Intrathoracic extramedullary hematopoietic tumor in hemoglobin H disease.
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 beta-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.
Wu
J H
JH
Division of Hematology-Oncology, Chang Gung Memorial Hospital, Taipei, Taiwan, Republic of China.
Shih
L Y
LY
Kuo
T T
TT
Lan
R S
RS
D000328:Adult; D001706:Biopsy; D001773:Blood Cells / Q000473:pathology; D003937:Diagnosis, Differential; D006411:Hematopoiesis, Extramedullary; D006525:Hepatitis, Viral, Human / Q000150:complications; D006801:Humans; D007565:Jaundice / Q000150:complications; D008297:Male; D013902:Radiography, Thoracic; D013899:Thoracic Neoplasms / Q000473:pathology; D013909:Thorax / Q000473:pathology / Q000503:physiopathology; D014057:Tomography, X-Ray Computed; D017085:alpha-Thalassemia / Q000097:blood / Q000150:complications / Q000503:physiopathology*