High grade B-cell lymphoma with MYC and/ or BCL2 and/or BCL6 rearrangement

Orpha code: 480541OMIM code:

Definition

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 <i>de novo</i> 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.

Disease data
Classification

Disease

ORPHA code
480541
OMIM code
-
ICD10 code
C85.7
ICD11 code
-

No additional description.

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