A Clinical Study on Paroxysmal Nocturnal Hemoglobinuria in Korea

Choi, Hee Jung; Lee, Soon Nam

Abstract

BACKGROUND
Paroxysmal nocturnal homoglobinuria is an uncommon acquired hemolyticanemia and characterized by increased sensitivity of erythrocytes to the lytic action of comptement system, developing intravascular hemolysis. PNH is complicated by anemia, infectionand thrombosis. The more prolonged survival comparable to literature was noted and so, weperformed this study. METHOD: The previously reported twenty nine cases and six cases were collected at EwhaWontons University Hospital from Dec.1979 to Dec.1994 and were analyzed for clinical characteristics and progress. RESULT: 1) The age distribution was ranged from 16 to 61 years with mean of 30 years andmale to female ratio was 1.5 : 1. The duration from the onset of symptoms to diagnosis wasranged from 3 days to 40 years and above 5 years was 22.9%. The subjective symptoms attributable to anemia were in 13 cases, to dark urine after sleep in 10 cases, to jaundice in 5 cases,to abdominal pain in 3 cases, to hemorrhage in 4 cases. The various diagnoses made beforePNH were aplastic anemia in 14 cases, hernolytic anemia in 5 cases, liver disease in 4 cases,iron deficiency anemia in 3 cases, idiopathic thrombocytopenic purpura in 1 cases, reversedcases from aplastic anemia to PNH in 2 cases. 2) Laboratory data showed ; (1) anemia(Hg below 12g/dL) in 35 cases with mean of 7.3g/dL. (2) leukocytopenia(below 4,000/mm3) in 5 cases, leukocytosis in 1 case with mean 4,100/mm3 except patient with chronic myelocytic leukemia. (3) thrombocytopenia(below 150,000/mm3) in 10 cases with mean 106,000/mm3. (4) reticulocytosis(over 2.0% ) in 20 cases with mean 4.3%. (5) Bone marrow examination revealed hypocellular in 2 cages, normocellular in 4 cases, hypercellular in 3 cases and erythroid hyperplasia in all cases. (6) positive Ham's test in 13 cases among 14 cases. (7) positive sucrose hemolysis test in 10 cases anions 10 cases. (8) decreased serum haptoglobin in 2 cases among 3 cases. 3) The treatment was consisted of blood transfusion in 26 cases among 35 cases, especiallywashed RBC in 13 cases, iron in 14 cases, folic acid in 10 cases, dextran in 5 cases, busulfanin 1 case, corticosteroid in 26 cases, androgen in 15 cases and anticoagulant in 3 cases. 4) The observed complications were infection in 3 cases, cerebral infarct in 2 cases andacute renal failure in 2 cases. 5) The nineteen patients were still alive, four patients dead, ten patients were lost duringfollow-up period. The duration of most prolonged survival case was 41 years 10 months. Theywere fo11owed from 0.3 to 115 months.
CONCLUSION
In analyzing the characteristics of PNH patients and prolonged survivals, thedata reported suggest, although the laboratory abnormalities persisted in the prolonged survival,the prognosis will be good if conservative tretment and prevention of complication is tried.