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LYMPHOID DISORDERS

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WBC Disorders Part 3

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LYMPHOID NEOPLASMS

LYMPHOID DISORDERS

Lymphocyte Development

Lymph Node3InfectionsAcute

ChronicTBToxoplasmaHIV, LGVCat Scratch diseaseInfectious mononucleosis

Autoimmune DisordersNeoplasms Lymphomas

MetastasisLymph Node Enlargement4L- Lymphoma, LeukemiaY-Yersinia Pestis (Plague)M-Mononucleosis or CMVP-Parasite - ToxoplasmosisH-Hodgkins Disease or HIV infectionN-Neoplasm or metastasisO-Obvious local infection or inflammation Other systemic infections: Hep B, Rubella, Cat scratchD-Drug- Procainamide , Phenytoin (Dilantin)E-Endocrine - Addisons, HypothyroidS- Syphilis SLE/Rheumatoid arthritis Serum sickness, Sarcoid

Lymphadenopathy

5INFECTIOUS MONONUCLEOSISSelf limiting lymphoproliferative diseaseEBVYoung adults 14 24 yrs Virus - infects resting B cells

Pharyngitis

Splenomegaly

Hepatomegaly, jaundice

Skin rashes

Clinical Features9Hematological findingsTLC - Lymphocytosis Atypical /reactive lymphocytes + (CD8 Cells)Downy IDowny IIHeterophile Ab - Monospot testPaul Bunnell test

Atyp. LympocytesAb. CytoplasmClear vaculationsAzurophilic granulesLYMPHOID NEOPLASMS

What is the difference between Lymphoma & Leukemia???

Lymphoma Proliferations arising as discrete tissue massesLeukemia Widespread involvement of BM & peripheral bloodWHO ClassificationPrecursor B-Cell NeoplasmsB cell acute lymphoblastic leukemia/ lymphoma (B-ALL)WHO ClassificationPeripheral B-Cell NeoplasmsChronic lymphocytic leukemia/ small lymphocytic lymphoma (CLL/SLL)B-cell prolymphocytic leukemiaLymphoplasmacytic lymphomaSplenic and nodal marginal zone lymphomasExtranodal marginal zone lymphomaMantle cell lymphoma

WHO ClassificationPeripheral B-Cell NeoplasmsFollicular lymphomaMarginal zone lymphomaHairy cell leukemiaPlasmacytoma/plasma cell myelomaDiffuse large B-cell lymphomaBurkitt lymphomaWHO ClassificationPrecursor T-Cell NeoplasmsT cell acute lymphoblastic leukemia/ lymphoma (T-ALL)WHO ClassificationPeripheral T-Cell and NK-Cell NeoplasmsT-cell prolymphocytic leukemiaLarge granular lymphocytic leukemiaMycosis fungoides/Szary syndromePeripheral T-cell lymphoma, unspecifiedAnaplastic large cell lymphomaAngioimmunoblastic T-cell lymphomaWHO ClassificationPeripheral T-Cell and NK-Cell NeoplasmsEnteropathy-associated T-cell lymphomaPanniculitis-like T-cell lymphomaHepatosplenic T-cell lymphomaAdult T-cell leukemia/lymphomaNK/T-cell lymphoma, nasal typeNK-cell leukemiaWHO ClassificationHodgkin LymphomaClassical subtypesNodular SclerosisMixed CellularityLymphocyte richLymphocyte depletion

Lymphocyte Predominance

CHRONIC LYMPHOCYTICLEUKEMIA/SLLProliferation of small mature B lymphocytes

DefinitionOld age, >50 yrs,maleFatigueLymphadenopathy, organomegalyMay have Ab production and auto-immune hemolytic anemia

Clinical FeaturesSustained lymphocyte count >4000/c mm

> 30% lymphocytes in bone marrow

Monoclonal B cellsCriteria for Diagnosis Hb

TC- 20,000 to 50,000/c mm

Lymphocytosis >10,000

Smudge cells

Hypercellular marrowHematological Findings

CLL Prolymphocytes 55%FAB ClassificationCells positive for Pan B cell Markers CD19 & CD20CD23CD5ImmunophenotypingHAIRY CELL LEUKEMIA

Abnormal mononuclear cells Hairy cytoplasmic projectionsAdults M>FMarked splenomegalyHematological findings

Pancytopenia Normocytic anemia Cells with hair like cytoplasmic projections Cells + for TRAP ( Tartrate Resistant Acid Phosphatase)

Cells positive for Pan B cell Markers CD19 & CD20CD11cCD25CD103Immunophenotyping

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