part 3 hodgkins lymphoma
DESCRIPTION
TRANSCRIPT
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HODGKINS NON-HODGKINSOFTEN LOCALIZED LESION
MULTIPLE NODES
ORDERLY SPREAD NONCONTIGUOUS SPREAD
RARE – WALDEYER/ MESENTERIC NODES
COMMON
UNCOMMON EXTRANODAL
COMMON EXTRANODAL
CLINICAL DIFFERENCES
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HODGKINS LYMPHOMA
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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:
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Diagnostic Reed-Sternberg Cell
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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:
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UnknownEBV plays a role 50% present in R-S cells (Mixed Cellularity)
High incidence Hx of IMGenetic Susceptibility
ETIOLOGY & PATHOGENESIS:
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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
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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
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PredictableSpread 1st to Contiguous Nodes◦Nodal Disease First Spleen Liver BM Extranodal
SPREAD :
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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:
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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:
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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
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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
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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:
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NODULAR SCLEROSIS
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MOST COMMON 65-70%MALE = FEMALESADOLESCENT & YOUNG ADULTS
EXCELLENT PROGNOSIS
Features:
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Stage 1 or 2 LOWER CERVICAL , SUPRACLAVICULAR & MEDIASTINAL L.N.
RARELY ASSOC. EBV
Clinical Maniifestation
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LACUNAR TYPE AbundantCOLLAGEN BAND NODULARPOLYMORPHOUS BACKGROUND
DXTIC RS CELLS LESS FREQUENT
Morphology
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Morphology
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Lacunar Type of RS cell
LACUNAR RS
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Mixed Cellularity
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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
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Microscopic
FREQUENTLY SEEMONONUCLEAR & DXTIC RS CELLS
DXTIC RS CELLSAre numerousMONONUCLEAR
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LYMPHOCYTE RICH TYPE
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UNCOMMON M>F OLDER ADULTS FREQUENT MONONUCLEAR AND DXTIC R-S CELLS◦REACTIVE T LYMPHOS PREDOMINATES
40% EBV ASSOC. PROGNOSIS EXCELLENT
LYMPHOCYTE RICH TYPE
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LYMPHOCYTE - PREDOMINANCE
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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
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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:
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Microscopic – Difficult to find diagnostic RS cells
POPCORN RS CELLSL & H variants
NUMEROUS MATURELYMPHOCYTES
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<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 :