Childhood idiopathic myelofibrosis : a case report and review of literature.
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 re...
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| Format: | Article |
| Language: | English English |
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Malaysian Society of Pathologists
2009
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| Online Access: | http://psasir.upm.edu.my/id/eprint/13585/ http://psasir.upm.edu.my/id/eprint/13585/1/Childhood%20idiopathic%20myelofibrosis.pdf |
| _version_ | 1848842152862285824 |
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| author | Zulkifli, Noor Fadzillah Leong, Chooi Fun Md Noor, Sabariah Cheong, Soon Keng |
| author_facet | Zulkifli, Noor Fadzillah Leong, Chooi Fun Md Noor, Sabariah Cheong, Soon Keng |
| author_sort | Zulkifli, Noor Fadzillah |
| building | UPM Institutional Repository |
| collection | Online Access |
| description | 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. |
| first_indexed | 2025-11-15T07:54:36Z |
| format | Article |
| id | upm-13585 |
| institution | Universiti Putra Malaysia |
| institution_category | Local University |
| language | English English |
| last_indexed | 2025-11-15T07:54:36Z |
| publishDate | 2009 |
| publisher | Malaysian Society of Pathologists |
| recordtype | eprints |
| repository_type | Digital Repository |
| spelling | upm-135852015-11-25T03:14:46Z http://psasir.upm.edu.my/id/eprint/13585/ Childhood idiopathic myelofibrosis : a case report and review of literature. Zulkifli, Noor Fadzillah Leong, Chooi Fun Md Noor, Sabariah Cheong, Soon Keng 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. Malaysian Society of Pathologists 2009-12 Article PeerReviewed application/pdf en http://psasir.upm.edu.my/id/eprint/13585/1/Childhood%20idiopathic%20myelofibrosis.pdf Zulkifli, Noor Fadzillah and Leong, Chooi Fun and Md Noor, Sabariah and Cheong, Soon Keng (2009) Childhood idiopathic myelofibrosis : a case report and review of literature. Malaysian Journal of Pathology, 31 (2). pp. 129-132. ISSN 0126-8635 http://www.mjpath.org.my/2009.2/index.html English |
| spellingShingle | Zulkifli, Noor Fadzillah Leong, Chooi Fun Md Noor, Sabariah Cheong, Soon Keng Childhood idiopathic myelofibrosis : a case report and review of literature. |
| title | Childhood idiopathic myelofibrosis : a case report and review of literature. |
| title_full | Childhood idiopathic myelofibrosis : a case report and review of literature. |
| title_fullStr | Childhood idiopathic myelofibrosis : a case report and review of literature. |
| title_full_unstemmed | Childhood idiopathic myelofibrosis : a case report and review of literature. |
| title_short | Childhood idiopathic myelofibrosis : a case report and review of literature. |
| title_sort | childhood idiopathic myelofibrosis : a case report and review of literature. |
| url | http://psasir.upm.edu.my/id/eprint/13585/ http://psasir.upm.edu.my/id/eprint/13585/ http://psasir.upm.edu.my/id/eprint/13585/1/Childhood%20idiopathic%20myelofibrosis.pdf |