[Severe microangiopathic hemolytic anemia as first manifestation of a CUP syndrome. Rapid hematologic remission under polychemotherapy].

Background: Microangiopathic hemolytic anemia (MAHA) represents a rare manifestation of malignant tumors. This complication, which is mainly lethal within a few weeks, is most commonly observed in association with mucin-producing adenocarcinomas, especially gastric cancer. The pathogenesis of tumor-associated mechanical hemolysis remains unclear. A standard therapy of this hematologic disturbance is not established.

Case report: The authors report on a 51-year-old patient, who presented himself in their clinic with severe anemia, thrombopenia and deterioration of his general condition. Extensive investigations revealed signet-ring carcinoma metastases in the whole vertebral column, pelvis and bone marrow. The primary tumor could neither be located endoscopically nor in imaging examinations. Under palliative chemotherapy with cisplatin, epirubicin and 5-fluorouracil continuous infusion, a rapid objective and subjective improvement could be achieved, which was reflected in normalization of the blood picture and regression of the osteoplastic bone metastases indicating partial remission. 9 months later, the patient died because of a new progression of his disease.

Conclusion: MAHA can be the first manifestation of malignant tumors. Upon the diagnosis of a MAHA, rapid diagnostics should be done to detect a possible underlying tumor, as effective antineoplastic therapy can induce both tumor and hematologic remission.