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Myelofibrosis: A Cause of Increased Transfusion Requirement in a Child With -Thalassemia Intermedia

Nirupam, Nilay MD; Maheshwari, Anu MD; Rath, Bimbadhar MD; Chandra, Jagdish MD; Kumar, Praveen MD; Basu, Srikanta MD; Nangia, Anita MD

Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e3182278266
Clinical and Laboratory Observations
Abstract

A 10-year-old girl presented with gradually increasing pallor and abdominal distension for 1 year and fever for 6 months. She required 3 packed cell transfusions during this interval. Investigations done revealed that the child had thalassemia intermedia and parents were carrier of β-thalassemia gene. The increased transfusion requirement in this case prompted further investigations, which revealed the presence of myelofibrosis. Case reports of myelofibrosis and myelodysplastic syndromes with acquired hemoglobin disorders exist in literature, especially α-thalassemia. To the best of our knowledge, this is the first reported case of β-thalassemia intermedia in association with myelofibrosis.

Author Information

*Department of Pediatrics, Lady Hardinge Medical College, Kalawati Saran's Children's Hospital

Department of Pathology, Lady Hardinge Medical College, Smt Sucheta Kriplani Hospital, New Delhi, India

Supported by none.

Contributors: All the authors were involved in management of the patient. Nilay Nirupam reviewed the literature and drafted the article. Anu Gulati and Jagdish Chandra critically reviewed the article. All authors approved the final version of the article.

The authors declare no conflict of interest.

Reprints: Anu Maheshwari, MD, Department of Pediatrics, Lady Hardinge Medical College, New Delhi-110001, India (e-mail: dranugulati@gmail.com).

Received February 18, 2011

Accepted April 4, 2011

Copyright © 2012 Wolters Kluwer Health, Inc. All rights reserved.