part 3 hodgkins lymphoma

HODGKINS NON-HODGKINSOFTEN LOCALIZED LESION

MULTIPLE NODES

ORDERLY SPREAD NONCONTIGUOUS SPREAD

RARE – WALDEYER/ MESENTERIC NODES

COMMON

UNCOMMON EXTRANODAL

COMMON EXTRANODAL

 CLINICAL DIFFERENCES

HODGKINS LYMPHOMA

Reed-Sternberg present◦ Derived from germinal center or post germinal

center B cells Background of Reactive Inflammatory Cells of Various types

Variable Fibrosis * LACK THE MONOMORPHIC APPEARANCE OF OTHER LYMPHOMA

CRITERIA FOR DIAGNOSIS:

Diagnostic Reed-Sternberg Cell

Account for 0.7% of new Ca in USA Arise in a Single or Chains of LN Most common Malignancy in YOUNG ADULTS ( 32y/o)

Curable in most cases May progress to NHL or Leukemia

◦spontaneously or after Tx.

FEATURES:

UnknownEBV plays a role 50% present in R-S cells (Mixed Cellularity)

High incidence Hx of IMGenetic Susceptibility

ETIOLOGY & PATHOGENESIS:

EBV (+) RS EXPRESS LATENT MEMBRANE PROTEIN-1◦PROTEIN ENCODED BY EBV◦HAS TRANSFORMING ACTIVITY◦UPREGULATES NF- Kb

EBV (-) RS cells ◦ACQUIRED MUTATION OF NEGATIVE REGULATOR OF NF-kB

ETIOLOGY & PATHOGENESIS

ACTIVATION OF NF-kB RESCUES DOOMED CELLS FROM APOPTOSIS

RS cell PRODUCE CYTOKINE ACCUM. REACTIVE CELLS SUPPORT GROWTH & SURVIVAL OF TUMOR CELLS◦IL-5, IL-6, IL-13 ,TNF

ETIOLOGY & PATHOGENESIS

PredictableSpread 1st to Contiguous Nodes◦Nodal Disease First Spleen Liver BM Extranodal

SPREAD :

Soft to hard , large Lymph Node Nodularity common in Nodular Sclerosis

Foci of necrosis + Heterogenous c/s except for LYMPHOCYTE PREDOMINANCE

Matted L.N. in Advanced cases

GROSS:

PAINLESS LYMPHADENOPATHY PAIN INVOLVED L.N. W/ ALCOHOL

◦Paraneoplastic Symptom CUTANEOUS ANERGY

◦Depressed Cellular immunity IMMUNE DYSFUNCTION

◦ UNKNOWN, PERSIST AFTER Tx. INCREASED RISK FOR 2ND CANCERS

◦AML, BREAST Ca GASTRIC Ca Limited Disease is Cured w/ Local RadioTx

CLINICAL COURSE & PROGNOSIS:

STAGE CRITERIA

I In one lymph node only

II In 2 or more LN on same side of the diaphragm

III In the Lymph nodes, Spleen, or Both AND on Both sides of the diaphragm

1 Above the renal vessels (eg. Spleen, splenic, hilar, celiac, portal nodes

2 In the lower abdomen ( periaortic, pelvic, or imguinal nodes )

IV Extranodal involvement ( eg. Bone marrow, Lung, Liver )

ANN ARBOR STAGING OF NHL & HL

A – absence of Systemic manifestationB – presence of Systemic manifestationNIGHT SWEATS, FEVER, WEIGHT LOSS

Stage I or II

Stage III or IV

Systemic s/s

Usually absent

Usually present

Type Nodular sclerosis

MC or LD

5 year disease free

90% 60-70%

CLINICAL COURSE & PROGNOSIS:

Systemic manifestation:NIGHT SWEATS, FEVER, WEIGHT LOSS

Tumor stage is most important Prognostic Variable◦ Not Histologic Type◦ R/T to current Tx protocols

Long term survivors of ChemoTx and RadioTx◦ Increased risk fro secondary Ca◦ Breast Ca common in:

Females Tx w/ RadioTx to chest during adolescence◦ AML/ Myelodysplastic syndrome common

ChemoTx

CLINICAL COURSE & PROGNOSIS:

NODULAR SCLEROSIS

MOST COMMON 65-70%MALE = FEMALESADOLESCENT & YOUNG ADULTS

EXCELLENT PROGNOSIS

Features:

Stage 1 or 2 LOWER CERVICAL , SUPRACLAVICULAR & MEDIASTINAL L.N.

RARELY ASSOC. EBV

Clinical Maniifestation

LACUNAR TYPE AbundantCOLLAGEN BAND NODULARPOLYMORPHOUS BACKGROUND

DXTIC RS CELLS LESS FREQUENT

Morphology

Morphology

Lacunar Type of RS cell

LACUNAR RS

Mixed Cellularity

20-25 % MALES > FEMALES STRONGLY ASSOC. W/ EBV OLDER & YOUNG ADULTS ( BIPHASIC ) > 50% PRESENT AS STAGE 3-4

accompanied by SYSTEMIC SYMPTOMS

Prognosis Very Good

MIXED CELLULARITY

Microscopic

FREQUENTLY SEEMONONUCLEAR & DXTIC RS CELLS

DXTIC RS CELLSAre numerousMONONUCLEAR

LYMPHOCYTE RICH TYPE

 UNCOMMON M>F OLDER ADULTS FREQUENT MONONUCLEAR AND DXTIC R-S CELLS◦REACTIVE T LYMPHOS PREDOMINATES

40% EBV ASSOC. PROGNOSIS EXCELLENT

LYMPHOCYTE RICH TYPE

LYMPHOCYTE - PREDOMINANCE

5% OF CASES , UNCOMMONMALES > FEMALES ( < 35 y/o )

◦Asymptomatic YOUNG MALES WITH CERVICAL OR AXILLARY LYMPHADENOPATHY

◦MEDIASTINAL INVOLVEMENT IS RARENOT ASSOC. W/ EBVPROGNOSIS EXCELLENT

LYMPHOCYTE - PREDOMINANCE

NODULAR PATTERN DUE TO EXPANDED B-CELL FOLLICLES◦POPULATED BY L&H CELLS AND

REACTIVE B-CELL◦DXTIC RS DIFFICULT TO FIND

3-5% PROGRESS DIFFUSE LARGE CELL  

LYMPHOCYTE - PREDOMINANCE:

Microscopic – Difficult to find diagnostic RS cells

POPCORN RS CELLSL & H variants

NUMEROUS MATURELYMPHOCYTES

<5 % LEAST COMMON OFTEN ASSOC. EBV COMMON

* OLDER PTS.* HIV * NON-INDUSTRIALIZED COUNTRIES

SYSTEMIC SYMPTOMS PRESENT AS ADVANCED STAGE PROGNOSIS LESS FAVORABLE

LYMPHOCYTE DEPLETION TYPE :