problem in mobilization of iron from GI to tissue
✓ anaphylaxis
Iron absorption need acid, in duodenum.
- PPI, tea (tannin) and tetracycline decrease absorption.
- Vitamin C & Cupper increase absorption.
Plummer Vinson syndrome (Paterson Kelly):
- Female dysphagia (E.web) + IDA + Koilonychia or glossitits.
N.B: Microcytic Anemia not treated by iron supplements: think of thalassemia.

2. Thalassemia:

Hemoglobin has two alpha & two beta chains

• Defect in alpha chain of HbA:
Alpha thalassemia: 4 genes, more in Asian.
1. One gene defect: Silent disease.
2. Two genes defect: mild Anemia like beta thalassemia minor.
3. Three genes defect: Hb H sever transfusion dependent Anemia like beta
thalassemia major.
4. Four genes defect: hydrops fetal is die of HF.
• Defect in Beta chain of HbA:
- Has 2 genes
- more in African.
1. One gene defect causes beta thalassemia minor: mild microcytic anemia.
- Decreased Hb, decreased MCV 60-55 (very low unlike IDA), increase Hb A2
and Hb F in Hb electrophoresis.

3|Page
 2. Two gene defect results in beta thalassemia major: sever transfusion dependent
Anemia.
Clinical features:
- Features appear after 6 months old, when Hb F decreases.
- BM expansion (hair on end skull x ray):Thalassemic face - Frontal bossing
(OSPE)
- Extra-medullary hemopoisis: Hepato-splenomegaly. In peripheral blood picture
you will find target cells.

MINTZER Index to differentiate between thalassemia & IDA.

MINTZER Index= MCV/ RBC count
If > 13: IDA
If < 13: thalassemia

Management of Beta thalassemia major:

- Blood transfusion, folic acid supplements, Iron chelator oral: disfruxamin or IV
difrasuruox.
- Definite management is bone marrow transplantation.

Cause of death is iron over load

4|Page
 3. Sideroblastic anemia:

- Ring Sideroblasts in bone marrow.

Causes:
✓ Idiopathic
✓ Lead poising
✓ Drugs: (isoniazid; also causes B6 deficiency)

*************************************************************

5|Page
 Macrocytic Anemia

Macrocytic Anemia (megaloblastic)

1. B12 deficiency anemia:

B12 deficiency: dietary B12 absorption needs intrinsic factor. Absorption occurs in
ileum.
Causes:
1) Strict vegans (years), unlike folic acid
2) Decrease IF: decrease in pernicious anemia (Ab attack partial cell- MCC)
3) Gastrostomy: decrease acid
4) Ileal disease i.e. Crohn’s disease,
Celiac disease: usually affects duodenum >>IDA, need duodenal biopsy for diagnosis
(villous atrophy)
5) Infection: D.latum (fish tape warm)
Clinical Presentation:
Anemia, peripheral neuropathy, optic atrophy, SACDC, reversible dementia, atrophic
glossitis or inflamed tongue
Anemia + neurological manifestation

Investigations:
- CBC: decrease Hb, increase MCV
- PBP: Macro-ovalocyte - hyper segmented neutrophils
- B12 level: decrease
- Increase in LDH, pancytopenia
- BM biopsy: megaloblasts

6|Page
 - MMA level methyl malonic acid increased in B12 deficiency only (specific)
- Homocysteine in both B12+ folic
- Schilling test: give IV B12 to saturate all receptors then give radioactive oral B12
Then measure in urine
✓ If dietary: it appears normally in urine >10% in urine
✓ If not in urine: malabsorption, pernicious anemia, D.latum
So give intrinsic factor with it to differentiate
✓ If it appears in urine this is pernicious anemia
If not in urine: malabsorption
- If Pernicious anemia: measure antibodies; anti paritial cell (Sensitive), anti-
intrinsic factor Ab (Specific)
Case scenario for pernicious anemia: Young female with other autoimmune diseases:
hashimoto thyroiditis / premature ovarian failure / Vitiligo.
Management: IM B12 or Hydroxycobalornine

2. Folic acid deficiency anemia:

Causes:
✓ Dietary: alcoholic (alcohol interfere with folic acid absorption).
✓ Drugs (anti-folate: methotrexate, phenytoin, trimethoprim)
✓ Increase demand pregnancy.
Second most common cause of anemia in pregnancy is folic acid deficiency.
- NO neurological symptoms
- NO increase in MMA / but increase in homocysteine
Investigations same as for B12:
- CBC: decreased Hb, increase MCV.
- PBP Macro-ovalocyte - hyper segmented neutrophils
- BM biopsy shows megaloblasts.
- Folic acid: low
Management: folic acid supplement.

7|Page
 - If combined B12 + folic acid deficiency treat B 12 first. If you treat folic acid
deficiency anemia first, this will increase the substrates and consumes B 12
precipitating neurological symptoms (MCQ).

3. Orotic aciduria:

MCQ in pediatric
- Increase orotic acid DDx
1. Orotic aciduria:
- Increase orotic acid + megaloblastic anemia
2. Urea cycle defect “Ornithine transcarbomylase (OTC) deficiency”:
- Increase orotic acid + Encephalopathy.
- Increase ammonia +Decrease urea (BUN)
Notes in metabolic disorders:
❖ Baby on breast feed hepatomegaly, Jaundice, cataract: Classic glactocemia.
❖ If symptoms appear after eating fruit, honey: Fructose intolerance.

Macrocytic (non megaloblastic anemia)

1. Diamond black fan anemia.

2. Drugs: cytotoxic (azathioprine), NRTI (Zidovudine), Hydroxuria.

Diamond black fan anemia:

- Macrocytic non megaloblastic anemia in infant.

- Anemia with increase MCV + triphalyngeal thumb.

*************************************************************

8|Page
 Normocytic Anemia

1. Anemia of chronic disease:

Pathogenesis: long lasting inflammation result in increase of hepcidin, which in turn traps
iron inside the cells (macrophages, liver cells ...) making it unavailable for use.

Iron studies in this case:

- Iron: Low
- Ferritin: Increased
- Total iron binding capacity: Decreased
- Transferring saturation: Normal

2. Aplastic anemia:

- Characterized by hypocellular fatty bone marrow.

Pic. Shows Bone marrow biopsy of aplitic anemia

▪ MCC of inherited aplastic anemia is

Fanconi anemia.

Fanconi anemia is autosomal recessive; disorder in

DNA repair.

Case scenario: pancytopenia, skin problems

(café au lait spots), skeletal deformities (short stature), thump deformities
(hypoplsatic or absent).

9|Page
Other causes of aplastic anemia:

Drug: chloramphenicol. Chemicals: benzene. Infections: HIV, hepatitis, EBV and parvo
B19 virus.

Café au lait presents in:

• Fanconi anemia.
• Neurofibromatosis.
• McCune Albright syndrome (café au lait, precocious puberty and polyostotic
fibrous dysplasia-bon eproblem- ).

Note: aplastic anemia increase risk of malignancy esp. Leukemia.

*************************************************************

10 | P a g e
 Hemolytic Anemia

- Markers of hemolytic anemia: Increase reticulocytes, increase LDH,

increase bilirubin and increase urobilinogen in urine (indicate hemolytic jaundice),
normocytic anemia (MCV8 0-100)
- Types of hemolytic: Intravascular and Extra vascular hemolysis.
1. Intravascular hemolysis: G6DP deficiency, cold autoimmune hemolytic
anemia, paroxysmal nocturnal hemoglobin urea and pyruvate kinase deficiency.
- Markers of intravascular hemolysis are: hemoglobinurea,
hemosiderinurea hemoglobenemia and decreased haptoglobin.
2. Extravscular hemolysis: occurs in spleen.

N.B: Hemolytic anemia can turn into macrocytic anemia if there is large increase in retics
(reticulosytosis).

1. Hereditary spherocytosis (HS):

- Autosomal dominant defect in RBC membrane proteins.

Case scenario: hemolytic anemia+ jaundice+ splenomegaly + family history.

Diagnosis:

1. CBC: low hemoglobin, normal MCV, increased MCHC.

2. Peripheral blood picture: spherocytes (spherical full of hemoglobin)
3. Positive osmotic fragility test (no longer used).
4. Flowcytometry: eosin- 5- maleimide assay (EMA) binding test

HS is positive if the EMA is abnormal.

Management: Folic acid supplement and splenectomy.

11 | P a g e
 Notes:
ddx of spherocytes in blood:

1. hereditary spherocytosis
2. autoimmune hemolytic anemia.

2. G6PD deficiency:

- Inherited as X-linked recessive mainly in males. In females if turner or extreme

lionization (random inactivation of X chromosomes).

Case scenario: child exposed to triggers then presented with dark urine
(hemoglobinurea).

- Triggers: infections, drugs: anti-malarial, high dose of aspirin, sulpha drugs,

dapsone (anti UTI), stress and Fava beans.

Diagnosis:

- Peripheral blood smear: Heinz bodies, bite cells.

- Heinz bodies are oxidized hemoglobin.
- G6dp produces NADPH which is needed for reduction of antioxidant.
- In G6PD deficiency there is no enough NADPH, oxidation occurs and hemoglobin
is oxidized then precipitated as Heinz bodies, RBCs contain Heinz bodies go
through the spleen or blood vessels.
- Measurement of G6PD level should be 4 weeks after the attack (will be low)
because of new retic shave normal G6PD.

12 | P a g e
 Heinz bodies:

Bite cells:

3. Sickle cell anemia:

- Autosomal recessive, defect in beta chain position 6 glutamic acid (-ve charge) is
replaced by valine (neutral).

In electrophoresis:

- HB S is slower than HB A.
- HB A is +ve closer to the anode.
- Sickle cells trait: one gene is affected, normal HB, normal MCV and no crisis, but
they present with kidney manifestations:
1. hypothenurea or isothenurea: decrease concentrating ability of urine with low
specific gravity.

13 | P a g e
 2. Hematuria (papillary necrosis)
3. Increase UTI risk

Sickle cell disease:

Crisis:

1. Vaso-occlusive crisis:
a) Dactylitis (hand and foot syndrome)→ painful crisis.
b) Acute chest syndrome: MCC of death in adults with SC disease.
Case scenario: adult known SCA present with fever, chest pain and dyspnea with
new infiltrate in chest x ray bilaterally.
c) Avascular necrosis (AVN) of the femur.
d) CVA: stroke is the MCC of death in children. 2nd MCC of death is infections after
splenectomy.
e) Priapism: painful sustained erection.
f) Auto splenectomy.

Crisis triggers: Infection, cold, hypoxia, acidosis and dehydration.

2. Sequestration crisis:

Scenario: child with hepatomegaly or splenomegaly and shock + drop in HB.

3. Aplastic crisis:
- Infection with parvo virus B19 with low reticulocytes
4. Hyper hemolytic crisis:
- Sudden severe drop in HB (reach 3 or 4) with high reticulocytes.

Other complications: leg ulcers, gallstones, retinal hemorrhages, osteomyelitis due to

salmonella.

Diagnosis:

- CBC: low HB, normal MCV, increase LDH, Increase bilirubin and retics.
- Sickling test
- Peripheral blood picture: sickle cells.

14 | P a g e
 - Diagnosis is best done by HB electrophoresis.>>goldstandard

Management:

Chronic management:

1. folic acid supplement.

2. Hydroxyurea: itnicreases Hb F, decreases vaso-occlusive painful crisis, decreases
acute chest syndrome and decrease the need for blood transfusion.
3. Vaccination against encapsulated organisms: Pneumococus, Haemophilus
influenzae, Neisseria meningitidis and Influenza.
4. Penicillin up to 5 years.

Acute management: Vaso-occlusive crisis management: Oxygen, IV fluids for

dehydration and acidosis, analgesia, antibiotic if suspected infection (3rd generation
cephalosporin)

Exchange transfusion indication:

- Stroke
- Acute chest syndrome
- Priapism
- Sequestration crisis (both exchange and blood transfusion)

Blood transfusion indication:

- Hyper hemolytic crisis (HB <5)

- Aplastic crisis
- Sequestration crisis

Notes:

▪ SC trait is protective against malaria, but SC disease is high risk for severe
anemia (cerebral malaria) and osteoporosis
▪ Auto splenectomy on Peripheral blood picture → Howell jolly bodies
▪ Thalassemia on PBP → Target cells

15 | P a g e
 4. Paroxysmal Nocturnal Hemoglobinuria:

- CD55 (Decay -accelerating factor) and CD 59 protect RBCs from complement

attack.
- GPI anchored proteins bind CD55, CD59 to RBC membrane, GPI deficiency result
in decrease CD 55, CD59 in RBC.

Case scenario: Pancytopenia and dark urine at morning (due to hypoxia and acidosis
at night).

- HB in blood triggers thrombosis leads to Budd-Chiari (central vein thrombosis) →

MCC of death (tender hepatomegaly and ascites )

Diagnosis:

- Flow cytometry: decrease or absent CD55,CD59 in RBCs membrane→diagnostic

test
- Ham test: sucrose lysis test

16 | P a g e
 5. Autoimmune Hemolytic anemia (AHA):
- Two types: cold and warm AHA

The table below illustrates the differences between them

Differences Worm AHA Cold AHA

Antibody involved IgG IgM

Causes CLL, solid organ CLL, infections: EBV ,

malignancy and other mycoplasma.
autoimmune disease like
SLE.
Hemolysis Extravascular mainly Intravascular mainly

Diagnosis - Diagnostic by Direct coombs test

direct coombs test +spherocytes
(detect antibodies
attached to RBC)
+spherocytes
- Indirect coombs
test is used for
screening
Triggers - Triggered by cold
- Associated with
Raynaud
phenomena
Treatment - Steroids, IVIG, - avoid cold mainly .
splenectomy - Treat the
- Rituximab underlying cause
- Rituximab

17 | P a g e
 Microangiopathic Hemolytic Anemia

Marker: fragmented RBC schistocytes in PBP.

1. Hemolytic Uremic Syndrome (HUS):

Triad:

1. Microangiopathic hemolytic anemia (shistocytes)

2. Acute renal failure (high urea and creatinine)
3. Thrombocytopenia

HUS occurs after bloody diarrhea caused mainly by Enterohaemorrhagic E. coli

O157:H7 or shigella dysentery

Management: supportive.

2. Thrombotic Thrombocytopenic Purpura (TTP):

MCQ: Pentad: Same as HUS triad + fever + CNS symptoms (confusion and coma)

Management: Exchange transfusion (plasmapheresis)

Macroangiopathic Hemolytic Anemia

- Schistocytes in PBP
- Occurs with prosthetic valves e.g.: Aortic stenosis pt.

*************************************************************

18 | P a g e
 Leukemia

1. Acute lymphocytic leukemia (ALL):

- Associated with Down syndrome, most common in children 2-5 years

- Symptoms of bone pain and pancytopenia (recurrent infection, bleeding and
anemia)

Classification:

- By morphology: 1, L2, L3 (have a vacuole, poor prognosis)

- By immunology: blast

Pre B cell (most common 80%, good prognosis)

B cell

T cell (cause anterior mediastinal mass, superior venacava syndrome)

Investigations:

- CBC: anemia low HB, low platelet, variable WBC

- PBP: blast cell
- BM biopsy: >20% blast cell (, lymphoblast), if AML (myeloblast):
- Immunophenotyping (flowcytometry): Detect markers e.g.:

preB cell CD10 (CALA, common ALL Ag )

T cell CD3

B cell CD19, CD20

- Immunohistochemistry: +ve TDt

- Genetic study:
o Most common translocation (12, 21): have good prognosis (Mcq)
o Philadelphia (9, 22): have Poor prognosis in ALL but good in CML

19 | P a g e
Poor prognosis:

1. age <2, >10

2. L3, T cell ALL
3. Male gender
4. Very high WBC > 100,000 at presentation
5. CNS involvement
6. Philadelphia chromosome translocation

Management of ALL: Chemotherapy:

- Induction phase: to induce remission

- Consolidation phase: to kill the residual cells
- Maintenance phase
- CNS prophylaxis: by intrathecal methotrexate or CNS radiotherapy

2. Acute Myelocytic leukemia (AML):

- Classified by FAB classification; M0 to M7

M3 (acute promyelocytic leukemia) APML:

- Have problem with vitamin A receptor so treated by all-trans retinoic acid.

- Have large amount of MPO(Myeloperoxidase) and Auer rods when released in
blood cause DIC. Translocation (15, 17)

M5 (monocyte): characterized by gum hyperplasia

M7 (megakaryocyte): have poor prognosis, associated with down syndrome

Investigation:

- CBC: same as ALL

- PBP: myeloblast, Auer rods (MPO)
- BM biopsy: > 20% myeloblast

20 | P a g e
 - Immunophenotyping
- Immunohistochemistry: MPO +ve
- Genetic study

Management:

- Same as ALL but no need for CNS prophylaxis

NB: Both ALL and AML have hepatosplenomegaly and lymphadenopathy

3. Chronic Myelocytic Leukemia (CML):

- Maturation stages: Promyelocyte to myelocyte to meta-myelocyte to Bands to

mature neutrophil
- Chronic leukemia is asymptomatic affect the mature cell so increases the WBC
in the blood at time of presentation (CBC is so important and BM not very
important)

CML VS leukomoid Reaction:

Both increase WBCs especially granulocyte; note the differences below

CML Leukomoid Reaction

No Dohle body +ve Dohle body response to infection ,toxic
granule
Low enzyme High leukocyte alkaline phosphatase enzyme
WBC >100,000 WBC <50,000
High basophil, all linage High neutrophil(specific), normal basophil
+ve Philadelphia chromosome -ve Philadelphia chromosome

Investigations:

- CBC: very high WBC

- PBP: increase promyelocyte, myelocyte in CML

21 | P a g e
 - Increase Bands, Meta in leukemic reaction
- PCR: for Philadelphia chromosome is diagnostic

Management: Tyrosine kinase inhibitor (Imatinib) in patients with Philadelphia

chromosome +ve

4. Chronic Lymphocytic Leukemia (CLL):

- Presentation: very old patient with lymphadenopathy

Investigations:

- CBC: increase WBC (mature lymphocyte)

- PBP: smudge cells
- Immunophenotyping: diagnostic is CD5+ve B cell

NB: Philadelphia chromosome=> CML

Auer rods=> AML

*************************************************************

22 | P a g e
 Lymphoma

1. B cell lymphoma:

Two types: Hodgkin and Non-hodgkin; see below

Differences Hodgkin lymphoma Non-Hodgkin lymphoma

Histopathology Reed Sternberg(owl eye cell) No Reed Sternberg cells
Spread Spread Contagiously (through the LN) Non contagious
prevalence More common

Prognosis - Better prognosis: presents with B - Poorer prognosis

symptoms early due to the cytokines - Prognosis is basically
secreted by the Reed- Sternberg cells determined by the
(fever, night sweat and weight loss) histopathologic type
- Prognosis as well as the mxx is
basically dependent on the staging

Site Extra nodal: Maltoma of the H.

pylori and HIV CNS lymphoma
Histopathologic 1. Nodular sclerosis 1. Diffuse Large B cell
types Most common, good prognosis lymphoma: most common
2. Lymphocytes rich type 2. Follicular lymphoma: wax
Best prognosis and waning pattern
3. Lymphocyte depleted 3. Mantle Cell lymphoma:
Worst prognosis poor prognosis
4. Mixed cellularity 4. Marginal Zone lymphoma:
in inflammatory condition
e.g. Hashimoto lymphoma,
H. pylori, and Sjogren
5. Burkitt lymphoma:
- t(8,14), C-myc ( N-myc in
neuroblastoma)
- associated with EBV
- Starry sky appearance
(histology)

23 | P a g e
 Types of Burkitt:
a) Endemic: in jaw, African
(OSBE)
b) sporadic: in abdomen
c) HIV associated Burkitt
lymphoma

Diagnosis LN biopsy, splenic biopsy

Staging By:
- CT (shows location)
- PET scan (shows the metabolic activity)

Classification Ann Arbor staging

Stage1: one LN group
- e.g. cervical LN
Stage2: 2 or more at the same side of
diaphragm
- e.g. cervical+ supraclavicular LN or
cervical+mediastinal+supraclavicular
Stage 3: 2 or more at different side of
diaphragm
- e.g. Inguinal LN + cervical or
Inguinal + splenomegaly (spleen
considered as LN)
Stage 4: disseminated Mets (liver, bone
marrow)
- All the stages have A and B subtypes

Management Chemotherapy [ABVD]: Adriamycin (SE:

DCM) / Bleomycin (pulmonary fibrosis) /
Vincristine (peripheral neuropathy) and
Dacarbazine
- All drugs cause myelosuppression

24 | P a g e
 2. Adult Cell Lymphoma-Leukemia:

- Associated with HTLV-1 virus

- Diagnosis by biopsy, staging by CT PET scan
- Treatment: R-CHOP chemotherapy: Rituximab, Cyclophosphamide, (O)
Vincristine and Prednisone

3. CNS Lymphoma:

- Ring enhancing lesion (solitary), DD; Toxoplasmosis (multiple lesion)

- In HIV patient

N.B. calcification in CMV: periventricular

In toxoplasmosis: intracranial, diffuse

*************************************************************

25 | P a g e
 Multiple Myeloma(MM)

- Elderly >60
- Characterized by CRAB: (C, hypercalcemia/ R, renal affection/A, anemia of
chronic illness/B, bone pain)

Causes of renal affection in MM:

1- Bence Jones protein: immunoglobulin light chain in urine

2- myeloma cast: lg

3- hypercalcemia: cause nephrocalcinosis

4- Amyloidosis: cause nephrotic syndrome

Investigations:

- CBC: anemia of chronic disease,

- PBP: packed RBC (rouleaux formation)
- ESR: high 3 figure
- Protein: high serum protien specialy the immunoglobulin
- Urine protein electrophoresis: Bence Jones protein
- Serum protein electrophoresis: M spike
- Skeletal survey: lytic lesions in X-ray
- BM biopsy: diagnostic, >10% monoclonal plasma cell

In monoclonal gammopathy of undetermined significance (MGUS): similar to MM,

<10% monoclonal plasma cell

NB: definitive management of leukemia, Lymphoma and MM is bone

marrow transplantation.

*************************************************************

26 | P a g e
 Myeloproliferative Disorders

- it’s proliferation of the myeloid cell.

- JAK 2 mutation
- Polycythemia: increase RBC
- CML: Increase WBC (granulocyte)
- Essential thrombocytosis: increase platelet
- Myelofibrosis

Polycythemia Rubra Vera:

Aquagenic pruritus due to increase in the mast cell and basophil when they are activated
release histamine

1. CBC: increase HB >16, increase Hematocrit.

2. Plasma volume or RBC mass: true polycythemia (increase RBC count) VS false
or relative Polycythemia (decrease plasma volume e.g shock, burn, prolong
diarrhea)
3. Measure erythropoietin: if low this is polythythemia vera.

If high EPO: there is oxygenation problem, or endogenous increase by tumor

4. then check oxygen saturation or PaO2;

If low: respiratory e.g. high altitude, COPD, lung disease

If normal O2: endogenous erythropoietin from HCC or RCC or hydronephrosis

Management of polycythemia vera:

- venesection
- hydroxyurea
- anti JAK2 (ruxolitinib)

*************************************************************

27 | P a g e
 Platelet Disorders

- At the injury site VWB attaches, then platelet bind to VWB through GP1B
receptor then and produce TXA2, ADP and Ca.
- When ADP binds to its receptor (P2Y12) on platelets this cause conformational
change in platelets to produce GP2B3A receptor which bind to another GP2B3A
receptor on adjacent platelets causing clumping

Clinical Correlation:

- Von Willebrand Disease (VWD): due to VWB factor deficiency

cause bleeding with prolonged bleeding time, normal platelets count,
normal PT, high PTT {affect factor 8}
- GP1b deficiency: cause Bernard-soulier syndrome (prolong bleeding
time, normal platelet count)
- Drugs: antiplatelet; NSAID (reversible inhibition) and Asprin
(irreversible inhibitor) block COX enzyme so decrease TXA2
- ADP receptor blocker: clopidogrel
- GP2B3A defect: cause Glanzmann’s thrombathenia (increase
bleeding time, normal platelet count) or antibodies against GP2B3A
causes ITP (idiopathic thrombocytopenic purpura, increase
bleeding time, decrease platelet count)
- Drug block GP2B3A: Tirofiban, Eptifibatide

➢ Normal measures:
- Normal bleeding time: (1-7 min) increases in platelet problems
- PT: (11-13s) increase in extrinsic pathway problems factors 7 + (common
pathway factors i.e. factor 10, 5, 2, 1)
- PTT: (25-40s) increase in intrinsic pathway problems (factors 12, 11, 9, 8) +
common pathway factors.

28 | P a g e
 ➢ Idiopathic thrombocytopenic purpura (ITP):
- Self-resolving.
- Indications for treatment: if very low count (< 10, 000) or major bleeding
- Treatment: steroids (1st line), plasmapheresis, IVIG, and anti D
➢ HUS, TTP and ITP:
- (prolonged bleeding time, low platelet count)
➢ VWD:
- prolonged bleeding time, normal platelets count, normal PT, high PTT {affect
factor 8}
➢ Hemophilia A:
- normal bleeding time, normal platelet count, normal PT, high PTT affect factor 8
- X-linked, male, present with hemearthrosis (deep joint bleeding)
- Treatment: mild: desmopressin (ADH). If Severe: (factor 8 concentrate)
➢ DIC:
- Increase bleeding time, decrease platelet count, increase PT, increase PTT,
decrease fibrinogen and increase fibrinogen degradation product (D-dimer)
- Occurs in patients with severe trauma, pancreatitis, ICU sepsis and snake bite
- Patient come with bleeding from puncture site and thrombosis
➢ Vitamin K deficiency and liver disease:
- Neonate present with bleeding from umbilical cord
- Normal Bleeding time, normal platelet count, increase PT, increase PTT
➢ Thrombophilia or thrombosis:

1. Hereditary: factor v leiden which is the resistance of factor v to degradation

by protein C and S, Anti-thrombin deficiency, protein C and protein S deficiency.

2. Acquired: causes: Antiphospholipid syndrome and anticoagulants (check

Kibreet sheet page 196)

N.B: remember protein C and S deficiency can present clinically as skin necrosis
after taking warfarin.

29 | P a g e
 Auer rods of AML Smudge cell of CLL Reed Sternberg (owl eye
cell).

CNS lymphoma Starry Sky appearance, Non- Hemearthrosis in hemophilia

Hodgkin lymphoma

rouleaux formation, MM Lytic lesion of MM

30 | P a g e
 Cardiology

Arrhythmia:
- Normal heart rate (HR) 60-100 beat/miute
- HR >100 is called tachyarrhythmeia
- HR<60 => bradyarrhythmeia

1. Tachyarrhythmeia
divided according to its origin into:

1. Ventricular: here the abnormality is in the ventricle. Has broad QRS on ECG
2. Supraventricular: the origin is above AV node
- types of supra ventricular tachyarrhythmia: Atrial fibrillation, atrial flutter,
multifocal atrial tachycardia, AVRT
- All have narrow QRS on ECG

➢ Sinus tachycardia:

- Normal rhythm but HR> 100, all waves

are normal.

Causes:

- Physiological: Anxiety, exercise

- Pathological: thyrotoxicosis, fever

31 | P a g e
 How to calculate the HR?

- Firstly, check if the rhythm regular or irregular by RR interval because based on it

we calculate the HR

For regular rhythms: HR= 300/No. of large

squares between RR wave

Alternatively, you could use: 1500 /No. of small

squares between RR

- On ECG paper small square=0.04s

- One large square (5 small squares) = 0.2s
- 5 large squares= 1s
- 30 large squares= 6s

For irregular rhythm: we calculate the number of R waves in 30 large squares ×10

(No. of R waves in 6s × 10)

- The formula above is more accurate and can also be used for regular rhythm
- Alternatively, you could use (number of R waves in 50 large squares ×6)
- Example: on ECG if you find 17 R waves in 30 large squares, calculate the HR?

17*10=170

MCQ: Any tachyarrhythmeia with one of the following is for synchronized DC shock:

1. Low BP(SBP<90)
2. Syncope
3. Angina (chest pain)
4. HF(dyspnea)

Sawaiq exam question: Patient was hypertensive and the symptoms go with left ventricular
hypertrophy. The ECG showed Bradycardia because the patient took Beta blocker as
treatment so this is sinus bradycardia (don’t answer it as LVH) check the ECG!!!!

32 | P a g e
 ➢ Atrial fibrillation(AF):
- has the following criteria
1. on ECG:
- Irregular (R_R interval), irregular rhythm
- No P wave but has fibrillatory waves
- Narrow QRS (supraventricular) (less than 3 small squares)
2. Absent (a) wave on JVP
3. Pulse deficit (clinical sign): normally there’s a difference between the pulse heard
by a stethoscope directly from the heart and the pulse calculated from radial artery.
This difference shouldn’t be more than 10. if it’s more than 10 then this is AF.

Causes of AF: could be anything

- IHD
- Valvular disease (especially MS)
- HTN (most common cause)
- Cardiomyopathy
- Surgery
- Thyrotoxicosis (common cause in surgery)
- Idiopathic AF (called Lone AF): here the patient is young and has no any heart
problems

33 | P a g e
Types of AF:

1. Paroxysmal AF: this comes and goes by itself

2. Persistent AF: this resolves by DC shock or drugs (electrical or chemical cardio
version)
3. Permanent AF: non resolving i.e. rhythm doesn’t go back to normal sinus rhythm.
That’s why we don’t give drugs to control the rhythm here. We only give drugs to
control the rate+ anticoagulation

Management of AF:

1. Rate control: BB, CCB (cardioselective:verapamil, diltiazem), digoxin

- we don’t give Nifedipine and Dihydrobyridine because it can cause reflux
tachycardia
- the selection of the drug depends on the case. For example, diabetic and asthmatic
patients don’t give BB. For patients with HF give digoxin

2. Rhythm control: either chemical or electrical

- Chemical: either Flecainide (if no structural heart disease; no valvular disease or
MI) or Amiodarone (used for patients with structural or valvular heart disease)
- N.B: Flecainide is used for Lone AF
- Electrical: synchronized DC shock
- Acute AF (<48 hrs): here there’s no risk of thrombus formation => rhythm control
immediately by DC
- Chronic (AF >48 hrs or unknown): here there’s a risk of thrombus formation which
can migrate and cause stroke. We don’t do DC here instead we either give warfarin
for 3 weeks then do the DC after 3 weeks then give warfarin again for one month
or do transoesphageal echo and if there’s no thrombus we go for DC if there’s a
thrombus we follow the regimen of warfarin

N.B: warfarin doesn’t prevent formation of thrombi but prevent propagation of already
formed thrombi. Also it causes thrombus fibrosis

34 | P a g e
Rate control VS rhythm control: they have the same efficacy, morbidity and mortality

- If Lone or young patient >>>>rhythm control

- If elderly patient >>>>>>rate control
3. Anticoagulation: by CHA2DS2 VASC score
- If score o (usually Lone AF): give Asprin or better give nothing
- If score 1: either give anticoagulant or better give Asprin
- Score 2 or more: for anticoagulation, warfarin or DOAC.
- Target INR here :2-3

N.B: we don’t use pacemakers for tachyarrhythmia. They’re used for

bradyarrhythmeias

Notes:

DOAC: direct oral acting anticoagulants e.g. factor 10 inhibitors (end with
xaban:(apixaban, rivaroxaban).

NOAC (is a form of DOAC): Novel oral acting anticoagulant e.g. factor 2 or thrombin
inhibitor (dabigatran the only one that given orally) other factor 2 inhibitors are given

I.V.

35 | P a g e
 category Score
C=CHF 1
H=HTN 1
A= Age≥75 2
D= DM 1
S2=Stroke/TIA 2
V=Vascular disease ( 1
atherosclerotic diseases)
(e.g. Limb ischemia, claudication,
chronic mesenteric ischemia,
carotid stenosis )
A=Age 65-74 1
S=Sex:
Female 1
Male 0

Notes:
Synchronized DC shock: here we give the shock while reading the ECG so that we can
give the shock at safe point to prevent ventricular fibrillation(VF)
N.B: if we give the shock here during the QRS, this could cause VF
Unsynchronized DC shock (defibrillator): this is part of advanced life support (ALS).
It’s used for VF without reading the ECG during the time of the shock
Types of rhythms:
A- shockable rhythm (for unsynchronized DC shock): VF, pulseless VT
B- non-shockable rhythm (only for CPR): asystole, pulseless electrical activity (PEA)

36 | P a g e
 ➢ Atrial flutter:

- Saw teeth on ECG *

Management: Same as atrial fibrillation.

➢ Paroxysmal Supraventricular tachycardia (SVT):

- Paroxysmal -regular-narrow QRS-fast heart rate (150_200)

Common OSPE picture: *

Management:

- Step 1: vagal stimulation maneuver: carotid massage, Valsalva maneuver, eye ball
pressure, ice packs.
- Step2: Drugs; IV adenosine (CI in asthma)
- if failed, give again 3 times and increase the dose.
- if failed, give verpamil CCB

37 | P a g e
 ➢ Ventricular tachycardia (VT):

- Wide QRS-regular -heart rate 200

➢ Monomorphic (VT):
- all QRS has the same shape.

Management:

1. If hypertension=synchronized DC shock
2. Drug of choice: amidarone, 2nd line is lidocaine

➢ Polymorphic VT: Torsades de Dointes:

- Risk factor for polymorphic VT is long QT which may be due to:

1. congenital long QT: K channel defect.
2. Electrolytes disturbances: hypo K, hypo Mg ,hypo ca+2
3. Drugs: ABCDE
- A: anti Arrhythmic
- B: ABx ( macrolides "azithromycin", quinolones "ciprofloxacin")
- C: anti Psychotics
- D: anti-Depressant
- E: anti Emetic "ondansetron"

38 | P a g e
 OSPE PICTURE:

Management:

1. DC shock if one of the 4 indications is found (hypotension/angina/heart

failure/syncope)

2. if no=Mg sulfate

Side effects of amiodarone:

1. lung fibrosis

2. gray man syndrome, due to deposition in skin &cornea.

3. Hepatotoxicity

4. Hypo or Heperthyroidism

- Notes:
- Pulmonary fibrosis and hepato_ toxicity >>> also are S. E of Methotrexate
- Flecainide is contraindicated in structural heart disease=class 1c
- Amiodarone is class 3 anti-arrhythmic drug

*************************************************************

39 | P a g e
 2. Bradyarrthymia

Heart rate <60 then think of:

1. sinus Bradycardia

2. heart block

1. Sinus Bradycardia:

Causes:

physiological: cold, sleep

pathological: Hypothyroidism.

2. Heart block:

1. 1st degree heart block:

Prolonged PR interval, normal (0.12-0.20s) (3_5 small squares)

Has pattern e.g. 3:1 2:1

2. 2nd degree heart block: Types:

a) Mobtiz 1(wenckebach phenomenon): Progressive lengthening of PR interval +
drop in QRS complex
b) Mobtiz 2: sudden drop in QRS complex... PR interval may be prolonged, short or
normal

40 | P a g e
 3. 3rd degree heart block: P-P regular, R-R irregular, independently

Management:

- 1st degree heart block and Mobitz 1: benign, no treatment.

- 3rd degree heart block and Mobitz 2: peacemaker may use atropine to increase
heart rate.

N.B. ST elevation: compare it to baseline. Concave or convex ST elevation

➢ Axis deviation:
- can be seen in lead I, II, III avF
- Normal: all positive

Left Axis Deviation:

- lead II is negative
- Lead I or III positive

Right Axis Deviation:

- lead I is negative
- Lead II or III positive

41 | P a g e
LVH: V5 V6 = tall R waves RVH: V1 V2= long R waves

V1 V2 =deep S waves V5 V6= deep S

Notes:

- If avR is positive suspect limb lead reversal because avR is always negative*
- RVH seen in COPD and HTN
- Rhythm script is lead II

*************************************************************
 Rheumatology

➢ Rheumatoid Arthritis:

- Def: chronic, inflammatory, multisystem autoimmune disease

- Female > Males
Associations:

HLA DR4, DR1 (SLE associated with HLA DR3) IHD (RA = Type II DM)

Dx: Female with arthritis and Morning stiffness >1hr and one of the followings (for ≥
6wks):

(The followings “1-4” are the criteria of the arthritis)

1. Polyarthritis ≥ 3 joints
2. Symmetrical involvement
3. Hand Involvement (MCPJ “Metacarpal phalangeal Joints”, PIPJ “Proximal
interphalengeal joints”, wrist (NO DIP)
4. +ve Rheumatoid Factor (RF) → IgM against Patient’s IgG
5. Radiological changes (X-ray):
• Early: Peri-articular (juxta-articular) osteopenia → risk of osteoporosis
• Late: bony erosions, subluxation
- In pathology of RA: TNF is a key factor
Extra-articular manifestations:

1. Subcutaneous nodules (rheumatoid nodules) → found near the elbow and the
olecranon process also may be found near the small joints of the hand (OSPE)
2. Pulmonary Fibrosis, B.obliterans, Pleural Effusion (serositis)
3. Serositis; pleural effusion, pericardial effusion
4. Depression
5. Increased risk of infections (Proteus mirabilis)

42 | P a g e
 6. Rare: Felty’s syndrome (RA + Splenomegaly + neutropenia), Caplan syndrome
(RA + Pneumoconiosis)
7. Rare:
- Ocular: most common is Keratoconjuctivitis sicca (directly or associated with
sjogren syndrome), episcleritis, uveitis
8. Neuro: Atlanto-axial Subluxation (C2→ Sudden death), Carpal Tunnel
Syndrome.
Note: Atlanto-axial Subluxation is found in rheumatoid arthritis, down syndrome
and juvenile idiopathic arthritis.
Complications:

- Osteoporosis.
- Amyloidosis causes nephrotic syndrome
- Ischemic heart disease (the risk is doubled)
- Deformities: Boutonnière and swan-neck deformities of fingers or Z-deformity of
thumbs

Investigations:

- CBC: Anemia
- ↑ESR ↑CRP
- Rh F: +ve in 70% (most sensitive)
- Anti-CCP (Anti Cyclic-citrullinated peptide antibodies) = most specific 98%
- Radiology
- Joint aspiration: WBC count → 20,000 – 50,000 (<200 is normal)
o 200 – 2,000 (Osteoarthritis “non-inflammatory”)
o 2,000 – 50,000 = (inflammatory “RA, gout”)
o >50,000 = Septic Arthritis
Poor Prognosis:

- Female
- Insidious onset
- HLA DR4 +ve

43 | P a g e
 - +ve RhF, Anti-CCP, Radiological changes
- Extra articular features
Mx:

- Acute: Rest, NSAIDs, Steroids

- Long term: 2 DMARDs (one Methotrexate) + short term Glucocorticoids

DMARDs: Disease-modifying antirheumatic drugs ↓ complications:

(Methotrexate + other DMARDs)

1. Methotrexate (anti-folate): Most common
- SE:
1. Bone marrow suppression (anti-cancer drug)
2. Liver cirrhosis (hepatotoxicity)
3. Pneumonitis → Pulmonary fibrosis
4. Mucocitis (oral ulcer)
2. Sulfasalazine (sulfa allergy rash, ↓sperm reversible, oral ulcers)
3. Hydroxychloroquine (Eye involvement; retinopathy … annual screening by
fundoscopy)
4. Lefluonimde (teratogenicity)
2nd Line: Biological Therapy (e.g. TNFa inhibitors)

- Screen for TB, Hepatitis B,C, HIV before starting

- Started when resistance to 2 DMARDs (including methotrexate)
- Infliximab, Rituximab, Tocilizumab, Abatacept
N.B: in pregnancy NSAIDs & Methotrexate are Contraindicated!

44 | P a g e
 ➢ SLE (Systemic Lupus Erythematosus):

- Females: Males 9:1 Ag-Ab complex deposition (Type 3 HSR)

Dx: ≥ 4 of the following: (at least 1 clinical + 1 laboratory) or biopsy proven lupus-
nephritis + +ve ANA or Anti-DNA

1. Malar Rash (spare nasolabial fold)

2. Discoid Rash “commoner, scaling or scar as a complication”
3. Photosensitivity rash
4. Oral ulcers
5. Serositis
6. Arthritis (≥ 2 peripheral joints) with no erosion on X-ray (to differentiate it from
rheumatoid arthritis
7. CNS: seizures, psychosis
8. Hematological: Anemia, neutropenia, lymphopenia, Thrombocytopenia (all are
Autoimmune)
9. Renal: (Persistent Proteinuria >0.5 g/day, casts) → Nephritis
10. Immunological: e.g +ve Anti-dsDNA, Anti-SM abs
11. +ve ANA
N.B: Abs in SLE:

- Most sensitive: ANA

- Most specific: Anti-Smith
- Follow-up: Anti-dsDNA (prognostic)
- Others: Antiphospholipid Abs [Anti-cardiolipin / Lupus anticoagulant]
- False +ve = syphilis
- 20% have +ve RhF
- Anti-histones with drug induced lupus
For monitoring & follow up:

- Anti-dsDNA (-ve in drug lupus)

- Complement levels (low C3, C4)

45 | P a g e
 - ESR (↑ ESR but normal CRP)
Multisystemic disorder + ↑ ESR but Normal CRP >> think of SLE

N.B Anti Ro / Anti La in pediatrics:

- ↑ risk of neonatal lupus

- Baby with heart block

Lupus nephritis [LN]:

- Stage I: Minimal mesangial LN (No tx)
- Stage II: Mesangioproliferative LN (may need steroids)
- Stage III: Focal LN (<50% of glomeruli)
- Stage IV: Diffuse LN (>50% of glomeruli) (Most common/most severe)
- Stage V: Membranous GN
- Stage VI: Advanced Sclerosing LN (ESRD)
o N.B: requires Renal Biopsy to diagnose.

o Tx: Mycophenolate(the most important drug)

o 3 – 6 of IV cyclophosphamide + IV high dose steroids ± RRT
o N.B. SE of Cyclophosphamide: Hemorrhagic cystitis, Infertility.

Drug induced Lupus:

- Isoniazid, Hydralazine, Procainamide, Penicillamine.

- N.B: OCP, Sulfonamides worsens Lupus
- Anti-histone Abs in 100
- N.B: Anti dsDNA is –ve
Lupus in pregnancy:

- Anti Ro- Anti La Abs = ↑ risk of neonatal complications (Heart block) [SSA]
Investigations:

- ↑ ESR - Normal CRP

- Abs - Complements: Low

46 | P a g e
Management:

- The management differs and needs specialist

- For mild symptoms (arthritis) → NSAIDS
- Rashes → steroid creams
- Maintenance treatment of the above symptoms by hydroxychloroquine
• Acute flares of the severe manifestations (e.g psychosis, nephritis,
hematologic):
- IV Cyclophosphamide (S.E hemorrhagic cystitis) + IV high dose steroids
• Chronic [Maintenance]:
- NSAIDs
- Hydroxychloroquine
- Steroid sparing agents (Azathioprine, Methotrexate, Mycophenolate)
- Steroids
N.B: mycophenolate can be used instead of cyclophosphamide

Raynaud’s Syndrome:

- In stress, cold. Causes: SLE, Scleroderma, vasculitis, drugs (beta blockers)

- Types:
- Primary: Teenage females + family hx (Raynaud’s disease)
- Secondary: Male / Female >30 yrs. (Raynaud’s Syndrome)
- Differentiated by nail fold capillary-scope, primary has no complications,
secondary can cause gangrene and ulcers
- Tx: by vasodilator or CCB
Livedo Reticularis:

- “see picture for it”

- Causes: SLE/APL

47 | P a g e
 ➢ Gout:

Definition: Deposition of monosodium urate (MSU) crystals in joints

- Typical Presentation: Acute attack of monoarthritis

- 60% in Metatarsophalangeal joint of big toe (Podagra)
- Long term: urate deposits (Tophi in pinna, tendons, joints) and uric acid stones
- More in males
Causes:

1. Hereditary: Lesch-Nyhan syndrome (+ learning difficulties + RF) & Von Gierke

disease
2. Secondary:
o Reduced Excretion: Renal impairment
o Increased production: tumors especially hematological malignancies →
Myeloproliferative / Lymphoproliferative (e.g Leukemia)
o Cytotoxic drugs (Tumor Lysis syndrome) ↑Ph ↑K ↑Uric acid ↓Ca
Precipitants and flare gout attack:

- Trauma/Surgery (stress)
- Dietary: ↑ purine, Alcohol, starvation
- Infections
- Diuretics (Thiazide hyper GLUC, hyperuricemia)
- Dehydration
Associations:

- HTN, IHD, metabolic Syndrome

- Hyperuricemia
Complications:

- Tophi (e.g. ear pinna) - Urate renal stones

Dx: X-ray: soft tissue swelling, recurrent punched out erosions

48 | P a g e
 • NB: joint space is preserved until late
- Joint Aspiration: microscopy:
- Negatively birefringent needle shape urate crystals (OSPE)
Tx:

- High dose NSAIDS “e.g.: Ibuprofen, indomethacin”. If CI (e.g. PUD) →

Colchicine
- If CI both (e.g. renal impairment) → Steroids
Allopurinol Indications (Xanthine Oxidase inhibitor):

1. Recurrent attacks (≥ 2/yr)

2. Tophi
3. Renal stones
- When to give: After 2 weeks from acute attack, under cover of
NSAIDs/Colchicine
- Why? It precipitates attack first
Lifestyle modifications:

- Weight loss, ↓ Purines, avoid starvation, avoid alcohol

N. B: D.Dx for gout: Pseudogout/Septic arthritis

➢ Pseudogout:

- Deposition of Ca+2 pyrophosphate dehydrate

- Large joints (Knee, wrist…etc)
Causes:

1. Hemochromatosis
2. Hyperparathyroidism
3. Hypothyroidism
4. ↓Mg2+ / ↓ PO4

49 | P a g e
Dx:

1. X-Ray: Chondrocalcinosis
2. Joint fluid microscopy: Positively birefringent rhomboid shape crystals
Tx: self-limiting. Can give NSAIDs

- NOTE about gout: serum uric acid maybe normal in acute attack
- Do not give Aspirin in acute attack (in low dose ↓ uric acid excretion)
- So in Gout: Acute attack: give NSAIDs. Maintenance: Allopuranol

➢ Scleroderma:
Collagen deposition (fibrosis)

1) Diffuse (systemic sclerosis):

✓ Involve all the skin
✓ Early visceral involvement:
- Pulmonary fibrosis
- Cardiac problem
- Nephrotoxicity (scleroderma renal crisis)
2) Limited form (involve face, hand, feet):
✓ CREST syndrome
✓ Limited skin involvement (face, hand, feet)
✓ Late visceral involvement:
- Pulmonary arterial hypertension
• CREST Syndrome: (OSPE)
o Calcinosis: calcified nodules in skin
o Raynaud's phenomena (**cyanosis in peripheral parts with cold)
o Esophageal motility disorder (**replaced the muscle by fibrous tissue = no
contractility)
o Sclerodactily (**fibrosis in digits)
o Telangiectasia
- On myometry: absent peristalsis + decreased LES tone.

50 | P a g e
▪ Antibody:

- Diffuse: Anti SCL 70 Topoisomerase

- Limited: Anti centromere
▪ Management:

- Symptomatic treatment
- Renal crisis by ACEI

➢ Sjogren's syndrome:

Common in OSBE
Case scenario: dry eye (Keratoconjunctivitis sicca) +dry mouth (Xerostomia)

- Anti Ro (SSA) / Anti La (SSB)

- ANA or Rh factor
- Same clinical picture + parotid enlargement + lacrimal enlargement → Mikulicz
syndrome

➢ Polymyositis:
- More in females
- Proximal muscle weakness + pain

➢ Dermatomyositis:
= poly myositis +pain+ skin manifestations

Skin manifestation of dermatomyositis:

- Heliotrope rash
- Gottron’s papules
- Shawl sign
▪ Antibody: Anti Jo 1

51 | P a g e
▪ Investigation:

- Diagnosis by muscle biopsy.

- Increase Creatinine Kinase.
- EMG (inflammatory pattern)
- Always screen for lung cancer. Associated with SCLC.

➢ Ankylosing Spondylitis:
X ray of ankylosing Spondylitis

- More in males
- Associated with HLA B-27
- Bamboo spine: of axial spine (vertebra + sacroiliac joint).
- >> Back pain + morning stiffness.
Complications:

1) Anterior uveitis
2) Aortic regurgitation
3) Restrictive lung disease: By fibrosis or restrict chest mobility.

52 | P a g e
 OSPE Pictures (Rheumatology)

Rheumatoid nodules Boutonniere deformity in PIP joint

SLE Malar rash Gouty arthritis (tophi)

Heliotropic rash → dermatomyocytits

Gottron papules

*************************************************************

53 | P a g e
 NOTES in Emergencies

1. Cardiology

➢ Management of Acute Coronary Syndrome (ACS):

1. Give MONA to all patients (Morphine IV with IV metoclopramide, O2, Nitrates
sublingually, and chewable Aspirin)
2. New guidelines:
- O2: is given depending on oxygen saturation
- Nitrates: avoid it in STEMI (patient has hypotension)
- Aspirin is given with clopidogrel

Management of STEMI: Management of STEMI: after giving MONA, do

reperfusion therapy then either of two:

a) PCI: Stent and ballon to open the artery, always superior to thrombolytic therapy,
the ideal time is within first 120 min (2 hrs) (Door to balloon time 90 min
optimally) if you couldn’t find it within 120 min give thrombolytics.
b) Thrombolytic therapy: by streptokinase or by tissue plasminogen activator
(tPA), the optimum time is 30 minutes (Door to needle time)

Contra-indications to thrombolytic therapy:

1. Hemorrhagic stroke EVER
2. Ischemic stroke in previous 6 months
3. Upper GI bleeding in previous 1 month
4. Major trauma or surgery in previous 3 weeks
5. Pregnancy
6. CNS tumors (because they are highly vascular)
7. Severe HTN
8. Bleeding disorders

54 | P a g e
Management of non-STEMI: If there is no ST elevation: this is either NSTEMI or
unstable angina, both of them has the same treatment; after giving MONA give heparin
(unfractionated or henoxaparin)

Further management: depends on risk factors... Tim score

(High risk patients are: patient with -Diabetes -High cardiac markers -ST depression -
Persistent or recurrent pain)

- If the patient has high risk do angiography in 72 hour or 96 hours, then do PCI or
CABG
- Before doing PCI: give glycoprotein II B / III A (abciximab, tirofiban,
eptifibatide)
- After PCI give: dual antiplatelet therapy (DAPT)= Aspirin+ clopidogril for 1
year

Discharge medications:

1. Aspirin for life

2. β Blockers for life
3. ACEI for life
4. Statin for life
- All the above drugs decrease the mortality

In heart failure:

- Most of the drug decrease mortality (ACEI inhibitor, b blockers, spironolactone

[given for stage 3 and 4 NYHA], Hydralazine Nitrates mixture and ICD.
- Digoxin, loop diuretic "Furosemide" and lastly ca channel blocker (not routinely
used)>>don’t decrease mortality.

➢ Hypertensive crisis:
- Blood pressure more than 180/120 Or 130

55 | P a g e
 ➢ Hypertensive emergency:
- Very high BP with end organ damage e.g. Brain (Encephalopathy, stroke), eye
(hemorrhage papilledema), kidney (AKI), heart (IHD), aorta (aortic dissection).
- Lower the bp immediately but don't lower more than 25% in first 2 hours (if 200
don't lower to 150 in the first 2 hours this can lead to ischemia stroke).
- Give drugs by IV route labetalol or Na nitroprusside (second line).

➢ Hypertensive urgency:
- Very high BP without end organ damage.
- Give oral: ACEI "captopril", b blacker, clonidine

N.B. Lower the blood pressure gradually over 1-2 days

➢ Malignant hypertension:
- It’s an obsolete expression.

56 | P a g e
 2. Renal

➢ Nephrotic Syndrome (NS):

Primary causes: The most important causes in children is Minimal Change Disease

- Others are Focal segmental glomerulosclerosis, membranous glomerulonephritis

Secondary causes:

• Amyloidosis
• Multiple myeloma (case presentation; proteinuria, hypercalcemia> 10, bone pain,
old age)

Nephrotic range: lab findings

- Protein 24 hrs > 3.5 gm

- Prt/crt>300
- ACR>200

Clues in the case:

- Frothy urine (Urine dipstick three crosses +++ or more, less than that it's not on
Nephrotic range)
- They have risk of infections because of loss of immunoglobulins in urine (The
responsible organism for infections in Liver cirrhosis is E. coli from the colon. In
NS it’s S.pneumonae causing SBB)

Case# Pt with nephrotic syndrome has loin pain, hematuria=renal vein thrombosis

- Nephrotic syndrome makes the person susceptible to thrombosis that’s why they’re
given heparin
- Thrombosis occurs mainly due to loss of antithrombin III, mainly.

Case# Adult with recurrent benign hematuria 1-2 days after respiratory tract infection
think of IgA nephropathy or Burger syndrome.

57 | P a g e
 3. Endocrine

➢ Panhypopitutarisim:
- usually because of Sheehan syndrome
- Here Hydrocortisone is given before the other hormones (MCQ)

➢ Thyroid emergencies:
- all should be managed in ICU

Thyrotoxic crisis:

- IV hydrocortisone not oral prednisolone (MCQ)

- Propyle thiouracil
- B blockers
- Lugol’s iodine is given after one hour after the propylthiouracil to block the
synthesis of the hormone (if you give it before the antithyroid medications, the
gland might use it to synthesis more hormone)
- Cooling

Myxedema coma(hypothyroidism):

- IV thyroxine
- Respiratory support (very important because they develop hypoventilation)

4. GI Emergencies
- Read about Rockall score of UGIB
- Antibiotic decreases the mortality rate in UGIB (it also decreases the mortality in
use of noninvasive ventilation in acute exacerbation of COPD)

*************************************************************

58 | P a g e
59 | P a g e