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PRCA with myelofibrosis: an unusual case report
Rakhee Kar1, Manoranjan Mahapatra, Hara P Pati
1Department of Hematology, AIIMS, IRCH Building, New Delhi, 110 029 India.
Summary
Pure red cell aplasia (PRCA) and myelofibrosis rarely co-occur. This case highlights the uncommon progression of PRCA with myelofibrosis to acute monocytic leukemia after 14 months of anemia.
Area of Science:
- Hematology
- Oncology
- Rare Diseases
Background:
- Pure red cell aplasia (PRCA) is a rare disorder characterized by the selective erythroid hypoplasia.
- Myelofibrosis is a myeloproliferative neoplasm often associated with bone marrow fibrosis.
- The co-occurrence of PRCA and myelofibrosis is exceptionally rare, with limited literature documenting such cases.
Purpose of the Study:
- To report an unusual case of co-existing PRCA and myelofibrosis.
- To describe the clinical course and transformation of this rare condition.
- To contribute to the understanding of leukemic transformation in PRCA associated with myelofibrosis.
Main Methods:
- Case report presentation.
- Review of relevant medical literature.
- Clinical and hematological assessment during disease progression.
Main Results:
- The patient presented with PRCA and myelofibrosis.
- After 14 months of transfusion-dependent anemia, the patient developed acute monocytic leukemia.
- This represents a rare documented instance of PRCA with myelofibrosis transforming into acute leukemia.
Conclusions:
- The association of PRCA and myelofibrosis is exceedingly rare.
- Leukemic transformation, particularly to acute monocytic leukemia, is an uncommon but possible progression in patients with PRCA and myelofibrosis.
- This case underscores the importance of vigilant monitoring in patients with rare hematological combinations.
