High mitotic activity with rapid cellular turnover and characteristic "Starry sky" appearance is seen in
Ans. c (Burkitt's lymphoma) (Ref. Robbins 7th/677).The types of NHL involving mature B cells are 4, namely - Burkitt's lymphoma, Diffuse large B-cell lymphoma, Mantle cell lymphoma, and Follicular lymphoma. Burkitt's lymphoma occrs in adolescents or young adults, associated with t(8;14)-- translocation of c-myc (8) and heavy-chain Ig (14), and histologically shows typical "Starry sky" appearance - sheets of lymphocytes with interspersed macrophages. It is known to be associated with EBV.BURKITT LYMPHOMA# <1% of lymphomas# 3 categories:- Endemic in Africa,- Sporadic elsewhere; (Clinically a jaw lesion in endemic form in Africa; pelvis or abdomen in sporadic form)- Increased frequency in immunosuppressed patients;# EBV infection seen in all categories of Burkitt's, which precedes cellular transformation.# Predominantly affects children;# Often presents with visceral involvement;# Highly aggressive.# Diffuse tissue involvement associated with apoptosis produces a "starry-sky" appearance.# Intermediate-sized round lymphoid cells with several nucleoli;# Immunophenotype:-Mature CD10+, CD19+, CD20+ B cells expressing surface IgM, monotypic kappa and lambda light chain and BCL6+.# Common translocations:- t(8,14), -1(2,8), -1(8,22)# C/f:- Endemic and sporadic cases commonly found in children and young adults- Most tumors manifest at extrannodal sites.EndemicSporadicMass involving mandibleAbdominal mass involving ileocecum and peritoneum.May involve abdominal viscera (kidney, ovary, adrenals)Bone marrow and peripheral blood involvement uncommon.# Rx: Treatment of Burkitt's lymphoma in both children and adults should begin within 48 h of diagnosis and involves the use of intensive combination chemotherapy regimens incorporating high doses of cyclophosphamide. Prophylactic therapy to the CNS is mandatory. Burkitt's lymphoma was one of the first cancers shown to be curable by chemotherapy. Today, cure can be expected in 70 to 80% of both children and young adults when effective therapy is administered precisely. EntityFrequencyMorphologyImmunophenotypeCommentsPrecursor B-cell lymphoblastic leukemia/ lymphoma85% of childhood acute leukemiaLymphoblasts with irregular nuclear contours, condensed chromatin,small nucleoli, and scant agranular cytoplasmTdT+immature B cells (CD19+, variable expression of other B-cell markers)Usually presents as acute leukemia; less common in adults; prognosis is predicted by karyotypePrecursor T-cell leukemia/ lymphoma15% of childhood acute leukemia; 40% of childhood lymphomasIdentical to pre-cursor B-cell lymphoblastic leukemia/ lymphomaTdT+immature T cells (CD2+, CD7+, variable expression of other T-cell markers)Most common in ado- lescentmales;presents as a mediasstinal mass due to thymic invo; high asso NOTCH1 mutation.Small lymphocytic lymphoma/ chronic lymphocytic leukemia3% to 4% of adult lymphomas; 30% of all leukemiasSmall resting lymphocytes mixed large activated cells lymph nodes effacedCD5+B-cell expressing surface IgOccurs in older adults; usually involves nodes, marrow, spleen; indolent; most pts have peripheral blood involvement;Follicular lymphoma40% of adult lymphomasFreq small "cleaved" cells mixed with large cells; growth usually nodular (follicular)CD10+BCL2+ mature B cells that express surface IgOccurs in older adults; usually involves nodes, marrow, and spleen; asso with t( 14; 18); indolentMantle cell lymphoma3% to 4% of adult lymphomasSmall to intermediate- sized irregular lymphocytes growing in a diffuse patternCD5+mature B cells that express cyclin D1 and have surface IgOccurs mainly in older males; usually involves nodes, marrow, spleen and GIT; t(11;14) +; moderately aggressiveExtranodal marginal zone lymphoma?5% of adult lymphomasVariable cell size and differentiation;40%show plasmacytic differentia"B cells home to epith creating "lymphoepith lesions"CDS- CD10- mature B cells with surface IgFrequently occurs at extranodal sites involved by chronic inflammation very indolent; may be cured by local excisionDiffuse large B-cell lymphoma40% to 50% of adult lymphomasVariable; most resemble large germinal center B cells; diffuse growth patternMature B cells with variable expression of CD10 and surface IgOccurs in all ages, but most common in olderadults; often arise at extranodal sites; aggressiveBurkitt Lymphoma<1% of lymphomas in the United StatesIntermediate-size round lymphoid cells with nucleoli; diffuse tissue invol asso with apoptosis produces a "starry-sky" app.Mature CD10+B cells expressing surface IgEndemic in Africa, sporadic elsewhere; increased frequency in immunosuppressed; predominantly kids; presents with visceral involvement; highly aggr.Plasmacytoma/ plasma cell myelomaMost common lymphoid neoplasm in older adultsPlasma cells in sheets, some- times with prominent nucleoli or inclusions containing IgTerminally differentiated plasma cells containing cytoplasmic IgMyeloma presents as disseminated bone disease, often with lytic lesions,Hypercalcemia, renal insufficiency, bacterial infections commonMycosis fungoidesMost common cutaneous lymphoid malignancyIn most cases, small lymphoid cells with markedly convoluted nuclei; cells often infiltrate the epidermis (Pautrier microabscesses)CD4+mature T cellsPresents with localized or more generalized skin involvement; generally indolent. Sezary syndrome, a more aggressive variant, is ch by skin erythema and peripheral blood involvementPeripheral T-cell lymphoma, not other wise specified (NOS)Most common adult T-cell lymphomaVariable; usually a spectrum of small to large lymphoid cells with irregular nuclear contoursMature T-cell phenotype (CD3+)Probably spans a diverse collection of rare tumors. Often disseminated, generally aggressive.
Follicular lymphoma
Diffuse lymphoma
Burkitt's lymphoma
Hodgkin's lymphoma
Blood