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Високодиференційована В-клітинна лімфома з перебудовою MYC та/або BCL2 та/або BCL6

ORPHA:480541· ICD-10 C85.7· High grade B-cell lymphoma with MYC and/ or BCL2 and/or BCL6 rearrangement

Визначення(English summary)

A rare aggressive B-cell non-Hodgkin lymphoma characterized by a rearrangement in MYC and BCL2 and/or BCL6 (so-called double-hit or triple-hit lymphoma). The category includes double-hit cases with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma, blastoid cases with a double-hit, and cases with a DLBCL, not otherwise specified, morphology with a double-hit. It refers only to de novo cases, not to lymphomas with a history of pre-existing or coexistent indolent lymphoma. Patients typically present with widespread disease, including involvement of lymph nodes, bone marrow, and central nervous system.