Disease
What is it?
Lymphoma
Proliferation of
lymphoid cells
more
Types
Hodgkin's
commonly B,
but can be T
NonHodgkin'
s
Myeloma
Monoclonal
proliferatio
n of B cells
Pathology
Aetiology
Symptoms
Diagnosis
Prognosis
Treatment
Reed - S tern b er y
cells giant multinucleate
malignant B cells
present on
histology. They
express no
antibodies
Slightly more
common in men.
Peak incidence in
Enlarged rubbery
lymph nodes
particularly in neck
Alcohol induced
pain in lymph
nodes
Itch,
fatigue,
anorexia
Bsymptoms fever,
night
sweats,
weight loss
Lymphadenopathy, B
Hepatosplenomegaly,
Lymphocytonpaenia,
Eosinophililia,
Raised LDH, Lymph
node biopsy for
histology fine
needle aspiration is
useless as it only give
cytology
Worse with B
symptoms and raised
LDH. Treatment is
often curative. Median
survival is longer than
5 years even in those
with
severe disease.
Irradiation with/without
chemo. Chemo for
more severe cases.
Disease is classified
using the Ann Arbor
scale. Survival rates:
All the investigations
of HL,as well as bone
marrow aspiration,
immunophenotyping
. Spread in noncontiguous
(multicentric)
Varies between
patients. if no
symptoms; monitor.
Radiotherapy can cure
worse prognosis, but
localised disease.
also a higher chance of Chemo used in
cure. Wo r s e i f B
widespread
s y m p t o m s and raised disease.Survival
LDH
rates:
grade:
50%
Incurable
Can beLow
divided
into
Malignant
tumour of
lymphoid cells
without RS cells
present
Infiltration
of
bone marrow by
lots of abnormal
B cells. leads to
pancytopaenia.
Lytic lesions
in bone.
3rdeand 6th-8th
decades.
EBV, CMV infection.
Genetic factors.
Incidence is stable
7th decade
onwards Incidence
is rising.
High incidence in
developing
countries due to
Burkitt's lymphoma
a T cell
lymphoma
caused
Over 60's. Slight
male predominance.
More common in
black Africans.
Genetic factors
ALL-acute
lymphoblasti
c leukaemia
AMLacute
myeloid
leukaemia
Basically an
acquired
genetic defect
that means blast
cells do not
mature, and
they also avoid
apoptosis, thus
proliferate
wildly.
compression
symptoms (extranodal
symptoms),
Pancytopaenia
often present
Hyp er vis co sity,
rec u rren t in f ec t io n s
due to reduced level
of normal Ig, usually in
resp.tract.
Bone pain. Often
asymptomatic
Renal impairment,
hypercalcaemia,
spinal cord
compression
Leuka emia
symptoms,
Unknown many
factors involved,
possibly
radiation,
benzene, genetic
susceptibility,
infection by
certain viruses
(HL1V-1)
FBC, ESR and CRP,
blood film, LDH,
calcium
(normal or raised),
total protein, urine
immunofixation
(Bence- Jones
proteins), DEXA scan
disease discovered on
routine bloods
Bone marrow
aspiration shows
loads of plasma cells.
Children! Anaemia,
bleeding, infection
(fever), bone pain,
petichae,
lymphadenopathy,
>20% blast cells in
Adults, 2/3 are
>60
Anaemia,
bleeding,
infection,
bone
pain
bone marrow,
raised WCC, low Fib,
low platelets
Hepatosplenomegaly,
testicular
enlargement
chronic
lymphocytic
leukaemia
Usually from B
The most common
WCC 20-40,
lymphocytes.
leukaemia.
hepatosplenomegaly
CIVIL -
Philadelphia
chronic
myeloid
leukaemia
chromosome
Peak age 40-60.
Slightly higher
incidence in
men.
CLL
this is the
abnormality in
97%ofcases.
Slow
progression
SOB (anaemia),
abdominal discomfort
(splenomegaly),
weight loss, fever
and sweats (B
symptoms),
lymphadenopathy,
gout, retinal
haemorrhage,
cerebral disorders.
WCC > 100,
hepatosplenomegaly,
high neutrophils,
blood film shows cells
at all stages of
development, high
uric acid
production (renal
failure, gout) due to
high cell turnover
Spread is
contiguous (only to
adjacent
Not generally
curable. Mild disease
will relapse/remit.
Sever disease has a
treatments just
induce remission.
Death from
infection, renal
failure, haemorrhage.
Median survival is 5
years, but 1/3 of
patients will die
within 3 months of
diagnosis.
Very poor without
treatment. Younger
patien ts mo re likely
to be cured. For ALL in
childhood, 80% will
have CR after 5 years.
For ALL, all under 60's
normally given
curative treatment, and
2/3's will not
relapse.
Media survival is 10
years.This is related
to the severity of the
symptoms at
presentation
Disease progresses
from crhonic stage
>>
aggressive
phase
>>
blast
crisis.
The
blast
crisis is essentially
terminal.
Stage i 95% @
5yrs Stage iV 40%
@ 5yrs
supportive (i.e. treating
symptoms and
comp licatio ns ) and
specific (i.e. treating
the disease, with
bisphosphonates). True
remission is never
maintained, and
patients will relapse
when treatment is
stopped.
if cure appears
possible: Remission
induced by chemo,
then bone marrow
transplantation (if
suitable HLA match)
then maintenance
therapy. This will
usually give 'complete
remission, which in
many is curative, but
some still relapse. Last
612 months in total.
Risk of death.
,
iif no cure possible,
then supportive (i.e
palliative)
care
is
I mitinab - prevents
the action of the
abnormal p ro tein
p ro du ced b y the Ph
gene.
Stem cell
transplantation is
curative in 70% of
casesbut
is
not
