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Acute lymphoid leukemia following polycythemia vera
M Camós1, F Cervantes, S Montoto
1Postgraduate School of Hematology, Farreras Valenti, Hematology Department, IDIBAPS, Hospital Clínic, Barcelona, Spain.
Leukemia & Lymphoma
|February 26, 1999
Summary
Acute lymphoid leukemia (ALL) is a rare complication of polycythemia vera (PV), particularly after cytotoxic therapy. This case highlights the infrequent B-cell ALL developing years after PV diagnosis and treatment.
Area of Science:
- Hematology
- Oncology
Background:
- Polycythemia vera (PV) is a chronic myeloproliferative disorder with a known risk of evolving into acute leukemia.
- Cytotoxic agent administration is associated with an increased risk of malignant transformation in PV patients.
Observation:
- A 63-year-old woman with a history of PV developed acute lymphoid leukemia (ALL) 6 years after diagnosis.
- The patient had received radioactive phosphorus and hydroxyurea for her PV.
- The ALL was B-cell type, L-3 subtype (FAB classification).
Findings:
- This case represents the eighth documented instance of ALL following PV, with all prior patients having received cytotoxic therapy.
- The median interval from PV diagnosis to ALL diagnosis was 10 years.
- A predominance of B-cell phenotype ALL was observed in these rare cases.
Implications:
- The development of ALL after PV, especially B-cell ALL, is a rare but serious complication.
- The findings suggest a potential link between PV, cytotoxic treatments, and the subsequent development of ALL.
- Further research into the etiological and pathogenetic mechanisms is warranted to understand this association.