Myelofibrosis: a cause of increased transfusion requirement in a child with β-thalassemia intermedia.
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.
Department of Pediatrics, Lady Hardinge Medical College, Kalawati Saran's Children's Hospital, New Delhi, India.
Nilay
N
Nirupam
Anu
A
Maheshwari
Bimbadhar
B
Rath
Jagdish
J
Chandra
Praveen
P
Kumar
Srikanta
S
Basu
Anita
A
Nangia
D002648:Child; D017707:Erythrocyte Transfusion; D005260:Female; D006801:Humans; D055728:Primary Myelofibrosis / Q000150:complications* / Q000503:physiopathology / Q000628:therapy*; D017086:beta-Thalassemia / Q000150:complications* / Q000503:physiopathology / Q000628:therapy*