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T-cell/Histiocyte-rich Large B-cell Lymphoma : Molecular Genetics

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T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) shows clonally rearranged immunoglobulin genes and germline T-cell receptor genes. BCL2 is rearranged in 25% of cases.

THRLBCL and nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) appear to be related and may represent different ends of the same disease spectrum with different clinical manifestations. They show distinct as well as similar cytogenetic and molecular abnormalities. Gains of 2p16.1 and losses of 2p11.2 and 9p11.2 have been reported in both.

A recent study demonstrated recurrent mutations at similar frequencies involving JUNB, DUSP2, SGK1, SOCS1, and CREBBP genes in both THRLBCL as well as NLPHL further cementing the relationship between the two entities. Reference: Schuhmacher, B et al. JUNB, DUSP2, SGK1, SOCS1 and CREBBP are frequently mutated in T-cell/histiocyte-rich large B-cell lymphoma. Haematologica 2018; Volume 104(2):330-337.

The image shows scattered large atypical lymphoid cells (B-cells) in a background of abundant small lymphocytes (T-cells).

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