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Pathobiology and treatment of Richter's transformation in chronic lymphocytic leukemia/small lymphocytic lymphoma

Author
Jennifer R Brown, MD, PhD
Section Editor
Arnold S Freedman, MD
Deputy Editor
Rebecca F Connor, MD

INTRODUCTION

Richter's transformation (RT, Richter's syndrome) was first described in 1928 by Maurice Richter as the development of an aggressive large-cell lymphoma in the setting of underlying chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). Although diffuse large B cell lymphoma is the most common histology seen in patients with RT [1], Hodgkin lymphoma [2,3] and T cell lymphomas [4] have also been reported less commonly. The clinical features, pathogenesis, and treatment of RT will be discussed here.

Evolution to a component of B cell prolymphocytic leukemia (B-PLL) during the natural history of relapsed CLL/SLL is also common, but is not usually included under the rubric of RT. (See "Staging and prognosis of chronic lymphocytic leukemia", section on 'Prolymphocytoid transformation'.)

INCIDENCE AND CLINICAL FEATURES

Incidence — The incidence of Richter's transformation (RT) from chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) to diffuse large B cell lymphoma has been variously estimated at 2 to 9 percent [1,5-9], making it less common than histologic transformation of other low-grade mature B cell malignancies (picture 1). The median time from the diagnosis of CLL/SLL to transformation has been in the range of two to four years [5,6,10]. For example, a series of 185 consecutive, previously untreated patients with CLL followed for a median of 47 months identified RT in 17 cases (9 percent) at a median of 23 months from diagnosis [8]. This group took an aggressive approach to repeat biopsy, which may explain the high rate of RT. In addition to those transforming to DLBCL, an additional 0.4 percent of patients will transform to Hodgkin lymphoma [11]. (See "Histologic transformation of follicular lymphoma", section on 'Clinical presentation and diagnosis'.)

Clinical features — The onset of RT is heralded by sudden clinical deterioration, characterized by a marked increase in lymphadenopathy at one or more sites (often abdominal), splenomegaly, and worsening "B" symptoms (ie, fever, night sweats, weight loss). The serum level of lactate dehydrogenase (LDH) is elevated in 50 to 80 percent of patients with RT compared with 8 percent of CLL patients [6,10,12]. Anemia with a hemoglobin <11 g/dL is seen in about 50 percent of cases, and thrombocytopenia with a platelet count <100,000/microL in 43 percent [12]. Symptoms are similar in the Hodgkin lymphoma variant. The clinical course of RT is rapidly progressive, with a median survival of 5 to 8 months [1,6,13-15].

Risk factors — Risk factors for the development of RT remain poorly defined.

               

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Literature review current through: Nov 2016. | This topic last updated: Thu Apr 30 00:00:00 GMT+00:00 2015.
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