Week 11: Lymphoproliferative Disorders Multiple myeloma Multiple myeloma Plasma cell Plasma cell...

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Week 11: Lymphoproliferative Disorders Multiple myelomaMultiple myeloma Plasma cellPlasma cell Monoclonal Monoclonal

gammopathygammopathy Bence-Jones proteinBence-Jones protein Protein ELPProtein ELP Hairy cell leukemiaHairy cell leukemia TRAPTRAP

LymphomaLymphoma Hodgkin’s diseaseHodgkin’s disease Reed-Sternberg cellReed-Sternberg cell Mycosis fungoidesMycosis fungoides Sezary cellSezary cell

Multiple Myeloma

Plasma cell leukemiaPlasma cell leukemia Monoclonal gammopathy ELP pattern with Monoclonal gammopathy ELP pattern with

sharp sharp region spike region spike High ESR, rouleaux, Bence-Jones protein, High ESR, rouleaux, Bence-Jones protein,

Platelet dysfunctionPlatelet dysfunction Osteolytic (punched out) bone lesionsOsteolytic (punched out) bone lesions

Myeloma cells in peritoneal fluid

Rouleaux

Other Plasma Cell Disorders

Waldenstrom’s macroglobulinemiaWaldenstrom’s macroglobulinemia IgMIgM Soft tissue involvementSoft tissue involvement CD 20 (B cell) but no CD 21 (mature B) CD 20 (B cell) but no CD 21 (mature B)

or PC 1 (plasma cell)or PC 1 (plasma cell) Heavy chain diseaseHeavy chain disease

Plasmacytoid lymphocytePlasmacytoid lymphocyte

Unusual plasma cells

Osteoblasts

Hairy Cell Leukemia

Leukemic reticuloendotheliosisLeukemic reticuloendotheliosis Probably B cell originProbably B cell origin CD 19, 20, 22, 24 (B cell), 25, 11c CD 19, 20, 22, 24 (B cell), 25, 11c

(granulocyte), sIg, some CD 5 (pan-T)(granulocyte), sIg, some CD 5 (pan-T) Tartrate resistant acid phosphatase (TRAP)Tartrate resistant acid phosphatase (TRAP)

Hairy CellLeukemia

LRE: Ruptured Spleen

Acid Phosphatase

Naphthol AS-BI phosphate ACP in acid pH > Free naphthol AS-BI + FastGarnet GBC

Magnesium > Maroon deposits

Tartrate Resistant ACP: TRAP + Tartrate

Non-Hodgkin’s Lymphoma Lymphocytes with clefted nucleiLymphocytes with clefted nuclei Sezary cells in mycosis fungoides have many cleftsSezary cells in mycosis fungoides have many clefts Rappaport classification based on morphology and Rappaport classification based on morphology and

differentiationdifferentiation Lukes-Collin classification with cell types identifiedLukes-Collin classification with cell types identified International Working Formulation with tumor International Working Formulation with tumor

aggressiveness and histologyaggressiveness and histology Revised European/American Lymphoma (REAL) Revised European/American Lymphoma (REAL)

classification is most recentclassification is most recent

Lymphoma Cells

Bone Marrow: Focal Involvement

WHO Classification of Acute Lymphoproliferative Syndromes

Precursor B Lymphoblastic Precursor B Lymphoblastic Leukemia/Lymphoma (ALL/LBL) -- ALL Leukemia/Lymphoma (ALL/LBL) -- ALL in children (80-85% of childhood ALL); in children (80-85% of childhood ALL); LBL in young adults and rare; FAB L1 or LBL in young adults and rare; FAB L1 or L2 blast morphologyL2 blast morphology

Precursor T ALL/LBL -- 15% of childhood Precursor T ALL/LBL -- 15% of childhood ALL and 25% of adult ALLALL and 25% of adult ALL

Burkitt Leukemia/Lymphoma (FAB L3)Burkitt Leukemia/Lymphoma (FAB L3)

Mycosis Fungoides: T-cell Lymphoma of skin

Skin Biopsy

Skin Imprint

Sezary Syndrome

PAS Positive: Sezary Syndrome

Hodgkin’s Lymphoma

Reed-Sternberg cell (RS): lobated nuclei, Reed-Sternberg cell (RS): lobated nuclei, mutinucleate variants, lacunar cells, mutinucleate variants, lacunar cells, lymphocytic-histiocytic cell (LH)lymphocytic-histiocytic cell (LH)

RS: has CD 15 (granulo/mono) and CD 30 (B RS: has CD 15 (granulo/mono) and CD 30 (B cell)cell)

Lymphocyte predominant type CD 20 (B cell)Lymphocyte predominant type CD 20 (B cell) Clinical staging I through IV according to Clinical staging I through IV according to

number and location of tumorsnumber and location of tumors

Reed-Sternberg Cell