Splenic marginal zone lymphoma with or without villous lymphocytes. Hematologic findings and outcomes in a series of 57 patients.
BACKGROUND
Splenic marginal zone lymphoma (SMZL) is a well defined pathologic entity. However, questions regarding the bone marrow infiltration rate, the minimal diagnostic data set, and therapy remain unanswered.


METHODS
Clinical-pathologic features and outcomes of 57 consecutive patients who had splenomegaly with no clinically significant lymphadenomegaly and who were diagnosed with SMZL with or without (+/-) villous lymphocytes (VL) were reviewed.


RESULTS
SMVL +/- VL occurred mostly in elderly males (median age, 62 years +/- 10 years; male-to-female ratio, (1.85). Anemia was recorded in 49% of patients, and 30% of patients had moderate thrombocytopenia. Leukocytosis and leukopenia were found in 33% and 14% of patients, respectively, and typical VL were found in 84% of patients. Serology for hepatitis C virus infection was positive in 16% of patients, and a small monoclonal component was detected in 36% of patients. The bone marrow was infiltrated with an intrasinusoidal component in all patients. Thirteen patients were monitored using a watch-and-see policy, and they remained alive 1-5 years after diagnosis. Overall, 21 patients (36%) underwent splenectomy; and, in all patients, the diagnosis of SMZL was confirmed histologically in the surgical specimens. Twenty-five patients received single-agent therapy, which included either alkylators or pentostatine, and they achieved an overall response rate (ORR) of 65% and 87%, respectively: Polychemotherapy was administered to 6 patients (ORR, 83%). The median survival for all patients in the series was not reached, and it is expected that 70% of patients will be alive at 5 years.


CONCLUSIONS
Up to 20% of patients who had SMZL +/- VL could be monitored using a watch-and-wait policy. The bone marrow intrasinusoidal infiltration pattern may be a valuable diagnostic hallmark, thus obviating diagnostic splenectomy. The issues regarding prognostic stratification and the best therapeutic strategy need to be addressed in properly designed, prospective trials.
Iannitto
Emilio
E
Department of Oncology, Hematology and Bone Marrow Transplantation Unit, University of Palermo, Palermo, Italy. eiannitto@tin.it
Ambrosetti
Achille
A
Ammatuna
Emanuele
E
Colosio
Maura
M
Florena
Ada M
AM
Tripodo
Claudio
C
Minardi
Viviana
V
Calvaruso
Giuseppina
G
Mitra
Maria Enza
ME
Pizzolo
Giovanni
G
Menestrina
Fabio
F
Franco
Vito
V
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000473:pathology*; D008223:Lymphoma / Q000097:blood / Q000401:mortality / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000097:blood / Q000401:mortality / Q000628:therapy*; D015996:Survival Rate