Paroxysmal nocturnal hemoglobinuria
JCPSP-Journal of the College of Physicians and Surgeons Pakistan. 2008; 18 (8): 512-514
in En
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| ID: emr-102930
Responsible library:
EMRO
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
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Index:
IMEMR
Main subject:
Bilirubin
/
Blood Transfusion
/
Coombs Test
/
Urinalysis
/
Adrenal Cortex Hormones
/
Hemoglobinuria, Paroxysmal
/
Hemosiderin
/
Anemia, Hemolytic
/
Mutation
Type of study:
Case_reports
Limits:
Humans
/
Male
Language:
En
Journal:
J. Coll. Physicians Surg. Pak.
Year:
2008