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Malignant histiocytosis with unusual features. Disseminated intravascular coagulation with severe hyperfibrinolysis, acute polyneuroradiculitis Guillain‐Barré, and a unique chromosome abnormality
Author(s) -
Haubenstock Alexander,
Base Wolfgang,
Bettelheim Peter,
Hinterberger Wolfgang,
Pavelka Margit,
Thaler Erik,
Radaszkiewicz Thaddäus,
Neumann Erich,
Schnedl Wolfgang,
Majdic Otto,
Lechner Klaus
Publication year - 1984
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19840401)53:7<1574::aid-cncr2820530725>3.0.co;2-b
Subject(s) - medicine , disseminated intravascular coagulation , hyperfibrinolysis , hepatosplenomegaly , vincristine , pathology , cyclophosphamide , malignant histiocytosis , prednisolone , histiocytosis , chemotherapy , gastroenterology , histiocyte , coagulopathy , disease
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.