Childhood idiopathic myelofibrosis

A case report and review of literature

Z. Noor-Fadzilah, Leong Chooi Fun, M. N. Sabariah, S. K. Cheong

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Idiopathic myelofibrosis occurs predominantly in older adults. It is very rarely seen in children. We describe a 3-year-old girl with Down's syndrome who presented with recurrent chest infections associated with anaemia and easy bruising. There was mild hepatosplenomegaly. Full blood picture revealed pancytopaenia with leucoerythroblastosis with absence of circulating blast cells. Repeated attempts at bone marrow aspiration and trephine biopsy were unsuccessful. A trephine biopsy from the tibia showed depressed myelopoiesis and erythropoiesis, megakaryocytes with atypical morphology and increased bone marrow reticulin fibres, findings compatible with idiopathic myelofibrosis. She was treated symptomatically as she was clinically stable. Review of the English literature online yielded 46 reported cases of childhood idiopathic myelofibrosis with variable outcome from spontaneous remission to an indolent course with shortened survival. 6 cases evolved to another malignancy. 5 cases were associated with Down's syndrome.

Original languageEnglish
Pages (from-to)129-132
Number of pages4
JournalMalaysian Journal of Pathology
Volume31
Issue number2
Publication statusPublished - Dec 2009

Fingerprint

Primary Myelofibrosis
Down Syndrome
Bone Marrow
Myelopoiesis
Reticulin
Spontaneous Remission
Megakaryocytes
Erythropoiesis
Needle Biopsy
Tibia
Anemia
Thorax
Biopsy
Survival
Infection
Neoplasms

Keywords

  • Childhood
  • Down's syndrome
  • Idiopathic myelofibrosis
  • Leucoerythroblastic picture
  • Pancytopaenia

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Cell Biology
  • Histology

Cite this

Noor-Fadzilah, Z., Chooi Fun, L., Sabariah, M. N., & Cheong, S. K. (2009). Childhood idiopathic myelofibrosis: A case report and review of literature. Malaysian Journal of Pathology, 31(2), 129-132.

Childhood idiopathic myelofibrosis : A case report and review of literature. / Noor-Fadzilah, Z.; Chooi Fun, Leong; Sabariah, M. N.; Cheong, S. K.

In: Malaysian Journal of Pathology, Vol. 31, No. 2, 12.2009, p. 129-132.

Research output: Contribution to journalArticle

Noor-Fadzilah, Z, Chooi Fun, L, Sabariah, MN & Cheong, SK 2009, 'Childhood idiopathic myelofibrosis: A case report and review of literature', Malaysian Journal of Pathology, vol. 31, no. 2, pp. 129-132.
Noor-Fadzilah, Z. ; Chooi Fun, Leong ; Sabariah, M. N. ; Cheong, S. K. / Childhood idiopathic myelofibrosis : A case report and review of literature. In: Malaysian Journal of Pathology. 2009 ; Vol. 31, No. 2. pp. 129-132.
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