Melanogenuria in a case of haemochromatosis secondary to sideroblastic anaemia.

IT IS now over 30 years since Sheldon's classical monograph on haemochromatosis, but in spite of much intensive research, many aspects of this condition remain enigmatic. We have recently observed a case of haemochromatosis secondary to sideroblastic anaemia in which the appearance of the clinical signs of haemochromatosis were unusually rapid and paralleled by a dramatic and fatal deterioration in the patient. In the later stages of the disease the patient became excessively pigmented and was observed to pass jet-black urine. Melanogens were identified by the laboratory. In a comprehensive review of the literature we found only six reports of melanogenuria occurring in the absence of melanotic