Paroxysmal nocturnal hemoglobinuria (PNH) with myelodysplastic syndrome (MDS)

Abstract

Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal hematopoietic disorder that may coexist with myelodysplastic syndrome (MDS), leading to diagnostic complexity.

Case: A 45-year-old male presented with generalized weakness and dark-colored urine, with a 10-year history of transfusion-dependent anemia. Examination revealed mild anemia and jaundice. Urine analysis showed hemoglobinuria without red blood cells. Bone marrow study demonstrated hypercellularity with dysplastic changes suggestive of MDS. Flow cytometry confirmed a PNH clone with CD55 and CD59 deficiency.

Diagnosis: PNH with MDS and urinary tract infection.

Management: The patient received supportive care, antibiotics, folic acid, and azacitidine. Eculizumab was indicated but not administered due to financial constraints.

Conclusion: PNH should be suspected in chronic unexplained hemolytic anemia. Coexistence with MDS necessitates detailed hematological evaluation. Limited access to definitive therapy remains a major challenMDS

Bangladesh J Medicine  2026; Vol. 37, No. 2(1): pp. 241-242