Malignant histiocytosis with unusual features. Disseminated intravascular coagulation with severe hyperfibrinolysis, acute polyneuroradiculitis Guillain-Barré, and a unique chromosome abnormality.

Autor(es): Haubenstock A.; Base W.; Bettelheim P.; Hinterberger W.; Pavelka M.; Thaler E.; Radaszkiewicz T.; Neumann E.; Schnedl W.; Majdic O.


Resumo: 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.


Imprenta: Cancer, v. 53, n. 7, p. 1574-1578, 1984


Identificador do objeto digital: 10.1002/1097-0142(19840401)53:7<1574::AID-CNCR2820530725>3.0.CO;2-B


Descritores: Guillain-Barre Syndrome - Cell ; Guillain-Barre Syndrome - Pathogenesis


Data de publicação: 1984