2. PAROXYSMAL NOCTURNAL HEMOGLOBINURIA GENERAL CONSIDERATION
     Paroxysmal nocturnal hemoglobinuria is a rare chronic hemolytic anemia of
variable severity, characterized by rather constant hemoglobinemia and hemosiderinuria and recurrent episodes of acutehemolysis with chills, fever, pain and hemoglobinuria.
     The basic disorder is a membrane defect; hemolysis is produced by interaction
between the abnormal cells and several factors present in normal serum; magnesium,
properdin and complementlike components.
     In traditional Chinese medicine, the disease is termed "deficiency of blood."
CLINICAL MANIFESTATIONS
     The disease is characterized by chronic hemolysis with hemoglobinemia and methemalbuminemia, usually increasing during sleep, regardless of the time when the patient sleeps. During the period of hemolysis sufficient to cause hemoglobinuria, the urine passed on arising is usually brown or reddish brown. Most patients usually have anemia and some patients may have jaundice. But a few patients never have hemoglobinuria and some may have hemoglobinuria after months and years. Spleen and liver may be slightly enlarged. White cell and platelet counts are often decreased and the reticulocyte count is increased. The bone marrow is usually hyperactive but may be hypoplastic. Aplastic anemia occasionally precedes the clinical development
of this disorder.
     The indirect serum bilirubin is elevated. Hemoglobinemia and methemalbuminemia are often present. Haptoglobins are absent. LDH is markedly elevated and the red cell acetylcholinesterase level is low. The intrinsic red cell defect is demonstrated by finding hemolysis on incubation of the patient's red cells in acidified normal serum (Ham test). The sucrose hemolysis ("sugar water") test can be used for screening. Hemoglobin electrophoresis and osmotic fragility are normal and the Coomb's test is negative.
TREATMENT
I. Treatment in Western medicine.
     A. Transfusion. Transfusion of washed, normal red blood cells is needed during
periods of severe anemia. Transfusion of whole blood should be avoided in most
cases, because constituents of normal plasma as well as leukocytes may accelerate
hemolysis.
     B. Androgens. Administration of androgens such as fluoxymesterone, 20 to
30mg daily by mouth, may be beneficial by stimulating erythropoiesis.
     C. Prednisone. Used in severely affected patients for a short period of time.
     D. Administration of iron. For those who develop evidence of iron deficiency.
     Folic acid, usually 5mg daily by mouth, is indicated to meet the increased folate requirement.
II. Treatment in traditional Chinese medicine.
     Herb therapy
     The general rules of treatment in traditional Chinese medicine are to activate
blood, clear pathogenic heat and eliminate the damp with Shi Wu Tang Jia Jian.
     Constituents:
     Chinese angelica 20g
     Unpeeled root of herbaceus peony  30g
     Chuanxiong 12g
     Motherwort 30g
     Capillary artemisia 30g
     Bracken  30g
Selfheal  30g
Cogangrass rhizome  60g
Inkfish bone 15g
Milk veteh  30g
Horsetail 12g
Decoction and dosage is the same as above.