Histologic transformation of chronic lymphocytic leukemia/small lymphocytic lymphoma

Abstract

Although generally considered a clinically indolent neoplasm, CLL/SLL may undergo transformation to a clinically aggressive lymphoma. The most common form of transformation, to DLBCL, is also known as Richter syndrome. Transformation determines the course of the disease and is associated with unfavorable patient outcome. Precise detection of transformation and identification of predictive biomarkers and specific molecular pathways implicated in the pathobiology of transformation in CLL/SLL will enable personalized therapeutic approach and provide potential avenues for improving the clinical outcome of patients. In this review, we present an overview of the pathologic features, risk factors, and pathogenic mechanisms of CLL/ SLL transformation.

Targeted multigene deep sequencing of Bruton tyrosine kinase inhibitor–resistant chronic lymphocytic leukemia with disease progression and Richter transformation

“…8,12,25,28 To the best of our knowledge, understanding of the genomic characterization of RT arising within the setting of BTKi therapy is limited, unlike RT in other settings, which has been relatively well studied. [29][30][31] Approximately 80% of the large B-cell lymphomas arising from patients with CLL are clonally related. 32 Our RT series lacks the V-D-J rearrangement data to determine the clonal relationship with underlying CLL.…”

Herpes simplex infection simulating Richter transformation: a series of four cases and review of the literature

“…HSV infection could be managed with antiviral therapy and supportive care, and patients usually respond remarkably well to this approach, whereas patients with RT require aggressive multiagent chemoimmunotherapy, [35][36][37] and, despite therapy, the prognosis for patients with RT is extremely poor, with overall survival of <1 year. 1 In summary, we describe four cases of CLL/SLL with superimposed HSV infection with characteristic histopathological features. These patients presented with clinical findings suggestive of RT, prompting lymph node biopsy.…”

“…The distinction between RT and HSV infection is obviously important, as these two entities have very different clinical and therapeutic implications. HSV infection could be managed with antiviral therapy and supportive care, and patients usually respond remarkably well to this approach, whereas patients with RT require aggressive multiagent chemoimmunotherapy, and, despite therapy, the prognosis for patients with RT is extremely poor, with overall survival of <1 year …”

“…Diffuse large B-cell lymphoma (DLBCL) arising in patients with chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL), also known as Richter syndrome or Richter transformation (RT), occurs in up to 11% of patients, and is associated with a poor prognosis. 1,2 In 70-80% of patients, the CLL/SLL and DLBCL are clonally related, and histological transformation reflects the acquisition of additional genetic abnormalities. Usually, CLL/SLL patients present with sudden onset of B symptoms, increasing lymphocytosis, progressive cytopenias, and elevated serum lactate dehydrogenase and b 2 -microglobulin (b2M) levels.…”

Prostatic-Like Syndrome in a Woman with Chronic Lymphocytic Leukemia: Sequential Kinase Inhibitor Therapy

“…RT carries a very poor prognosis and should be suspected when a CLL patient develops sudden onset of B symptoms, accompanied by enlarging lymph nodes. Laboratory studies commonly associated with RT include an important elevation of lactate dehydrogenase [5]. …”