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Hodgkin’s lymphoma

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.

B) nodular lymphocyte predominants –


lymphocytic / histiotic RS cell predominates
AKA popcorn cells, bubbly outline of nucleus
cells, express B cell markers, but does not
express CD 15 and CD 30.
Clinical presentation
Nontender lymph nodes enlargement ( localised )
neck and supraclavicular area 60-80%
mediastinal adenopathy 50%
other ( abdominal, extranodal disease )
systemic symptoms (B symptoms) 30%
fever
night sweats
unexplained weight loss (10% per 6 months)
other symptoms
fatigue, weakness, pruritus
cough , chest pain, shortness of breath,
abdominal pain, bowel disturbances, ascites
bone pain
pathology
 Affected lymph nodes enlarged, mostly
laterocervical lymph nodes.
Macroscopy
Microscopy

Popcorn cell, the reed Sternberg in nodular


lymphocyte predominant. A variant.
Mixed cellularity nodular sclerosing

Lymphocyte
predominant
Management
With appropriate treatment about 85% of patients
with Hodgkin disease are curable

I A,B: radiation therapy


II A : combination chemotherapy + radiotherapy
IIB IIIA,B IVA,B : combination
chemotherapy (+/-)\
radiation therapy
Radiation therapy
mantle field
Inverted Y field
Combination chemotherapy regimen.
ABVD
BEACOPP
Standford v
 ABVD std rx of HL , iv, for evry 2 weeks
for 6/12- 8/12.
 BEACOP rx for stage > ii in europe, c- rate
10-15% higher than ABVD in advanced
states.
 heamatopoetic stem cell transplantations.
Prognosis
Stage and subtype dependent
Favourable in:
Lympocyte predominant
Nodular sclerosing type 1
Pooor in:
Lumphocyte depleted disease stage ii
Nodular sclerosing stage ii
Overall 10 year survival rate for Pts with
HD
1. Nodular lymphocyte predomonat type 1 > 80%
2. Nodular sclerosis 75%
3. Mixed cellularity type 60%
4. Nodular sclerosis type ii 53%
5. Lymphocyte depleted type 5%
with 40% having 5 year survuval rate.
complications
 increase of bleeding tendncies
Infertility in both genders
Predispose to vascular disease and stroke
 secondary malignancy:
tumours in he lungs .
Breast cancers in female patient. Mantle RT.
Vascular diseases
Thyroid dysfunction
 non- hodgkins lymphoma
THE END!!!!!!!!!!

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