Hematological disorders in pregnancy

Dr Suhair Qudsieh
Maternal fetal medicine consultant
Yarmouk University
ANAEMIA IN PREGNANCY
Classification of anemia

• Physiological anemia of pregnancy

• Pathological
– Deficiency anemia
• Fe deficiency
• Folic acid deficiency
• Vit B12
• Protein deficeincy
– Haemorrhagic
• Acute – following bleeding in early months or APH
• Chronic – Hookworm infestation, bleeding piles
– Hereditary
• Thalassemias
• Sickle cell haemoglobinopathies
• Other haemoglobinopathies
• Hereditary haemolytic anemias
– Bone marrow insufficiency
– Anemia of infection
– Chronic disease (renal) or neoplasm
Physiological anemia
• In pregnancy:
– Increase in plasma volume, RBC volume and Hb
mass
– Marked demand of extra iron specially in the second
half which cannot be overcome by diet.
• Thus, there always remains a physiological iron
deficiency state during prenancy.
• As a result, there is not only a fall in Hb conc and
hematocrit value in the second half of pregnancy,
but there is also associated low serum iron.
Criteria for physiological anemia

• Hb – 10gm%
• RBC: 3.2 million/mm3
• PCV: 30%
• Peripheral smear: normal morphology of RBC
with central pallor
Degree of anemia

• Hb
• Total Red cell count
• Determination of PCV
Mild Between 8 -10 gm%

Moderate Less than 8 – 7gm%

Severe Less than 7gm%

Type of anemia

• Peripheral blood smear

• Haematological indices: MCHC, MCV and MCH

• Serum iron value

Complication of severe anemia

• During pregnancy:
– Intercurrent infection
– Heart failure
– Preterm labour
• During labour:
– PPH
– Cardiac failure
– Shock
• Puerperium:
– Puerperal sepsis
– Subinvolution
– Failing lactation
Effects on baby:

• LBWt- low birth weight

• Increased incidence Intra uterine death
Prognosis:

• Maternal: if detected early  good

• Fetal: if detected early and responsive to
treatment , good prognosis.
– But in severe and neglected cases, there can be
prematurity
Treatment

• Prophylactic:
– Avoidance of frequent child births
– Supplementary iron therapy
– Dietary prescription
– Adequate treatment
– Early detection of falling Hb level is to be made
• Curative:
– Hospitalisation
– General treatment:
• Diet
• To improve appetite and facilitate digestion
• Effective therapy to cure the disease
Specific therpay:

• Depends on:
– Severity
– Duration of pregnancy
– Assoc complicating factors
Iron therapy

• Oral route:
– Drawbacks:
• Intolerance
• Unpredictable absorption rate
– Response:
• Sense of well being
• Increased appetite
• Improved outlook of the patient
• Haematological examination
• Rise in Hb level
– Improvement is usually within 3
weeks
– Failure:
• Improper typing of anemia
• Defective absorption
• Pt fails to take iron
• Concurrent blood loss
– Contraindication:
• Intolerance
• Severe anemia in advanced pregnancy
• Parenteral therapy:
– Intravenous and Intramuscular
– Indications:
• Contraindications of oral therapy
• Pt not co-operative to take oral iron
• Cases seen for the first time during the last 8 – 10
weeks in severe anemia
• Intravenous route:
– Total dose diffusion: deficit of iron is calculated and
the total amount of iron required is administered by a
single sitting intravenous infusion
– Advantages:
• Eliminates repeated and painful intramuscular
injections
• Treatment completed in a day
• Intramuscular therapy:
– Total dose to be administered is calculated
– After an initial dose of 1ml, the injections are given
daily or on alternate days in doses of 2ml
intramuscularly.
– Drawbacks:
• Painful
• Chance of abscess
• Reactions
• Blood transfusion:
– Limited. But indications are:
• PPH
• Severe anaemia in later months of pregnancy
• Refractory anemia
– Quality and quantity: fresh. Only packed cell. 80 –
100 ml at a time
– Advantages:
• Increased oxygen carrying capacity of the blood
• Hb may be utilised for the formation of new red
cells.
• Stimulated erythropoiesis
• Improvement expected after 3 days
• There is derangement in red cell maturation with the
production in the bone marrow of abnormal
precursors known as megaloblasts due to impaired
DNA synthesis.

• Vit B12 or folate defieciency or both.

• Vit B12 deficiency is rare in pregnancy.
• In pregnancy, due to folic acid deficiency.
Folic acid deficiency…

• Inadequate intake due to:

– Nausea, vomiting, loss of appetite.
– Dietary insufficiency – green leafy veg, cauliflower, spinach,
liver, kidney
• Increased demand:
– Increased maternal tissue including red cell volume.
– Developing product of conception.
• Diminished absorption:
• Abnormal demand:
– Twins, infection, haemorrhagic states.
• Failure of utilisation:
• Anticonvulsant drugs. Diminshed storage:
– Hepatic disorders and vitamin c deficiency.
Incidence

• 0.5 – 3%
• Common in multiparae and multiple
pregnancy.
Haematological examination
and other blood values
• Hb – 10gm%
• Stained blood film: hypersegmentation of the
neutrophils, macrocytosis and anisocytosis.
Megaloblasts.
• MCV- 100 µm3
• MCH –high, MCHC –normal
• Associated leucopenia and thrombocytopenia
• Serum iron is normal or high
• Serum folate – 3ng/ml
• Serum B12 level below <90 pg/ml
• Bone marrow – megaloblastic erythropoiesis
COMPLICATIONS

• Abortion
• Prematurity
• Abruptio placentae
• Fetal malformation
Prophylactic therapy

• All woman of reproductive age should be given

400µg of folic acid daily.
• Additional amount (4mg) should be given where
demand is high.
HAEMOGLOBINOPATHIES
SICKLE CELL ANAEMIA
• Hereditary disorders in which the red cells contain
Hb-S.
• Produced by substitution of valine for glutamic
acid at the position 6 of the β-chain of normal
haemoglobin.
Pathophysiology

• Red cells with HbS in oxygenated state behave

normally but in the deoxygenated state it
aggregates, polymerises and distort the red cells
to sickle.
• These sickle shaped cells block the
microcirculation due to their rigid structure.
Effects on pregnancy

• Increased incidence of abortion,prematurity,

IUGR and fetal loss.
• Perinatal mortality is high.
• Preclampsia, postpartum haemorrahage and
infection is increased.
Effects on the disease.

• There is chance of sickle cell crisis which usually

occurs in the last trimester.
• Haemolytic crisis
• Painful crisis.
Management

• Preconceptional counselling
• During pregnancy: antenatal supervision, regular
blood transfusion at 6 weeks interval is indicated
in certain cases.
• Contraception: sterilisation, oral pill, barrier
method is ideal.
Thalassaemia syndromes

• Commonly found genetic disorders of the blood.

• Basic defect is a reduced rate of globin chain
synthesis.
• As a result, the red cells being formed with an
inadequate haemoglobin content.
• Types – alpha and beta (depending upon the
chain)
 Alpha thalassaemia

• Incompatible with life.

• Α-peptide chain production is controlled by 4 genes,
located on chromosome 16. Depending upon the degree
of deficient synthesis – 4 clinical types.
• Mutation of one gene: no clinical or laboratory
abnormalities. Silent carrier
• Mutation in 2 – 4 genes: minor. Often goes
unrecognised and pregnancy is well tolerated.
• Mutation in 3 – 4 genes: Hb H disease. 
hemolytic anaemia.
• Mutation in all four genes: major. No alpha globin
chain. Fetus dies either in utero or soon after birth.
Beta thalassemia

• beta chain production is directed by 2

genes – one on each copy of chromosome
11.
• Major – when mutation affect both the
genes. – red cell destruction- no
erythropoiesis – blood transfusion
necessary for survival.
• Minor – mutation of one gene. – can
tolerate pregnancy –oral folic acid
supplementation is continued.
Thrombocytopenia in
pregnancy
- Gestational thrombocytopenia.
- Pre-eclampsia
- ITP
- TTP
- DIC
- SLE
Idiopathic thrombocytopenic purpura
ITP

• Caused by antibodies to surface antigen on platelets,

leading to platelet destruction.
• Diagnosis is by exclusion of other causes.
• Pregnancy does not affect the disease, but due to a
lower median platelet count in pregnancy usually the
disease worsen in pregnancy.
Fetal risk

• IgG antiplatelet antibodies can cross the placenta

and cause fetal thrombocytopenia .
• Difficult to predict which fetus will be affected(it
has no relation to maternal platelet count.
• Can lead to antenatal and intrapartum intracranial
hemorrhage.
Maternal risk and mangement

• Bleeding is unlikely if platelet count is>50

• Patient with bleeding or platelet count < 50 should be started
on steroid and if there is no adequate response patient will
be offered IV IG.
• Splenectomy is rarely performed in pregnancy.
• Platelet transfusion is required if rapid response is needed.
• In labor avoid:
• Fetal scalp electrodes
• Fetal blood sampling
• Ventouse delivery
• No fetal benefit from C-section.
Gestational thrombocytopenia

• The incidence of gestational thrombocytopenia is

5-11% of all pregnancies and accounts for more
than 70% of cases of thrombocytopenia in
pregnancy.

• The pathophysiology of gestational

thrombocytopenia is unknown, but 2 main factors
are associated with GT.
• -Accelerated platelet activation is suspected to
occur at placental circulation.
• -Accelerated consumption of platelets is due to
the reduced lifespan of platelets during pregnancy.
• Asymptomatic patient with no history of abnormal bleeding
• Mild thrombocytopenia (counts >70,000/μ L)
• Usually detected incidentally on routine prenatal screening
• No specific diagnostic tests to definitively distinguish
gestational thrombocytopenia from mild ITP
• Usually develops in the mid second to third trimester
• Platelet counts normalize within 1-2 months following delivery.
• No pathological significance for the mother or fetus is noted. No
risk for fetal hemorrhage or bleeding complications is observed