AI Article Synopsis
- Splenic marginal zone lymphoma (SMZL) is a rare type of lymphoma, making up only 1% of all cases, and a study reviewed the experiences of 13 patients with this disease.
- Among these patients, prognostic evaluations indicated that 31% had good prognosis, 38% had intermediate, and another 31% had poor prognosis, especially those with high-risk factors like anemia and elevated LDH.
- The presence of autoimmune hemolytic anemia (AIHA) significantly worsens survival rates, leading to a grim outcome with a 100% mortality rate in those affected, indicating that AIHA is an important negative prognostic factor in SMZL.
Article Abstract
Splenic marginal zone lymphoma is a rare disease, accounting for 1% of all lymphomas. We reviewed our single center experience of 13 patients with splenic marginal zone lymphoma (SMZL). Based on the prognostic model developed by Intergruppo Italiano Linfomi, 31% (4/13) of our patients had good, 38% (5/13) had intermediate and 31% (4/13) had a poor prognosis. The presence of two out of three prognostic factors (anemia, elevated LDH, low serum albumin) assignes the patient into the high risk category. In patients with anemia and an elevated LDH due to hemolysis, the outcome seems to be especially poor. Three out of 13 (23%) cases were complicated by autoimmune hemolytic anemia. All patients with autoimmune hemolytic anaemia (AIHA) died 7-28 months after the diagnosis. The mean follow-up time of those nine patients who are still alive is longer than 5 years (36-100 months). Patients with AIHA had significantly (p < 0.001) worse survival than those without AIHA. The main finding of our study is that the presence of AIHA is an adverse prognostic factor in SMZL.
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| http://dx.doi.org/10.1007/s12253-009-9159-8 | DOI Listing |
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