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Intrathoracic extramedullary hematopoietic tumor in hemoglobin H disease
Author(s) -
Wu JinHou,
Shih LeeYung,
Kuo TsengTong,
Lan RayShee
Publication year - 1992
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830410412
Subject(s) - medicine , extramedullary hematopoiesis , hereditary spherocytosis , jaundice , asymptomatic , splenectomy , thalassemia , anemia , hemolysis , hemolytic anemia , gastroenterology , surgery , haematopoiesis , genetics , spleen , stem cell , biology
We report a Chinese patient with hemoglobin H (Hb H) disease who developed intrathoracic extramedullary hematopoiesis (EMH) 17 years following splenectomy for a blunt abdominal injury. The patient initially presented with extreme hyperbilirubinemia and multiple intrathoracic tumors. Hb H disease was diagnosed after investigation, and the marked jaundice, which declined gradually after supportive treatment, was attributed to his chronic hemolysis superimposed on an acute hepatitis C virus infection. A biopsy of the intrathoracic tumors revealed an EMH. Intrathoracic EMH, which is usually encountered in patients with β‐thalassemia and hereditary spherocytosis, has never been reported in Hb H disease. In areas where thalassemia is prevalent, EMH should be considered in the differential diagnosis of patients who have chronic anemia with asymptomatic intrathoracic tumor to avoid unnecessary surgical interventions. © 1992 Wiley‐Liss, Inc.