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[Polycythemia vera terminating in myelodysplastic syndrome]
S Kimura1, N Uoshima, R Tanaka
1First Department of Internal Medicine, Kyoto Prefectural University of Medicine.
[Rinsho Ketsueki] the Japanese Journal of Clinical Hematology
|January 1, 1990
Summary
This case study details a patient who developed myelodysplastic syndrome (MDS) after treatment for polycythemia vera (PV). The secondary MDS showed complex chromosomal abnormalities and was unresponsive to vitamin D3 therapy, leading to sepsis.
Area of Science:
- Hematology
- Oncology
- Medical Genetics
Background:
- Polycythemia vera (PV) is a myeloproliferative neoplasm.
- Alkylating agents like carboquone and busulfan are used in PV treatment.
- Secondary myelodysplastic syndrome (MDS) can arise after chemotherapy.
Observation:
- A 77-year-old male with a history of PV treated with carboquone and busulfan developed MDS 8 years later.
- Peripheral blood showed pancytopenia, and bone marrow exhibited hypercellularity with trilineage abnormalities.
- Cytogenetic analysis revealed complex chromosomal abnormalities involving chromosomes 5 and 7.
Findings:
- The patient was diagnosed with secondary MDS, likely induced by alkylating agents.
- Treatment with 1,25(OH)2 vitamin D3 was ineffective, with a subsequent increase in myeloblasts to 14.4%.
- The patient's condition progressed despite supportive care.
Implications:
- This case highlights the risk of secondary MDS following chemotherapy for PV.
- Complex cytogenetic abnormalities, particularly involving chromosomes 5 and 7, are associated with poor prognosis in MDS.
- Further research is needed to explore effective therapeutic strategies for chemotherapy-induced MDS.