Hemoglobin Zürich. I. A New Hemoglobin Anomaly Associated with Acute Hemolytic Episodes with Inclusion Bodies after Sulfonamide Therapy

Abstract

A new abnormal hemoglobin was observed in 15 members over four generations of a large Swiss family and has been termed "Hemoglobin Zürich." The discovery of this hemoglobin was prompted by a severe hemolytic crisis in two members of the family after sulfonamide therapy. During this episode, virtually all erythrocytes and reticulocytes contained a single large inclusion body which was visible with Giemsa and brilliant cresyl blue stains. Outside the hemolytic episode, the erythrocytes revealed no morphologic abnormalities. The results of enzyme studies were all within normal limits. The association of a hemoglobinopathy with a drug-induced inclusion body anemia without any demonstrable enzyme defect is a new entity. The anomalous hemoglobin is inherited as a dominant character and affects both sexes. Thus far, only the heterozygous form has been observed.