title: Splenic marginal zone lymphoma with or without villous lymphocytes. Hematologic findings and outcomes in a series of 57 patients.
abstract: 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.
authors.affiliation: Department of Oncology, Hematology and Bone Marrow Transplantation Unit, University of Palermo, Palermo, Italy. eiannitto@tin.it
authors.forename: Emilio
authors.initials: E
authors.lastname: Iannitto
authors.forename: Achille
authors.initials: A
authors.lastname: Ambrosetti
authors.forename: Emanuele
authors.initials: E
authors.lastname: Ammatuna
authors.forename: Maura
authors.initials: M
authors.lastname: Colosio
authors.forename: Ada M
authors.initials: AM
authors.lastname: Florena
authors.forename: Claudio
authors.initials: C
authors.lastname: Tripodo
authors.forename: Viviana
authors.initials: V
authors.lastname: Minardi
authors.forename: Giuseppina
authors.initials: G
authors.lastname: Calvaruso
authors.forename: Maria Enza
authors.initials: ME
authors.lastname: Mitra
authors.forename: Giovanni
authors.initials: G
authors.lastname: Pizzolo
authors.forename: Fabio
authors.initials: F
authors.lastname: Menestrina
authors.forename: Vito
authors.initials: V
authors.lastname: Franco
mesh_terms: 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