A case of subacute-onset myelodysplastic syndrome with infection mimicking thrombotic thrombocytopenic purpura: a case report with literature review

Abstract Background Schistocytosis, which is often observed in thrombotic thrombocytopenic purpura (TTP) patients, is a rare complication in myelodysplastic syndrome (MDS) patients. Case presentation We report on a 59-year-old man with progressive anemia and thrombocytopenia. Schistocytes were observed in his peripheral blood; consciousness disturbance, renal insufficiency, and fever were subsequently observed, fulfilling the classic pentad of TTP. He also showed massive hematuria, headache, chest pain, and abdominal pain, and brain magnetic resonance imaging scan demonstrated small infarctions. Cefmetazole was not effective, and there was no focus of infection. Disseminated intravascular coagulation (DIC) score was low at that time. We suspected TTP, and therapeutic plasma exchange was initiated. Simultaneously, antibiotic was changed to piperacillin/tazobactam. After the initiation of those treatments, his general condition improved. ADAMTS13 activity did not decrease significantly; therefore, we stopped the therapeutic plasma exchange. His D-dimer level increased and antithrombin level decreased. Heparin treatment was initiated; however, anemia and thrombocytopenia persisted. Bone marrow examinations revealed dyspoiesis in all cell lines, and a complex karyotype indicated a high risk of MDS. His TTP-like condition relapsed; again, piperacillin/tazobactam was effective. Enterococcus faecalis and Haemophilus influenzae were detected in urine culture later. Infection and/or DIC superimposed on MDS might be involved in the pathophysiology of this case. Conclusions It should be noted that subacute-onset of MDS with infection can mimic TTP