Cancer-related
microangiopathic hemolytic anemia (CR-MAHA) is a
paraneoplastic syndrome characterized by Coombs-negative
hemolytic anemia with schistocytes and
thrombocytopenia. We reviewed and analyzed all cases of CR-MAHA reported since 1979 (the time of the last published review on this topic) according to predefined criteria. We found 154 cases associated with solid
cancer and 14 with
lymphoma. Among the solid
cancers, gastric, breast, prostate, lung, and
cancer of unknown primary (CUP) were most common; 91.8% of
cancers were metastatic, and in 19.4% of solid
cancers CR-MAHA did not occur until recurrence of
cancer.
Lymphoma cases included
Hodgkin disease, angiotropic
lymphoma, diffuse large cell lymphoma, and myeloma. Evaluation of the clinical and laboratory findings revealed that only a minority of cases presented with the features of
thrombotic thrombocytopenic purpura (
TTP) or
atypical hemolytic uremic syndrome (aHUS), with the exception of
prostate cancer, where aHUS was a common presentation. Compared to hereditary or immune
TTP or aHUS,
disseminated intravascular coagulation and pulmonary symptoms were more common in CR-MAHA.
Plasma exchange or fresh frozen plasma was rarely effective except in
prostate cancer patients with aHUS. CR-MAHA responded to antitumor
therapy in many patients with gastric, breast, lung, and CUP
cancers. These patients had a superior survival compared to patients without
chemotherapy. Compared to the prognosis of patients with metastatic
cancer without CR-MAHA, the prognosis of CR-MAHA patients was greatly inferior. There is evidence that some cases of CR-MAHA in
lymphoma are immune mediated.