Deoxycoformycin (pentostatin) in the treatment of splenic marginal zone lymphoma (SMZL) with or without villous lymphocytes.
BACKGROUND
Splenic marginal zone lymphoma (SMZL) is an infrequent B-cell neoplasm that pursues an indolent course. Signs and symptoms, mostly related to hypersplenism, are successfully managed by splenectomy. However, the therapy of patients who are not fit for a surgical procedure or who relapse after splenectomy, is still an unsettled issue.


METHODS
We report a phase-II study on 16 patients with SMZL, three therapy naïve and 13 pretreated, all showing systemic symptoms or progressive worsening of peripheral cytopenia, who were treated with pentostatin at a dose of 4 mg/m2 every other week for 6-10 wk. In relapsed patients, the median interval between diagnosis and treatment was 26 month (range: 8-49).


RESULTS
Overall, 68% of the patients showed a clinical response. Two out three patients, who received pentostatin as first line therapy, attained a complete response (CR). One CR and seven minor or good haematological responses were recorded in relapsed patients. Treatment toxicity, mostly haematological, proved manageable. With a median follow-up of 35 month the median overall survival (OS) is 40 month and the median progression free survival (PFS) is 18 month.


CONCLUSIONS
Our data show that pentostatin administered every other week has a good degree of activity in the treatment of SMZL and suggest that this schedule could be considered a possible therapeutic option for patients who are not fit for splenectomy or have relapsed.
Department of Oncology, Hematology and BMT Unit, University of Palermo, Italy. iannitto@tin.it
Emilio
E
Iannitto
Viviana
V
Minardi
Giuseppina
G
Calvaruso
Emanuele
E
Ammatuna
Ada Maria
AM
Florena
Antonino
A
Mulè
Claudio
C
Tripodo
Gerlando
G
Quintini
Vincenzo
V
Abbadessa
D000328:Adult; D000368:Aged; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000473:pathology; D018442:Lymphoma, B-Cell, Marginal Zone / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary; D015649:Pentostatin / Q000008:administration & dosage* / Q000633:toxicity; D012074:Remission Induction; D013160:Splenic Neoplasms / Q000188:drug therapy* / Q000401:mortality; D016019:Survival Analysis
D015649:Pentostatin