A fatal myeloproliferative syndrome in a family with thrombocytopenia and platelet dysfunction

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 a...

Full description

Saved in:
Bibliographic Details
Published in:Cancer 1978-05, Vol.41 (5), p.1959-1963
Main Authors: Luddy, Ruth E., Champion, Lorraine A. A., Schwartz, Allen D.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Alkaline Phosphatase - analysis
Blood Platelet Disorders - complications
Blood Platelet Disorders - genetics
Child
Child, Preschool
Family Characteristics
Female
Fetal Hemoglobin - analysis
Humans
Infant
Leukemia, Monocytic, Acute - genetics
Leukemia, Myeloid - genetics
Leukocytes - enzymology
Male
Myeloproliferative Disorders - complications
Myeloproliferative Disorders - genetics
Pedigree
Thrombocytopenia - complications
Thrombocytopenia - genetics
Vitamin B 12 - blood
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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 B12, 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.
ISSN:0008-543X
1097-0142
DOI:10.1002/1097-0142(197805)41:5<1959::AID-CNCR2820410540>3.0.CO;2-8