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Hodgkins Lymphoma by Dr. Anum Usman

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Hodgkin

Lymphoma
LEARNING OBJECTIVES

Define Hodgkin lymphoma

Classify Hodgkin lymphoma

Describe pathogenesis of Hodgkin lymphoma

Describe morphology of Reed Sternberg Cell

Describe morphology of each type of Hodgkin lymphoma

Enlist clinical features of Hodgkin lymphoma

Describe staging of Hodgkin lymphoma

Tabulate the differences between Hodgkin & Non Hodgkin lymphoma


Theme / Scenario
 A 25 year old lady
1 month of evening
rise of temperature,
night sweats and
noticed a lump in her
neck
On examination
pallor, generalized
lymphadenopathy
Normal Structure LN
To recognize
lymph node
pathology,
one has to be
familiar with
normal lymph
node
anatomy
LN usually not palpable. Sometimes those in the S/M,
axillary or inguinal regions may be palpable
in healthy people.

Lymphadenopathy :
Nodes that are abnormal in either size, consistency
or number.

Lymphadenopathy may be:

-- Generalized" if lymph nodes are enlarged in two or


more noncontiguous areas

--Localized" if only one area is involved.


Lymphadenopathy
 Infections
 Reactive hyperplasia
 Sarcoidosis
 Metastatic tumors
 Primary Neoplasms---Lymphomas.
Lymphomas are all malignant
 Non-Hodgkin lymphomas-NHL
 Hodgkin lymphomas- HL
Lymphoma
 Clonal malignant disorders that are derived
from lymphoid cells: either precursor or
mature T-cell or B-cell

 Majority are of B- cell origin

Divided into 2 main types :


1. Hodgkin lymphoma
2. Non – Hodgkin lymphoma
Hodgkin’s Disease

 It was named after Thomas Hodgkin (1832).


 Dorothy Reed and Carl Sternberg first
described the malignant cells of Hodgkin’s
lymphoma called Reed Stenberg cells

 Histologically & clinically a distinct malignant


disease
 Predominantly, B-cell disease
RS Cell & Variants
RS Cell
Reed–Sternberg
cells are very large cell
(15–45 microns) with
enormous multilobate
or have bilobed nucleus
with prominent
eosinophilic inclusion-
like nucleoli surrounded
by clear zone (thus
resembling an "owl's
eye" appearance) and
abundant cytoplasm.
Hodgkin Lymphoma
 Incidence:
- 2-4 cases per 100000 population /
year
- bimodal age distribution :
15-30 years and above 55 years
- male predominance M:F = 1.7:1
- rare in children younger than 10 years
Etiology

 The exact cause of Hodgkin lymphoma is not known

 Viruses:
The Epstein-Barr virus has been implicated as a
cause of Hodgkin lymphoma. The presence of the
genome of this virus is seen in 20%-80% of
Hodgkin lymphoma tumors.
Clinical features
 Bimodal age distribution :
 young adults ( 15-30 yrs) & elderly (> 55yrs)
May occur at any age
 M>F
 Lymphadenopathy:
 most often cervical region
 asymmetrical
 non-tender
 elastic character on palpation ( rubbery)
 not adherent to skin
 fluctuate in size
 Contiguous spread via the lymphatic chain
eg. involvement of abdominal & thoracic
LNs

 Hepato-splenomegaly
Clinical Presentation
 Non tender lymph nodes enlargement ( localised )
 neck and supraclavicular area 60-80%
 mediastinal adenopathy 50%
 other ( abdominal, extranodal disease )
 Other symptoms
 fatigue, weakness, pruritus
 cough, chest pain, shortness of breath, vena cava
syndrome
 abdominal pain, bowel disturbances, ascites
 bone pain
 Constitutional symptoms ( B symptoms )
 Night sweats,
 sustained fever > 38 degree C,
 loss of weight >10% of body weight in 6 months
 Fever sometimes cyclical (‘Pel-Ebstein fever’)
 Pallor
Classification: REAL/ WHO
 Classic:
 Nodular Sclerosis HL
 Lymhocyte Rich HL
 Mixed Cellularity HL
 Lymhocyte depleted HL

 Non-Classic
 Nodular Lymphocyte predominant (NLPHL)
MORPHOLOGY of
HODGKIN LYMPHOMA
HL : Nodular Sclerosis

 Most common subtype in developed countries


 Accounts for 50-75% of all cases of HL
 Accounts for 40% of younger patients and 70%
of adolescents with HL
 Often involves lower cervical, supraclavicular,
and mediastinal nodes
 CD 15 and CD 30 positive; CD 45 Negative
Lymph node: Nodular sclerosis HL
HL: NS

Thickened lymph node


capsule, organized
collagenous bands forming
circumscribed nodules

Lacunar cells are seen


HL : NS

Large single multilobe irregular nuclei with


multiple small nucleoli,
cytoplasm retracts when
fixed with formalin so
nuclei gives the
appearance of cells
that lie in empty spaces
(lacune).
Mixed Cellularity HL
 Accounts for 15-30% of all cases of HL
 Common in older people (above 50 years)
 Most frequent subtype in HIV patients
 Frequently presents with advanced disease
and extranodal extension at diagnosis
HL : MC

Many Reed-
Sternberg cells are
present

LN has inflammatory
background with
lymphocytes, plasma
cells, eosinophils,
histiocytes, and
malignant reticular
cells
HL : MC
Immuno staining: CD-30
Lymphocyte Rich - HL
 Uncommon form of HL
 Lymph nodes are diffusely effaced with
reactive lymphocytes.
 Mononuclear hodgkin cells and classical
RS cells are present.
 CD15 and CD 30 are positive
 About 40 % are EBV associated.
 Very good to excellent prognosis
LR - HL
Lymphocyte Depletion - HL
 Rare in children, predominantly in elderly.
 Many bizarre, malignant reticular cells
 Many RS cells
 Few lymphocytes
 Diffuse fibrosis and necrosis
 Often presents with widespread disease
with bone and bone marrow involvement
LD - HL
Nodular LP HL
 <5% of Hodgkin lymphomas
 Accounts for 10-15% of children with HD
 Often presents as localized disease; cervical,
axillary or mediastinal
 More common in males (2:1)
 LN structure partially or completely destroyed
 Positive for CD20, CD 45•
Negative for CD15, CD 30.
HL: NLP

Reed-Sternberg cells
are rare.

“L&H” cells or
“Popcorn” cells are
seen
(delicate puffy
nucleus resembling
popcorn cell)
Investigations
 CBP :
Anemia ( normochromic / normocytic),
eosinophilia, neutrophilia, lymphopenia
 ESR -raised
 LFT- (liver infil / obs at porta hepatis)
 RFT- prior to treatment
 LDH - adverse prognosis
 CXR- mediastinal mass
 CT thorax / abdomen / pelvis-for staging
 Other: Gallium scan, PET, Lymphangiography ,
Laporotomy
 LN FNAC / biopsy :

 Malignant REED-STERNBERG ( RS) Cell:


Bi-nucleate cell with a prominent nucleolus.

 Reactive background of eosinophils,


lymphocytes, plasma cells

 Fibrous tissue
HL - FNAC
Staging
 Stage I : Involvement of single LN region (I) or extra
lymphatic site (IAE )
 Stage II : Two or more LN regions involved (II) or an
extra lymphatic site and lymph node regions on the
same side of diaphragm
 Stage III : Involvement of lymph node regions on both
sides of diaphragm, with (IIIE) or without (III) localized
extra lymphatic involvement or involvement of the
spleen (IIS) or both (IISE)
 Stage IV : Involvement outside LN areas (Liver, bone
marrow)

A : Absence of ‘B’ symptoms


B : B symptoms present
Treatment

 Radiotherapy
 Chemotherapy
 Bone MarrowTransplant / Stem cell
Transplant
 Antibody treatment: Rituximab target CD-20
 Supportive
Hodgkin Lymphoma(HL) Non Hodgkin Lymphoma (NHL)
Dr Thomas Hodgkin---1832
1 Malignancy of lymphocytes Malignancy of lymphoid cells
2 Usually lymph nodes Lymph nodes & other organs
3 Disease of B-lymphocytes Can be B or T Lymphocytes
4 About 10 % of lymphomas More than 90 % of Lymphomas
5 Age( 10-20 and more than 50 yrs More than 60 yrs
6 Homogenous group(5 types) More than 60 types
7 Painless lymphadenopathy Painless lymphadenopathy in
usually upper part of body throughout body
8 Progress in orderly fashion and Diagnosed at advanced stage
diagnosed at early stage

9 HL—treatable----5 yr survival Low survival rate (30 – 40 %)


more than 90 %
Quiz Time
B Symptoms

Viral association

Diagnostic Cell in HL

The Most Common Presentation


The most common presentation
of Hodgkin’s

Painless LAD
Cervical/thoracic
B symptoms include…
Fever > 38 C on 3 consecutive days

*Drenching night sweats

*Unexplained 10% wt loss

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