Lymphoma-induced antiphospholipid syndrome as a cause of splenic infarction [Lymphom-induziertes Antiphospholipidsyndrom als Ursache von Milzinfarkten]

摘要

History and admission findings: A 71-year-old man presented with bilateral sialadenosis of the parotid gland, episodes of fever up to 39 C, general malaise and weight loss of 5 kg within the last 6 weeks. At physical examination peripheral lymph nodes were not palpable. Investigations: Laboratory studies revealed a normal white blood cell count, anemia and thrombocytopenia. Serum C-reactive protein and lactate dehydrogenase were elevated on admission and rose further. Plasmatic coagulation was characterized by prolonged partial thromboplastin time and reduced prothrombin time. Abdominal computed tomography showed an enlarged spleen with irregular hypodense areas, indicating splenic infarctions. Enlarged lymph nodes were noted at the paraaortic region and in the splenic hilum. Diagnosis: As the patient's condition deteriorated from day to day a diagnosis had to be enforced. Splenectomy was thus performed which confirmed the CT findings of numerous infarcted areas. A marginal zone lymphoma was found within the splenic hilar lymph nodes. High titer serum antibodies against cardiolipin confirmed the diagnosis of secondary antiphospholipid syndrome (APS). Treatment and course: Oral anticoagulation with phenprocoumone was started; in addition, chemotherapy with rituximab, cyclophosphamide, vincristin and prednisolone (R-CHOP) was initiated. Despite clinical recovery serological markers of APS remained elevated. The lymphoma recurred only six months after chemotherapy had been completed, and the patient died two months later. Conclusion: Because of its potentially fatal consequences anticoagulation and treatment of the underlying disease are crucial in secondary APS.