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A fatal myeloproliferative syndrome in a family with thrombocytopenia and platelet dysfunction
Author(s) -
Luddy Ruth E.,
Champion Lorraine A. A.,
Schwartz Allen D.
Publication year - 1978
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(197805)41:5<1959::aid-cncr2820410540>3.0.co;2-8
Subject(s) - medicine , myeloproliferative disorders , leukemia , chronic myelogenous leukemia , platelet , immunology , acute leukemia , gastroenterology
Three siblings with a lifelong history of a bleeding disorder and thrombocytopenia died from a myeloproliferative disease. In 2, the terminal event resembled juvenile chronic myelogenous leukemia, and in the third, the diagnosis was acute monocytic leukemia. A family study revealed that the mother and 5 other siblings had a variety of hematologic abnormalities. These included chronic thrombocytopenia, abnormal platelet function, elevated concentrations of HgbF or serum vitamin B 12 , and low leukocyte alkaline phosphatase (LAP) scores either singly or in combination. At the time of study, none had evidence of malignancy. Members of this family have a myeloproliferative disorder that has the potential for terminating in nonlymphocytic leukemia, a combination of events which appears not to have been reported previously.