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Transient Abnormal Myelopoiesis and Diffuse Hepatic Necrosis in Down's Syndrome with Prominent Bleeding Diathesis
Author(s) -
TSUDA T.,
KOMIYAMA A.,
AONUMA K.,
AKABANE T.,
NAKAYAMA M.
Publication year - 1990
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1990.tb11449.x
Subject(s) - medicine , hepatosplenomegaly , bleeding diathesis , extramedullary hematopoiesis , necrosis , coagulopathy , myelopoiesis , pathology , autopsy , surgery , haematopoiesis , platelet , disease , stem cell , biology , genetics
.The first case known to us with Down's syndrome with transient abnormal myelopoiesis and diffuse hepatic necrosis is reported. The infant had prominent bleeding diathesis and hepatosplenomegaly. She died on the 7th day because of intractable bleeding. The autopsy disclosed extramedullary hematopoiesis and extensive hepatic cell necrosis. Characteristic in our case was the outstanding bleeding diathesis due to coagulopathy.