Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic Syndrome

Pancreatic lipomatosis is defined as deposition of fat cells in pancreatic parenchyma. Although the etiology of this condition is still unclear, it is not uncommon in the elderly obese individuals, and a variety of transfusion-dependent hematologic diseases such as β-thalassemia major. Pancreatic li...

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Main Authors: Wei-Ching Lin, Jeon-Hor Chen, Chien-Heng Lin, Wu-Chung Shen
Format: Article
Language:English
Published: Elsevier 2007-08-01
Series:Journal of the Formosan Medical Association
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0929664608600273
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spelling doaj-80b974cf8f164d4db1042cacbbe02c662020-11-24T22:10:09ZengElsevierJournal of the Formosan Medical Association0929-66462007-08-01106867667910.1016/S0929-6646(08)60027-3Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic SyndromeWei-Ching Lin0Jeon-Hor Chen1Chien-Heng Lin2Wu-Chung Shen3Department of Radiology, China Medical University Hospital, Taichung, TaiwanDepartment of Radiology, China Medical University Hospital, Taichung, TaiwanDepartment of Pediatrics, Jen-Ai Hospital, Taichung, TaiwanDepartment of Radiology, China Medical University Hospital, Taichung, TaiwanPancreatic lipomatosis is defined as deposition of fat cells in pancreatic parenchyma. Although the etiology of this condition is still unclear, it is not uncommon in the elderly obese individuals, and a variety of transfusion-dependent hematologic diseases such as β-thalassemia major. Pancreatic lipomatosis associated with transfusion-dependent myelodysplastic syndrome (MDS) has never been reported. We present a 17-year-old male patient with transfusion-dependent MDS. He received transfusion of a total of 345 units of blood in a period of 18 months but without iron chelating agent. Progressive fatty replacement of the pancreas parenchyma was found by a series of computed tomography images over seven hospital admissions due to repeated infections. Bone marrow biopsy revealed hemosiderin deposition. Because of his poor response to induction chemotherapy, stem cell transplantation was suggested, but the patient died of sepsis before the therapeutic procedure could take place. Although most patients with pancreatic lipomatosis have neither clinical symptoms nor abnormal laboratory data, it may cause endocrine and exocrine pancreas dysfunction. In this reported case, mild exocrine dysfunction was noted on the last admission. Clinicians should be cautious of hemosiderin deposition after large amount of blood transfusion and chelating therapy should be given to avoid iron overload.http://www.sciencedirect.com/science/article/pii/S0929664608600273blood transfusionlipomatosismyelodysplastic syndromepancreas
collection DOAJ
language English
format Article
sources DOAJ
author Wei-Ching Lin
Jeon-Hor Chen
Chien-Heng Lin
Wu-Chung Shen
spellingShingle Wei-Ching Lin
Jeon-Hor Chen
Chien-Heng Lin
Wu-Chung Shen
Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic Syndrome
Journal of the Formosan Medical Association
blood transfusion
lipomatosis
myelodysplastic syndrome
pancreas
author_facet Wei-Ching Lin
Jeon-Hor Chen
Chien-Heng Lin
Wu-Chung Shen
author_sort Wei-Ching Lin
title Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic Syndrome
title_short Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic Syndrome
title_full Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic Syndrome
title_fullStr Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic Syndrome
title_full_unstemmed Rapidly Progressive Pancreatic Lipomatosis in a Young Adult Patient with Transfusion-dependent Myelodysplastic Syndrome
title_sort rapidly progressive pancreatic lipomatosis in a young adult patient with transfusion-dependent myelodysplastic syndrome
publisher Elsevier
series Journal of the Formosan Medical Association
issn 0929-6646
publishDate 2007-08-01
description Pancreatic lipomatosis is defined as deposition of fat cells in pancreatic parenchyma. Although the etiology of this condition is still unclear, it is not uncommon in the elderly obese individuals, and a variety of transfusion-dependent hematologic diseases such as β-thalassemia major. Pancreatic lipomatosis associated with transfusion-dependent myelodysplastic syndrome (MDS) has never been reported. We present a 17-year-old male patient with transfusion-dependent MDS. He received transfusion of a total of 345 units of blood in a period of 18 months but without iron chelating agent. Progressive fatty replacement of the pancreas parenchyma was found by a series of computed tomography images over seven hospital admissions due to repeated infections. Bone marrow biopsy revealed hemosiderin deposition. Because of his poor response to induction chemotherapy, stem cell transplantation was suggested, but the patient died of sepsis before the therapeutic procedure could take place. Although most patients with pancreatic lipomatosis have neither clinical symptoms nor abnormal laboratory data, it may cause endocrine and exocrine pancreas dysfunction. In this reported case, mild exocrine dysfunction was noted on the last admission. Clinicians should be cautious of hemosiderin deposition after large amount of blood transfusion and chelating therapy should be given to avoid iron overload.
topic blood transfusion
lipomatosis
myelodysplastic syndrome
pancreas
url http://www.sciencedirect.com/science/article/pii/S0929664608600273
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