Affiliation:
1. Department of Pathology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
2. Department of Pathology and Laboratory Medicine, Hospital for Sick Children and University of Toronto, Toronto M5G1X8, Canada
Abstract
Massive hepatomegaly is a common finding in hydrops fetalis (HF) arising from hemoglobinopathies. It has been suggested that extramedullary hematopoiesis, which is markedly increased in response to anemia, plays a principal role in hepatomegaly via sinusoidal obstruction and distortion of the intrahepatic architecture. To test this concept, the authors compared 20 cases of α-thalassemia (hemoglobin Bart, 15 cases; hemoglobin H, 5 cases) with 19 cases of HF arising from other causes and 39 control nonhydrops cases. The mean liver weight in α-thalassemia cases was significantly heavier than in control cases and in hydrops fetalis cases due to other causes ( P < 0.01). This was not explainable on the basis of extramedullary hematopoiesis (erythropoiesis or myelopoiesis) or hemosiderin deposition, since cases of HF from other causes, especially related to anemia, showed similar findings to α-thalassemia cases. While these processes no doubt contribute to hepatic weight, the major factor would still be high-output cardiac failure causing hepatic congestion.
Subject
General Medicine,Pathology and Forensic Medicine,Pediatrics, Perinatology, and Child Health
Cited by
4 articles.
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