High‐grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis

Abstract

SummaryIn a series of 48 patients with splenic marginal zone lymphoma (SMZL) with circulating villous lymphocytes, we describe the clinical and laboratory features of nine cases that transformed to high-grade B-cell lymphoma. These patients had a significantly greater incidence of peripheral lymph node involvement at diagnosis when compared to SMZL patients who did not transform (P < 0AE03). While transformation in the bone marrow is frequently refractory to therapy and associated with poor outcome in SMZL, lymph node transformation responds well to chemotherapy with durable progression-free and overall survival.

Clinical and biological diversity of splenic marginal zone lymphoma

“…Transformation to large-cell lymphoma has been documented. The incidence of this event is uncertain but may be approximately 10% [29,30]. Do we have information on the variables that have a prognostic impact regarding disease progression, transformation and/or survival?…”

Dysregulation of global microRNA expression in splenic marginal zone lymphoma and influence of chronic hepatitis C virus infection

“…However, in a minority of cases it can pursue a more aggressive course with the possibility of transformation into a diffuse large B-cell lymphoma. 3 microRNAs (miRNAs) are members of a class of small, noncoding RNAs that modulate gene expression at the posttranscriptional level in a sequence-specific manner. They have a role in controlling a variety of biological functions, including developmental patterning, cell differentiation, cell proliferation, genome rearrangements and transcriptional regulation.…”

Splenectomy with chemotherapyvssurgery alone as initial treatment for splenic marginal zone lymphoma

“…The established positivity of hepatitis C virus antibodies varied from 1% [1] , which was similar to our results, to 19%, while hepatitis B virus antibodies were detected in 5% of cases [25] . Serum LDH is an important activity parameter of aggressive disease, while in low-aggressive ones, its increase can designate the transformation of disease to more severe form [18] . In our study group, this parameter was within referential limits in 72% of patients, which suggested low-grade disease status.…”

“…The frequency of mutation in the 5' non-coding region of the bcl-6 gene has been used as a marker of germinal center derivation, which might be used to establish the molecular heterogeneity [14][15][16] . Therefore, SMZL is a primary disease of the spleen, with subsequent bone marrow (BM) and peripheral blood (PB) involvement [17][18][19] . The diagnosis is based on the spleen PH, in accordance with clinical data.…”