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

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 crisisin two members of the family after sulfonamide therapy. During this episode,virtually all erythrocytes and reticulocytes contained a single large inclusionbody which was visible with Giemsa and brilliant cresyl blue stains. Outsidethe hemolytic episode, the erythrocytes revealed no morphologic abnormalities.The results of enzyme studies were all within normal limits. The associationof a hemoglobinopathy with a drug-induced inclusion body anemia withoutany demonstrable enzyme defect is a new entity. The anomalous hemoglobinis inherited as a dominant character and affects both sexes. Thus far, onlythe heterozygous form has been observed.

Submitted on April 9, 1962 Accepted on June 20, 1962