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Favourable response to splenectomy in familial myelodysplastic syndrome
J Abdul-Wahab1, M Naznin, A Suhaimi
1Department of Paediatrics, Faculty of Medicine, International Islamic University of Malaysia, PO Box 141, Kuantan 27510, Malaysia. nasyrah_wahab@yahoo.com
Insights
Familial myelodysplastic syndrome (MDS) is a rare childhood cancer. In a unique case, two siblings with MDS showed stable disease, with one experiencing complete recovery after splenectomy.
Area of Science:
- Hematology
- Pediatric Oncology
- Genetics
Background:
- Familial myelodysplastic syndrome (MDS) is an exceptionally rare hematological malignancy in children.
- Early-onset MDS often suggests an underlying genetic predisposition.
Observation:
- Two siblings from a consanguineous marriage presented with pancytopenia.
- The siblings, aged three and 18 years, were diagnosed with myelodysplastic syndrome.
- Both patients maintained clinical stability during their illness.
Findings:
- Myelodysplastic syndrome in young siblings is a rare occurrence.
- Splenectomy was performed on one sibling.
- The splenectomy procedure appeared to fully resolve the cytopenia in the treated sibling.
Implications:
- This case highlights the potential genetic basis of familial MDS in pediatric patients.
- Splenectomy may be a viable therapeutic option for cytopenia correction in select cases of familial MDS.
- Further research into the genetic factors and treatment modalities for childhood MDS is warranted.
Abstract:
Familial myelodysplastic syndrome occurring at a young age is a very rare childhood haematological malignancy. Two siblings, aged three and 18 years, from a consanguineous marriage, presented with pancytopenia and was subsequently diagnosed to have myelodysplastic syndrome. Both remained clinically stable throughout the illness. Splenectomy appeared to have fully corrected the cytopenia in one of them.
