Changing Complement Sensitivity and PNH Red Cell Population During Clinical Course in a Case of PNH with Hypoplastic Bone Marrow

Abstract

A 57-year-old male was admitted to our department because of palpitation, shortness of breath and pancytopenia on June 18, 1985. On admission, conjunctiva was anemic, and petechiae and ecchymoses were found in the skin. Laboratory findings revealed pancytopenia, a reticulocyte count of 24‰, positive Ham's test, NAP score of 68, red cell acetylcholine esterase of 0.8 U, hemosiderinuria, LDH 842 WrU, and haptoglobin less than 10mg/dl. Bone marrow showed hypocellularity (0.85×10⁴/cmm). Diagnosis of PNH with hypoplastic bone marrow was made. Although treatment consisting of anabolic steroids and prednisolone was not effective, LDH activity was decreased during the clinical course. To evaluate the abnormal complement sensitive cells (PNH III cells) in the patient, complement lysis sensitivity test was repeated during the clinical course. The result indicated disappearance of the abnormal red cell populations and a change in the sensitivity to complement.
In early January 1986, the patient suffered from anal abscess, and died of cerebral hemorrhage on Febrary 16, 1986.