Extramedullary Haematopoietic Tumor Producing
Small Intestinal Intussusception in a Beta-Thalassemia/
Hemoglobin E Thai Boy : A Case Report
SANSANEE WONGWAISAYAWAN,M.D.*,
SUMATE TEERARA TKUL, M.D.***,
SUPORN TREEPONGKARUNA, M.D.****,
RATANAPORN PORNKUL, M.D.**,
SAMART PAKAKASAMA, M.D.****,
PRAWAT NITHIYANANT,M.D.*
Affiliation : * Department of Pathology,
* * Department of Radiology,
*** Department of Surgery,
**** Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand.
AbstractThe authors report a case of beta-thalassemia/hemoglobin E disease with extramedul-
lary haematopoietic tumor which developed at the small intestine and caused intussusception.
A 7 year-old boy with homozygous beta-thalassemia/hemoglobin E presented with recurrent
abdominal pain. The abdominal ultrasonography showed ileo-ileal intussusception with a solid
mass as the leading point. Resection of the ileal segment was performed. Pathological examina-
tion revealed an extramedullary haematopoietic tumor forming an intraluminal polypoid mass,
being the leading point of the intussusception. Extramedullary haematopoiesis in the intestinal
tract is rare. To our knowledge, this is the first case of extramedullary haematopoietic tumor
that produced intussusception of the small intestine in a beta-thalassemia/hemoglobin E patient.
Keywords : Thalassemia, Extramedullary Haematopoietic Tumor, Intussusception , Small Iniestine
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