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Clinical Chemistry, Vol 18, 459-461, Copyright © 1972 by the American Association for Clinical Chemistry
1 South African Medical Research Council/University
of Cape Town Renal-Metabolic Research Group, Department of
Medicine, Cape Town University Medical School, and Groote
Schuur Hospital, Cape Town, Republic of South Africa.
This report deals with the case of a 64-year-old white woman with long-standing Felty’s syndrome who developed symptomatic porphyria during the last three years of her life. Clinical and biochemical findings were typical of those for symptomatic porphyria, except for lack of exposure to alcohol and the absence of hepatic siderosis. The course of this patient was dominated by repeated infections, which were undoubtedly related to the persistent neutropenia and the multiple portals of infection provided by the porphyric skin lesions. Splenectomy and steroid therapy led to a brief increase in the leukocyte count. Despite the apparent effectiveness of methenolone therapy in returning the leukocyte count toward normal, she died as a result of a severe pulmonary infection, and at autopsy a lung abscess was demonstrated. Daily porphyrin excretion progressively declined during the two periods of hospitalization, but cutaneous porphyric lesions continued to appear, although with less intensity. The large amount of porphyrin excreted in the early phases provided abundant material for the intensive investigations reported in Part II of this report.
Submitted on August 9, 1971
Accepted on November 29, 1971
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