Abstract
Background: Splenic marginal zone lymphoma (SMZL) is an uncommon indolent B-cell malignancy usually involving the spleen, bone marrow and blood. Splenectomy is frequently performed for both diagnostic and therapeutic purposes, but it is unclear if it affects outcome. To further explore this issue, we reviewed clinical and pathological characteristics, and treatment outcomes of SMZL patients (pts) who underwent splenectomy in our institution.
Methods: Medical records of pts who had splenectomy at the Cleveland Clinic and were diagnosed with a B-cell lymphoproliferative disorder between 1994 and 2004 were reviewed. Pathology slides were reviewed to confirm the diagnosis of SMZL by WHO criteria. Data collection included: clinical and biochemical characteristics at diagnosis including symptoms; reason for and date of splenectomy; hematological response following surgery; additional therapies other than surgery including rationale, regimen used and outcome; and follow-up data.
Results: We identified 18 pts with SMZL having splenectomy in our institution. Twelve (67%) were females and 6 (33%) males. The median age was 65 years (range 43–85). At the time of diagnosis, 9 pts had splenic and bone marrow involvement; 8 splenic, bone marrow and nodal; 1 spleen involvement only. Four pts were treated with chemotherapy prior to splenectomy for presumed CLL, hairy-cell leukemia or unspecified B-cell lymphoproliferative disorder, but none achieved CR. None of the pts received radiation. Splenectomy was performed in 9 pts for diagnostic, and in 9 pts for therapeutic purposes. The most common reason for therapeutic splenectomy was cytopenia (n=5) and abdominal discomfort (n=4). Median follow-up from splenectomy to death or last follow-up is 30 months (range 1–99). Of the 9 pts who had splenectomy for diagnostic purposes the median time to subsequent systemic therapy (n=5) was 7 months (range 0–33). Of the 9 pts treated for therapeutic purposes abdominal symptoms resolved in all (n=4) and 4/5 with cytopenias achieved hematologic response. Seven of 9 pts with only spleen and marrow involvement, achieved complete hematologic remission following surgery lasting from 8+ to 46+months. Of 8 pts presenting with spleen and lymph node involvement, 6 had cytopenias that resolved in only 3 pts following splenectomy. Six of these 8 pts received chemotherapy after splenectomy with median time from surgery to systemic treatment of 16 months (range 3–60). One pt continues to have stable lymphadenopathy observed clinically for 33 months, and 1 pt had stable lymphadenopathy for 24 months and was subsequently lost to follow-up.
Conclusion: Splenectomy, whether performed diagnostically or therapeutically, may result in long remissions in the absence of chemotherapy. Splenectomy appears to be a viable treatment alternative for SMZL.
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