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Pathology - Mantle cell lymphoma
A mature B-cell neoplasm characterized by monomorphic, small to medium-sized lymphoid cells exhibiting uneven nuclear outlines and a CCND1 translocation.
Epidemiology • Constitutes 3–10% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults, with a mean age of 60 years.
Aetiology: Unknown. Genetics • Nearly all instances exhibit a t(11;14) translocation involving the CCND1 (cyclin D1) and IGH genes. Deregulated expression of cyclin D1 leads to unrestrained proliferation of lymphoid cells.
Presentation: The majority of individuals exhibit lymph node involvement. The liver, spleen, bone marrow, or peripheral blood may also be affected. Extranodal areas, especially the gastrointestinal tract, may also be involved. Histopathology • Affected tissues are substituted by sheets of monomorphic, tiny to medium-sized lymphoid cells exhibiting irregular nuclear contours. • Hyalinized small blood arteries and dispersed epithelioid histiocytes are frequently observed. Immunophenotype: B-cell markers PAX5, CD20, and CD79a exhibit positivity. • CD5 and cyclin D1 exhibit positivity. • CD23 and CD10 are often negative.
Prognosis • Despite its unremarkable look, the prognosis is typically unfavorable, with a median survival of merely 3–5 years.
A mature B-cell neoplasm characterized by monomorphic, small to medium-sized lymphoid cells exhibiting uneven nuclear outlines and a CCND1 translocation.
Epidemiology • Constitutes 3–10% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults, with a mean age of 60 years.
Aetiology: Unknown. Genetics • Nearly all instances exhibit a t(11;14) translocation involving the CCND1 (cyclin D1) and IGH genes. Deregulated expression of cyclin D1 leads to unrestrained proliferation of lymphoid cells.
Presentation: The majority of individuals exhibit lymph node involvement. The liver, spleen, bone marrow, or peripheral blood may also be affected. Extranodal areas, especially the gastrointestinal tract, may also be involved. Histopathology • Affected tissues are substituted by sheets of monomorphic, tiny to medium-sized lymphoid cells exhibiting irregular nuclear contours. • Hyalinized small blood arteries and dispersed epithelioid histiocytes are frequently observed. Immunophenotype: B-cell markers PAX5, CD20, and CD79a exhibit positivity. • CD5 and cyclin D1 exhibit positivity. • CD23 and CD10 are often negative.
Prognosis • Despite its unremarkable look, the prognosis is typically unfavorable, with a median survival of merely 3–5 years.
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