Malignant histiocytosis with unusual features. Disseminated intravascular coagulation with severe hyperfibrinolysis, acute polyneuroradiculitis Guillain-Barré, and a unique chromosome abnormality.
The case of a 25-year-old man with the characteristic features of malignant histiocytosis (proliferation of abnormal histiocytic cells with erythrophagocytosis, hepatosplenomegaly, increased serum acid phosphatase, hypercalcemia, and bone pain) is reported. Chromosome studies revealed a near tetraploid karyotype with a pair of marker chromosomes. A few hours after initiation of chemotherapy with cyclophosphamide, Adriamycin (doxorubicin), vincristine, and prednisolone (CHOP regimen), the patient developed an acute ascending paralysis. Cerebrospinal fluid (CSF) findings were consistent with a diagnosis of Guillain-Barré Syndrome. On the next day, disseminated intravascular coagulation (DIC) with severe hyperfibrinolysis occurred. After intensive chemotherapy, complete remission could be achieved.
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects; D001780:Blood Coagulation Tests; D002869:Chromosome Aberrations / Q000150:complications*; D025063:Chromosome Disorders; D004211:Disseminated Intravascular Coagulation / Q000150:complications*; D005342:Fibrinolysis; D006801:Humans; D007621:Karyotyping; D008206:Lymphatic Diseases / Q000150:complications* / Q000188:drug therapy; D008297:Male; D008854:Microscopy, Electron; D011129:Polyradiculoneuropathy / Q000139:chemically induced / Q000150:complications*