High-grade transformation in splenic marginal zone lymphoma with circulating villous lymphocytes: the site of transformation influences response to therapy and prognosis.
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.
Dungarwalla
M
M
Department of Haematology, The Royal Marsden Hospital NHS Foundation Trust, London, UK.
Appiah-Cubi
S
S
Kulkarni
S
S
Saso
R
R
Wotherspoon
A
A
Osuji
N
N
Swansbury
J
J
Cunningham
D C
DC
Catovsky
D
D
Dearden
C E
CE
Matutes
E
E
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