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MBIMBI JOSEPH
MBChB LEVE III
25th may 2011
Definition:
neoplastic disorder with development of specific
infiltrate containing pathologic Reed-Sternberg
cells. It usually arises in lymph nodes and
spreads to contiguous groups.
Named after, Thomas hodgkin, 1834.
First to describe the abnormalities in
lymphatic system.
reed- stern berg cells, after 2 workers who
identidied its significance.
causes
unknown / multifactorial.
association with Epstein bar virus infection
Generation of B cells with abnormal ig.
Risk factors
• male gender
•Age 15-30 and those over 55 yrs
•History of infenction with EBV
•Compromised immunity e.g in Hiv/AIDS
Epidemiology
2-4 cases per 100000 population / year
- bimodal age distribution :
15-35 years and above 50 years
- male predominance M:F = 1,7:1
Pathogenesis
immunoglobulin mutation of the B cells.
production of pools, of B cells with
crippled( abnormal) genes.
formation of Rscs.
possess somatic mutation and contains high
levels of transcrip. Factor B and nuclear factor B.
Somatic mutation, NF-KB inhibition, thus, KB
function is abolished.
Failure to inhibit, NF-KB. Results in high levels of
transcription factor, NF-KB.
cause, excessive production of B cells and
protection of B cells fro pro-apoptotic signals, B cells
proliferation.
High numbers of B cells.
reed stern berg cells
secretion of il-5 , interleukin-13 and transforming
growth factor B.
IL-13, autocrine stimlstin towards RSCs
Il-5, causes activation and attraction of eosinophils,
inflammatory cells which attracts other
inflammatory cells, Accumulates in B cells.
Results in pathologic enlargement of the
lymphnodes.
Staging classification
clical staging uses, Ann arbor classification system
stage I: cancer located in a single region,
one lymph node and the surrounding area
no outward symptoms
Stage II:
one or more lymph nodes involved, same
side of the diaphragm
Stage III:
lymph nodes on both sides of the
diaphragm are involved.
Stage III:
lymph nodes on both sides of the
diaphragm are involved.
Stage IV:
disseminated involvement of extra
lymphatic organs, eg
liver, lungs and bone marrow.
The letters A and B are appended to the various
stages.
Indicates presence or absence of constitutional
symptoms.
presence of B symptoms ( B)
Absence of B symptoms (A)
Pathological classification
•Rye classification, adopted in 1966. ( similar to WHO)
divides classical HL into 4 subtypes, based on the Rs
cell morphology and reactive cell infiltrate seen in
lymphnode biopsy.
a) Classical HL
Lymphocyte rich – rare subtype, favorable
prognosis
Nodular sclerosing type – common sub/t, large
tumour nodules. Shows scattered lacunar RS cells.
Mixed cellularity – common, numerous clasic RS
cells, admixed with inflammatory cells. Without
sclerosis.
Lymphocyte depleted – rare s/t, large numbers
Of pleomorphic reed sternberg cells.
Lymphocyte
predominant
Management
With appropriate treatment about 85% of patients
with Hodgkin disease are curable