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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.
Abstract:
The association of PRCA and myelofibrosis is very rare with only two such cases reported in the literature. Leukemic transformation in myelofibrosis is known but the progression of PRCA to acute leukemia is very rare. We present an unusual case of PRCA with myelofibrosis which after 14 months of transfusion dependent anemia transformed to acute monocytic leukemia.
Insights
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.
