Which type of Hodgkin's disease is commonest in female and associated with excellent prognosis?
Ans. a (Nodular sclerosis) (Ref. Robbin's pathology 7th/pg. 688)Note-Classical is more common than lymphocyte predominanceCLASSIFICATION OF HODGKIN LYMPHOMASub typeMorphology and ImmunophenotypeTypical clinical featuresNodular sclerosisFrequent lacunar cells and occasional diagnosticR-S cells; background infiltrate composed of T lymphocytes, eosinophils, macrophages, and plasmacells; fibrous bands dividing cellular areas into nodules.Stage 1 or 2 disease most common.R-S cells CD15+, CD30+; EBV-.Frequent mediastinal involvement. F=M, most patients young adultsMixed cellularityFrequent mononuclear and diagnostic R-S cells; background infiltrate rich in T lymphocytes, eosinophils, macrophages, plasma cells. R-S cells, CD15+, CD30+; 70% EBV +More than 50% present as stage 3 or 4 disease. M > F. Biphasic incidence, peaking in young adults and again in adults older than 55Lymphocyte-richFrequent mononuclear and diagnostic R-S cells; background infiltrate rich in T lymphocytes, R-S cells are CD15+, CD30+, 40% EBV+.Uncommon. M>F. Tends to be seenin older adultsLymphocyte depletionReticular variant: Frequent diagnostic R-S cells are variants with a paucity of background reactive cells; diffuse fibrosis variant; hypocellular fibrillar background with scattered diagnostic R-S cells and variants and few reactive cells.R-S cells CD15+, CD30+; most EBV+.Uncommon. More common in older males, HIV infected individuals, and in developing countries.More likely to present with advanced disease.Lymphocyte predominanceFrequent L & H (popcorn cell) variants in a background of follicular dendritic cells and reactive B cells. R-S cells CD20+, CD15-, C30-; EBV-.Uncommon. Young males with cervical or axillary lymphadenopathy.MediastinalNodular Sclerosis Type # equal frequency in males and females.# It has a propensity to involve the lower cervical, supraclavicular, and mediastinal lymph nodes of adolescents or young adults and is only rarely associated with EBV.# The prognosis is excellent.Mixed Cellularity Type# Is more common in males.# strongly associated with EBV# older age,# systemic symptoms such as night sweats and weight loss, and advanced tumor stage.# Nonetheless, the prognosis is very good. FrequencySalient MorphologyImmunophenotypeCommentsPrecursor 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 precursor B-cell lymphoblastic leukemia/lymphomaTdT+immature T cells (CD2+, CD7+, variable expression of other T-cell markers)Most common in adolescent males; often presents as a mediastinal mass due to thymic involvement; highly associated with mutations in NOTCH1Small lymphocytic lymphoma/ chronic lymphocytic leukemia3% to 4% of adult lymphomas; 30% of all leukemiasSmall resting lymphocytes mixed with variable numbers of large activated cells; lymph nodes diffusely effacedCD5+B-cell expressing surface IgOccurs in older adults; usually involves nodes, marrow, and spleen; most patients have peripheral blood involvement; indolentFollicular lymphoma40% of adult lymphomasFrequent small "cleaved" cells mixed with large cells; growth pattern is usually nodular (follicular)CD10+BCL2+mature B cells that express surface IgOccurs in older adults; usually involves nodes, marrow, and spleen; associated with t(14; 18); indolent /Mantle 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, and spleen; GI tract also commonly affected; t(11;14) is characteristic; moderately aggressiveExtranodal marginal zone lymphoma~5% of adult lymphomasVariable cell size and differentiation; 40% show plasmacytic differentiation; B cells home to epithelium, creating "Lymphoepithelial lesions"CD5- CD10- mature B cells with surface IgFrequently occurs at extranodal sites involved in 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 older adults; often arise at extranodal sites; aggressiveBurkitt lymphoma<1% of lymphomas in the United StatesIntermediate-sized round lymphoid cells with several nucleoli; diffuse tissue involvement associated with apoptosis produces a "starry-sky" appearanceMature CD10+B cells expressing surface IgEndemic in Africa, sporadic elsewhere; increased frequency in immunosuppressed patients; predominantly affects children; often presents with visceral involvement; highly aggressivePlasmacytoma/ plasma cell myelomaMost common lymphoid neoplasm in older adultsPlasma cells in sheets, sometimes with prominent nucleoli or inclusions containing IgTerminally differentiated plasma cells containing cytoplasmic IgMyeloma presents as a disseminated bone disease, often with destructive lytic lesions Hypercalcemia, renal insufficiency, and bacterial infections are 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 characterized by diffuse skin erythema and peripheral blood involvementPeripheral T-cell lymphoma, not otherwise 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 aggressiveHodgkin lymphoma, nodular sclerosis typeMost common type of Hodgkin lymphomaLacunar Reed-Sternberg cell variants in a mixed inflammatory background; broad sclerotic bands of collagen usually also presentCD15+, CD30+Reed- Sternberg cellsMost common in young adults, often arises in the mediastinum or cervical lymph nodesHodgkin lymphoma, a mixed cellularity typeSecond most common form of Hodgkin lymphomaFrequent classic Reed-Sternberg cells in a mixed inflammatory backgroundCD15+, CD30+Reed- Sternberg cellsMost common in men, more likely to present at advanced stages than the nodular sclerosis type EBV+in 70% of cases
Nodular sclerosis
Lymphocyte predominant
Lymphocyte depleted
Mixed cellularity