Paroxysmal Nocturnal Hemoglobinuria

Asad Mahmood; Muhammad Ashraf Sharif; Badar Murtaza

Author(s)

Asad Mahmood Department of Medicine, Combined Military Hospital,Bahawalnagar, Pakistan

Muhammad Ashraf Sharif Department of Pathology, Combined Military Hospital, Bahawalnagar, Pakistan

Badar Murtaza Department of Surgery, Combined Military Hospital, Bahawalnagar, Pakistan

Abstract

Paroxysmal Nocturnal Hemoglobinuria (PNH) literally means to have episodes of hemoglobin in the urine during the night. It is a Coomb's negative rare hemolytic disorder characterized by non-malignant clonal expansion of haemopoietic stem cells due to acquired genetic mutations. A 30 years old male patient presented with 5 years history of transfusion dependent anemia with intermittent episodes of passing dark colored urine in the morning. Blood complete picture showed decreased hemoglobin and reticulocytosis upto 30%. Coomb's test was negative with unconjugated hyperbilirubinemia and markedly raised serum LDH. Urine analysis showed marked hemosiderinuria and flow cytometry revealed 60% RBCs deficient for CD-59, confirming the diagnosis of paroxysmal nocturnal hemoglobinuria. The management of the patient depends on whether anemia is due to hemolysis or as consequence of impaired erythropoiesis. Corticosteroids at a dose of 0.25-1 mg/kg/day was selected as it is amongst the various treatment options in patients with predominant hemolysis.

Publication Details

Page(s) 512-514

DOI DOI not available

Published Journal: Journal of College of Physicians and Surgeons--Pakistan : JCPSP, Volume: 18, Issue: 8, Year: 2008

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Keywords

Paroxysmal nocturnal hemoglobinuria. Hemolysis. Anemia.