A Rare Case of Richter Transformation to Both Clonally Unrelated and Clonally Related Diffuse Large B-Cell Lymphoma in the Same Patient

TL;DR

This paper reports a rare case where a patient developed two different types of lymphoma from the same leukemia, with distinct genetic mutations and prognoses.

Contribution

First reported case of both clonally related and unrelated Richter transformation to DLBCL in the same patient.

Findings

The patient first developed clonally unrelated diffuse large B-cell lymphoma (DLBCL).

Subsequently, the patient developed clonally related DLBCL with more aggressive mutations.

Next-generation sequencing revealed distinct mutational landscapes between the two lymphomas.

Abstract

Richter transformation (RT) is a rare sequelae of chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL). The clonal relationship of the RT to the underlined CLL/SLL is an important prognostic factor as clonally related RT has a worse prognosis than that of clonally unrelated RT. The development of more than one RT in the same patient is exceedingly rare and prior reports have shown cases consisting of RT to diffuse large B-cell lymphoma (DLBCL) and a subsequent or synchronous Hodgkin lymphoma. Here, we present a rare case of RT first to a clonally unrelated DLBCL and subsequently a clonally related DLBCL. Additionally, we retrospectively conducted next-generation sequencing studies of both RT's and found different mutational landscapes, including more clinically aggressive mutations identified in the clonally related RT. To our knowledge, this is the first reported case of clonally related and clonally unrelated RT, both of which are DLBCL, in the same patient.

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