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Case Reports
. 2014 Sep;30(Suppl 1):363-5.
doi: 10.1007/s12288-014-0412-2. Epub 2014 Jul 2.

Paediatric idiopathic myelofibrosis

Annapurna Saksena  1 , Prerna Arora  1 , Nita Khurana  1 , G R Sethi  2 , Tejinder Singh  1
Affiliations
Case Reports

Paediatric idiopathic myelofibrosis

Annapurna Saksena et al. Indian J Hematol Blood Transfus. 2014 Sep.

Abstract

Pediatric myelofibrosis is a rare disorder. It is usually secondary to other diseases. Rarely, when no underlying cause is found, it is termed idiopathic. We present here, a rare case of idiopathic myelofibrosis in a 10 year old male child. Bone marrow aspirate was dilute. Bone biopsy showed marrow fibrosis, with grade 2-3 reticulin fibres, with no evidence of granuloma, parasite or infilterative disorder. Acid fast bacillus stain was negative. Iliac lymph node biopsy showed reactive sinus histiocytosis with extramedullary hematopoeisis. Thus, diagnosis of pediatric idiopathic primary myelofibrosis was made. Idiopathic pediatric myelofibrosis should be suspected in a child with progressive pallor, hepatosplenomegaly and dry tap on bone marrow aspiration and marrow fibrosis on bone biopsy, after exclusion of secondary causes.

Keywords: Idiopathic; Myelofibrosis; Pediatric.

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Figures

Fig. 1
Fig. 1
Bone marrow biopsy showing marrow fibrosis (H&E × 40×)
Fig. 2
Fig. 2
Bone marrow biopsy showing grade 3 reticulin fibres on reticulin stain
Fig. 3
Fig. 3
Lymph Node biopsy showing extramedullary hematopoisis (H & E × 400×)
See this image and copyright information in PMC

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