title: High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis.
abstract: In 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 < 0.03). 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.
authors.affiliation: Department of Haematology, The Royal Marsden Hospital NHS Foundation Trust, London, UK.
authors.forename: M
authors.initials: M
authors.lastname: Dungarwalla
authors.forename: S
authors.initials: S
authors.lastname: Appiah-Cubi
authors.forename: S
authors.initials: S
authors.lastname: Kulkarni
authors.forename: R
authors.initials: R
authors.lastname: Saso
authors.forename: A
authors.initials: A
authors.lastname: Wotherspoon
authors.forename: N
authors.initials: N
authors.lastname: Osuji
authors.forename: J
authors.initials: J
authors.lastname: Swansbury
authors.forename: D C
authors.initials: DC
authors.lastname: Cunningham
authors.forename: D
authors.initials: D
authors.lastname: Catovsky
authors.forename: C E
authors.initials: CE
authors.lastname: Dearden
authors.forename: E
authors.initials: E
authors.lastname: Matutes
mesh_terms: D000368:Aged; D002471:Cell Transformation, Neoplastic / Q000473:pathology*; D016009:Chi-Square Distribution; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008198:Lymph Nodes / Q000473:pathology*; D008214:Lymphocytes / Q000473:pathology*; D018442:Lymphoma, B-Cell, Marginal Zone / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D013160:Splenic Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D015996:Survival Rate