Successful treatment of catastrophic antiphospholipid antibody syndrome (CAPS) associated with splenic marginal-zone lymphoma with low-molecular weight heparin, rituximab and bendamustine.

Case Report: A 69-year-old woman with splenic marginal-zone lymphoma was admitted with progressive abdominal pain and splenomegaly as the suspected cause of pain. Rituximab treatment (375 mg/m) had been initiated on the day of admission. Abdominal computerized tomography revealed splenic infarction. Laboratory tests showed elevation of liver enzymes and creatinine, low platelet count, prolonged partial thromboplastin time, and lupus anticoagulant positivity. The diagnosis of catastrophic antiphospholipid antibody syndrome was made. Weight-adjusted low-molecular weight heparin therapy was initiated. Freedom from symptoms and normalization of liver enzymes and creatinine occurred within 4 weeks. Treatment was continued with 6 cycles of bendamustine monotherapy (90 mg/m) and heparin, leading to partial remission of lymphoma and lupus anticoagulant negativity.

Conclusions: In case of multiorgan failure in patients suffering from lymphoma and showing features of disseminated intravascular coagulation, catastrophic antiphospholipid antibody syndrome should be considered. In our patient, rituximab followed by weight-adjusted low-molecular weight heparin and bendamustine therapy led to recovery.