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Lymphomas + Multiple Choice Questions By Mojgan Talebian 1

Lymphomas+ Multiple Choice Questions

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Lymphomas

Lymphomas+ Multiple Choice QuestionsBy Mojgan Talebian1

DefinitionSolid tumors of the immune systemArise from cells of lymphoid tissues; lymphocytes, histiocytes, and reticulum cellsAnywhere in the immune system, but usually in lymph nodes, spleen, marrow, and tonsilsLocation and the behavior of lymphomas separate them from leukemiasThe malignancy starts and restricted to lymphoid tissues and progress to involve the BM and appears in PB, at this stage it may be named, lymphosarcoma cell leukemia2

Classifications of Lymphomas

A heterogeneous disorder

Hodgkin lymphoma or Hodgkin disease Lymphocytic lymphomas or referred to as non-Hodgkins lymphoma (NHLs), most cases are fatal

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Staging of LymphomasBased on the following categoriesPhysical examination, measurement of the enlarged lymph node/spleenBone marrow examination, Aspirate/biopsyRadiology studies/CT scansHematology studiesChemistry tests; pr electrophoresis, kidney and liver function testsLapratomy to determine the spread of the diseaseLymph node biopsy/histological features

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Ann arbor stagingStage Ionly 1 lymph node area or lymphoid organ (I)1 area of a single organ outside of the lymph system (IE)Stage IIon the same side of the diaphragm(above or below)in 2 or more groups of lymph nodes ( II)extends from a single group of lymph node(s) into a nearby organ (IIE)Stage IIIin lymph node areas on both sides of (above and below) the diaphragmIt may also have spread into an area or organ next to the lymph nodes (IIIE), into the spleen (IIIS), or both (IIISE).Stage IVspread outside the lymph system into an organ that is not right next to an involved node.spread to the bone marrow(IVM ) , liver ( IVL), brain or spinal cord, or the pleura (thin lining of the lungs

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Staging SubscriptsA, asymptomatic B, presence of any or all of the following signs:Greater than 10% of total body weight loss in the last 6 monthsUnexplained fever above 38 C, night sweats6

Hodgkin Lymphoma

A malignant lymphoma with clonal proliferation Unknown etiology majority of cases both classical HL(CHL) and nodular lymphocyte predominant HL(NLPHL) are clonal proliferation of B cellsmay caused by infectious transmission in the host:Epidemiologic evidence Presence of two peaks period of onsetRecovery of the EB Virus DNA from lesions of approximately 80% of cases of HLcertain RNA tumor virusesCMV, herpes virus 6Presence of Reed-Sternberg cells accompanied with H-cells (malignant histiocytes), these cells are surrounded by lymphocytes, plasma cells, and eosinophils 7

Reed Sternberg CellsThe hallmark of the HLVery large cells, 50 -100 m, irregular with abundant pale acidophilic cytoplasm, usually with 2 lobulated nuclei each contains a gigantic azurophilic nucleolus with a perinuclear haloThe usual immunophenotypes: CD45-,CD30+, CD15+These cells can be found in other diseases: Rubella, EBV, IMN, Toxoplasmosis, T-cell lymphoma, multiple myeloma8

Morphological Variants of RS Cells

Classical LacunarPopcorn cellsSarcomatous

Each type of these variants is associated with a specific histological type of HLImmunophenotypes:

More types of RS cells are HLA-DR + expressing T-cell null lineage receptors for IL-2(CD25+), CD13 +, CD15+, CD45-,CD30+Not reactive to B-cell antigensClonal T-cell rearrangement T zone distributionLymphocyte predominant form of HDRS variant typically displays Pan B-cell antigens, tendency of invasion to B-cell areaSome RS variants have feature of monocyte-macrophage lineage; phagocytic and contain lysozyme and alpha -naphthyl esterase

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Variant of Reed-Sternberg cells (Nondiganostic)

10Mononuclear Hodgkin cell

L& H (popcorn cell)Characteristic cell in NLPHL

Pleomorphic Reed-Sternberg cell

Lacunar cell

Rye classification SystemHistological classification of HD is based on histological examination of a lymph nodeRye classification system is a modification of Lukes and Butler systemLymphocyte predominant, * WHO recognizes it as a distinct form of HD Nodular sclerosingMixed cellularityLymphocyte depleted* Each classification is based on: the amount of Lymphocyte presents, the type of RS cells, and the degree of fibrosis

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WHO ClassificationLymphocyte predominant Classic HDNodular sclerosing Mixed cellularityLymphocyte rich Lymphocyte depletion12

WHO:Lymphocyte PredominantPatients: usually young, asymptomatic and commonly in stage I or II, good prognosis The lymph node:Small lymphocytes, mature B-cellsVarying number of histiocytesA few plasma cells

There is no: neutrophils, eosinophils, necrosis and fibrosis RS variant cells: Popcorn cells/L&H cells in large numbers: small cells with lobulated nuclei and small nucleoliL&H cells relates to proliferating germinal center (centroblast) cells Intra-follicular patternB cells; CD30+ and CD15-

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WHOLymphocyte Predominant

Two types of LPNodular patternMalignant cells surrounded by lymphocytesMale predominantIn younger patients

Diffuse patternCluster of malignant cells surrounded by loosely arranged non- malignant reactive T cells

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NLPHLhttp://www.pathpedia.com/

WHO: Classic HD Nodular Sclerosing (NSHL) Common type of HD, 60% to 80% cases of HLMore common in female patient less than 50Invasion to mediastinumGood prognosis because of the patients fibrogenic defense Lymph node:Histological pattern: follicular, birefringent collagenous sclerosisNodules of H-cellsVariable number of lymphocytes separated by thick bands of collagenT, B, Null cells; CD30+ and CD15+Classic Reed-Sternberg cellsLacunar RS cellsAn empty space /lacuna around the nucleusBecause of cytoplasm shrinkage duringformalin fixation

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Nodular Sclerosis HL

16Nodular Sclerosis HL, Collagen bands between the lymphoid tissue

Classic HD:Mixed Cellularity (MCHL)15% to 30% cases of HLPatients usually present in stage III or IV, poor prognosisHeterogeneous mixed Cellularity:Diffuse or follicular histologic patternNeoplastic cells outnumber the reactive cellsNumerous classical RS and RS variants (mononuclear)Lymphocytes, macrophages, eosinophils and plasma cells, histiocytesPresence of fibrosis but no collagenT, B and Null cells;CD30+ and CD15+

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MCHL

Classic HD:Lymphocyte- Rich5% of HL casesRich in small lymphocytesSmall numbers of classic RS and H cellsClinical and pathological features more close to Mixed Cellularity HD

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Classic HD:Lymphocyte DepletionRarest (