Pathology · Hematological Malignancies (Leukemias, Lymphomas, Myeloma)

A 55-year-old with diffuse large B-cell lymphoma (DLBCL) has biopsy demonstrating MYC rearrangement AND BCL-2 rearrangement by FISH. This entity is classified as:

  • A High-grade B-cell lymphoma with MYC and BCL-2 rearrangements (double-hit lymphoma)
  • B Burkitt lymphoma
  • C Follicular lymphoma grade 3B
  • D Primary mediastinal large B-cell lymphoma
Correct answer: A. High-grade B-cell lymphoma with MYC and BCL-2 rearrangements (double-hit lymphoma)

Explanation

The 2022 WHO classification specifically designates B-cell lymphomas harboring concurrent MYC plus BCL-2 and/or BCL-6 rearrangements as 'High-grade B-cell lymphoma with MYC and BCL-2 rearrangements' (HGBL-MYC/BCL2), informally called double-hit or triple-hit lymphoma. These have extremely aggressive behavior and poor prognosis with standard R-CHOP, requiring intensified regimens like DA-EPOCH-R. They are distinct from Burkitt lymphoma which has isolated MYC rearrangement with specific morphology.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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