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HODGKIN’S LYMPHOMA Introductio n Lymphocyte Predominanc e Classical HL Nodular Sclerosis Mixed cellulari ty Lymphocy te rich Lymphocy te depletion Special morpholog y HODGKIN’S LYMPHOMA Raid Jastania Jan 22/03

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Page 1: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

HODGKIN’S LYMPHOMA

Raid JastaniaJan 22/03

Page 2: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Definition

• Presence of Reed-Sternberg cells and their variants in

an appropriate background of inflammatory cells

• 2 biologically and clinically distinct entities:– Nodular lymphocyte predominance

HL– Classical HL

• Evidence of B-cell origin• In Caucasians

– HL: 25-40% of all lymphomas– Peak in 2nd-3rd decade, another peak in 6th

• In Orientals – HL: 5-10% of all Lymphomas

Page 3: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Classification

• REAL/WHO Classification:– Nodular Lymphocyte Predominance – Calssical:

• Lymphocyte-rich• Mixed cellularity• Nodular Sclerosis (50-80%)• Lymphocyte depletion (rare)

• Not classifiable cases• Relation to Non Hodgkin’s Lymphomas

– HL and Follicular lymphoma– CLL can progress to HL– Difficult distinction , diffuse large B-

cell, anaplastic large cell.

Page 4: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Staging

• Ann Arbor Staging system

I single LN region (I) or single extralymphatics (I E)

II2 or mare LN regions in same side of the diaphragm (II) or localized extralymphatics and LN region on the same side of the diaphrag (II E)

IIILN regions on both sides of the diaphragm (III) , with localized area of extralymphatics (III E), or the Spleen (III S) or both (III ES)

IV Diffuse one or more extralymphatics

Page 5: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Nodular Lymphocyte Predominance HL

• 5% Of all HL• Children , young and middle aged• M:F 3:1• Solitary enlarged peripheral LN,

neck , groin and axilla• Mediastinal LN very rare• Relapse is common 19-32%• Median time for relapse 4 y• Stage I disease excellent 95%• Stage VI 24%• Treatment : ? Surgery for I, limited

radiation.

Page 6: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Pathology

• Nodal architecture is obliterated• Multiple, crowded, large, dark-

staining nodules of small lymphocyte

• Eosinophils and plasma cells scanty• Lympho-histiocytic ( L&H ) cells• CD20+, bcl-6+, and EMA+• CD30-• CD3+ T-cells form rosettes around

the L&H cells• PCR of microdissected L&H cells

showed clonal rearrangement of Ig genes (B-cell nature)

Page 7: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Page 8: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Page 9: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Pathology

• Trasformation to Large cell Lymphoma:– 3-4% transform to diffuse large cell

lymphoma– Localized disease, favorable

prognosis– Overgrowth of L&H cells or NHL

• Diffuse Lymphocyte predominance HL:– Rare– Minor nodular component on reticulin

stain– More likely to be stage III or IV– Difficult to differentiate with T cell

rich B cell lymphoma and lymphocyte rich classical HL

Page 10: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Classical Hodgkin’s Lymphoma

• Presents with lymphadenopathy• Extranodal disease is uncommon• Spread:

– Contiguous pattern– Similar to carcinomas– To spleen following para aortic LN– Then blood borne spread

• The neoplastic Reed-Sternberg cells– Diagnostic R-S cells– Mononuclear R-S cells– Lacunar cells– Pleomorphic R-S cells– Mummified cells

Page 11: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Reed-Sternberg Cells

• Histogenesis controversial– Histiocyte, interdigitating dendritic

cells, Follicular dendritic cells, myeloid cells and lymphocyte

– B-cell lineage• B-cell specific activator protein and

sometimes CD20• Ig gene rearragement

– Immuno:• CD45RB (LCA) –• CD30+, CD15+(para nuclear globules with

or without membranous staining)• B-cell markers , like CD20 occasionally

– Genetics:• Ig gene rearrangement in single neoplastic

cell microdissection

Page 12: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Page 13: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Classical Hodgkin’s Lymphoma

• Association with EBV:– 40-50% of cases in the West,

and 100% in underdeveloped countries and patients with AIDS

– MC type 60%, NS type 35%– Stronger association in the

head and neck region and at the extremes of life

Page 14: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Nodular Sclerosis

• 5-year survival 80%• Pathology:

– The capsule is thickened with multiple fibrous bands and nodularity

– Lacunar cells with small lymphocytes, plasma cells, eosinophils, neutrophils and histiocytes

– Syncytial variant of NSHL– Necrosis, geographic– The cellular phase: lack fibrous bands

• Subclassification/grading:– British National Lymphoma Investigation (BNLI)

• Grade I (54% 5-year survival)• Grade II (37% 5-year survival ):

– Any of : > 25% of cellular nodule contain numerous bizarre R-S cells

– >25% of cellular nodules show lymphocyte depletion

– >80% of cellular nodules show a fibrohistiocytic pattern

Page 15: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Page 16: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Page 17: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Mixed Cellularity

• Cervical and supraclavicular LN

• Complete remission in 70%• Pathology:

– Background of small lymphocytes, plasma cells, eosinophils, and histiocytes

– Mononuclear and diagnostic R-S cells

– Focal necrosis, secondary fibrosis

Page 18: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Page 19: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Lymphocyte-rich HL

• Often early stage disease• Uncommon in the

mediastinum• Worse survival than N-LPHL• 5-year survival 80%• Pathology:

– Mononuclear and rare diagnostic R-S cells

– Not L&H cells

Page 20: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Page 21: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Lymphocyte depletion HL

• Prognosis poor , 80% dying within 3 years

• Pathology:– 2 recognized types

• Diffuse fibrosis type- rare R-S cells• Reticular type- abundant

diagnostic and pleomorphic R-S cells

Page 22: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Page 23: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Anaplastic Large Cell Lymphoma, Hodgkin’s-like

• Young adult with LN involvement and large mediastinal mass

• Patients do poorly with therapy for HL, but good with aggressive chemo for NHL

• Pathology:– Cohesive growth of large bizarre

neoplastic cells– Architecture resemble HL, capsular

thickening , nodular growth, sclerosis band

– Admixed reactive cells– Most called nodular sclerosis, syncytial,

NS2 or LDHL , reticular type– Immuno: like ALCL

• CD30+, LCA+/-, EMA+/-, CD15+/-, CD3-/+

Page 24: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Special morphologic features of classical HL

• Interfollicular HL– Can be in any type. Inter follicular growth

• Foamy Histiocytes in HL– Confusion with xanthogranulomas

• Epithelioid histiocytes– Mimic the pattern of Lennert’s lymphoma

• HL in monocytoid B-cell clusters• Treatment altered HL• In the Spleen:

– The number of nodules should be counted– First affect the pulp, in a periarteriolar location or

in the marginal zone• In the Liver:

– First in the portal areas• In the marrow:

– Focal or diffuse fibrosis with non specific infiltrates

– Marrow eosinophilia

Page 25: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

Prognostic Factors in Classical HL

• Stage• Histologic type• Sex and age: male and old , worse

prognosis• B symptoms• Total tumor burden• Response to treatment• Vascular invasion• Lack of CD15, expression of CD20

Page 26: HODGKIN ’ S LYMPHOMA Introduction Lymphocyte Predominance Classical HL  Nodular Sclerosis  Mixed cellularity  Lymphocyte rich  Lymphocyte depletion

HODGKIN’S LYMPHOMA

Introduction

Lymphocyte Predominance

Classical HLNodular Sclerosis

Mixed cellularity

Lymphocyte rich

Lymphocyte depletion

Special morphology

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