LYMPHOID NEOPLASMS

LYMPHOID NEOPLASMS

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)Most common of NHL in western countriesMiddle age Lymph node enlargement , involvement of extranodal sitesArises from Germinal Center B CellsChromosomal translocations involving BCL2Asymptomatic patient - watchful waiting as spontaneous regression in some cases Indolent - Palliative chemo/radiotherapy Follicular Lymphoma5

B cell CD19,20,10t (14,18)BCL2 +Centrocytes and centroblasts620% of NHL, aggressive60yrsImmunodeficiencyRapidly enlarging single nodal/extranodal massRemission intensive chemo

B cell CD19,20Diffuse Large B Cell Lymphoma7 B-cell lymphoma EBV-virusChromosomal translocation (8;14 or 8;22)ChildrenExtra nodal site Jaw involvement: Maxilla:Mandible=2:1

Burkitts Lymphoma8Three distinct forms Sporadic burkitt Endemic burkitt Immune deficiencyRapidly progressiveAbdominal viscera, metastasize to CNS Chemotherapy

Burkitts Lymphoma

Starry sky pattern

B cell 19,20,10 +vet (8;14)EBV DNA11Aggressive60-65yrs; M>FProliferation of cells in Mantle zone of LNCD5, cyclin D1 positiveCD23 negativeStem cell transplant is offered often as front-line consolidation treatment in younger patientsMantle Cell LymphomaHeterogenous group of lymphoproliferative disorders

Localization of neoplastic T- cells to the skin.

65% of primary cutaneous lymphomas are of T- cell types.Primary Cutaneous T Cell LymphomaMC - cutaneous lymphoma

Infiltrate of small to medium sized T lymphocytes with epidermotropismMycosis Fungoides3 Phases of progression:Macular eruptionPlaque/Patch phase, resembles eczema/psoriasisNodular stage associated lymphadenopathy or visceral involvement

Sezary Syndrome:Circulating malignant (Sezary) cells

Clinical Features

Presence of atypical Tcells in the epidermis= Pautriers microabscesses

Szary syndrome: cutaneous T-cell lymphoma+ systemic involvement20

Multiple MyelomaNormal Plasma cellFORMED IN THE LYMPH NODEAntigen

B-lymphocyte

Activated B Lymphocyte

Immunoblast

Plasmacytoid lymphocyte

Plasma cell

9-20 Eccentric nucleusCartwheel chromatin Basophilic cytoplasmPerinuclear clearing hoffNo NucleoliCytoplasm Ig< 4% in bone marrow

Plasma cellMalignant clonal proliferation of plasma cells in bone marrow

Multiple lytic bone lesions

Production of monoclonal Ig(M protein)

DefinitionUnknown

Some postulated causes are :RadiationOcupational exposure Infection ( HHV -8 )Cytogenetics (translocation { 11 ,14 } ,13q14 &17p13 deletions ,trisomy 9)IL 6 overproduction

Etiology>50 yrs

M>F

Infiltration of boneBone painFractures

Clinical FeaturesRenal failure

Recurrent infections

Neurological symptoms

AmyloidosisClinical FeaturesMajorPlasmacytoma on tissue biopsyBM- plasma cells >30%Monoclonal globulin spike IgG>35g/lUrine- Bence-jones proteins (light chains)

Diagnostic CriteriaMinorBM-10-30% plasma cellsIgG 20% - Plasma cell Leukemia

Hematological Findings

Plasma Cell Leukemia

Bone marrowHypercellularErythroid, myeloid & megakaryocytic- supressedPlasma cells - >30%Plasmablasts, Multinucleated cells, Flame cells, Mott cells, Russell & Dutcher bodies

Hematological Findings

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