Fig. 3

Clinical course of the patient. The patient’s central nervous system lesions disappeared completely on day 143 after treatment with methylprednisolone pulse therapy, cyclophosphamide administered intravenously, tacrolimus, and etoposide. However, he experienced a recurrence of the lesions and ultimately died of sepsis on day 348. Laboratory data show that from day 0 to day 80 his white blood cells and platelets decreased and increased. His creatine kinase levels reached a normal range at an early stage, and his soluble interleukin-2 receptor and lactate dehydrogenase levels, biomarkers of malignant lymphoma, were almost normal throughout. In the early stage, we could not distinguish his central nervous system lesions from central nervous system infectious disease owing to hemophagocytic lymphohistiocytosis with dermatomyositis; therefore, we administered meropenem and acyclovir. In the first 5 months, we administered sedatives, such as propofol and dexmedetomidine, and antiepileptic drugs, such as phenytoin and levetiracetam. Ara-c cytarabine, CK creatine kinase, CSF cerebrospinal fluid, DEX dexamethasone, IL-6 interleukin-6, IVCY cyclophosphamide administered intravenously, IVIG intravenous immunoglobulin, LDH lactate dehydrogenase; MA therapy combination therapy with methotrexate and cytarabine, MKC megakaryocyte, mPSL methylprednisolone, MTX methotrexate, NCC nuclear cell count, PLT platelet, PSL prednisolone, sIL-2R soluble interleukin-2 receptor, WBC white blood cell