Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon hematologic disease
characterized by an abnormal sensitivity of blood cells to the lytic action of serum
complement. Although many reports had been made in foreign literature, only few
scattered cased described and no one had published data derived from clinical
information on a large stories of PNH in this country. This paper presents some clinical
data from total 14 cartes of PNH: 6 from the Severance Hospital, Yonsei University
College of Medicine from December, 1567 to September, 1979, 3 from Wonju Christian
Hospital from November, 1975 to February, 1980, and 5 from the literature reported in
this country since January, 1962. The results obtained were as follows:
1. Age distribution was between 16 and 58 years with mean of 28 yearn. Male and
female were 10 and B respectively with male to female ratio of 2.5:1.
2. Duration from the onset of symptoms to diagnosis was between 17 days and 10
years with mean of 3.4 years and median of 3.5 years.
3. The chief complaints on admission were anemic symptoms in 8 cartes, hemolytic
manifestations in 3 and hemorrhage in 4.
4. The various diagnoses made before PNH were aplastic anemia in 7 cases, iron
deficiency anemia in 3, hemolytic anemia in 2, and liver disease in 3 among 10 patients.
5. Inciting causes of hemolysis were noted in 11 cased: blood transfusion in 5 cartes,
infection in 4, iron preparation in 3, exercise in 2, and pregnancy and menstruation in 1
respectively.
6. The subjective symptoms attributable to anemia were in 14 cartes, to hemorrhage
in 6, and abdominal pain in 4.
7. On physical examination, pallor was noted in 13 cartes, jaundice and mild
hepatomegaly in 5 respectively but no lymphadenopathy. There was no splenomegaly
except 1 patient associated with chronic myelocytic leukemia
8. Hematologic data revealed;
a. anemia(Hgb below 12 gm%) in all cases with mean of 6.7(1.3∼11.8)gm%.
b. reticulocytosis(over 2%) in 10 cartes with mean count of 8.5(0.2∼22.9)%.
c. leukopenia(below 4,000/㎣) in 4 cartes, leukocytosis(over 10,000/㎣) in 8 with mean
count of 5,400(1,800∼15,600) ㎣ except chronic myelocytic leukemia associated case.
d. thrombocytopenia(below 150,000/㎣) in 11 cases, thrombocytosis(over 400,000/㎣) in
1 case(chronic myelocytic leukemia associated patient), and normal range of platelets in
1 with mean of 85,800(23,000∼258,000)/㎣ except chronic myelocytic leukemia associates
one.
9. Other laboratory data showed;
a. decreases leukocyte alkaline phosphatase score in 3 cased out of 3.
b. negative Coomb's teat in direct and indirect in 8 and 7 crises respectively among 8.
c. positive Ham's teat in 12 eases out of 13.
d, positive sugar water teat in 12 cartes among 12.
e. hemosiderinuria in 8 cased out of 10.
f. inulin test was positive in 1 patient
g. increased bilirubin in 7 cases among 11.
h. increased LDH(over 450 mU/ml) in 5 cased among 6.
10. Bone marrow swear demonstrated;
a. hypercellular in 9 cases, normocellular in 2, and decreased cellularity in 3.
b. decreased iron storage in 7 cased among 9.
c. erythroid hyperplasia in 11 cages out of 11.
11. Medical treatments were consisted of b1ood transfusion in 12 eases, adrenal
corticosteroids in 8, androgens in 5, iron preparation in 5, dextran solution in 2, and
folate in 1. Busulfan was administered in a cabs associated with chronic myelocytic
leukemia.
12. The overall therapeutic results were improved in 6 patients, not improved in 3
among which 2 were died of cerebrovascular diseases, and unknown in 5 due to no
follow up.
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