Abstract
R. H., a 58 aged housewife was admitted in our hospital on June 11, 1962, with complaints of photosensitivity, marked anemia, jaundice and hepato-splenomegaly. Laboratory findings revealed porphyrinuria, hyperchromic macrocytic anemia with myeloblastoid-cells in peripheral blood and megaloblastoid-cells in the bone marrow, marked increase of direct and inderect bilirubin, reticulocytosis, and shortened lifespan of erythrocytes. The liver damage was indicated by BSP retention, positive colloid reactions, and high serum level of GOT and GPT. Porphrin analysis revealed marked increase of protoporphyrin in erythrocytes, plasma, bone marrow and stool. A considerably large number of flourescing normoblasts were seen in bone marrow. The patient died of hepatic coma with pneumonia on January 10, 1965, after 3.5 years treatment by blood transfusion and various drugs, repeating improvement and aggravation of the symptoms. Autopsy studies showed hypoplasia of bone marrow with increased reticulum cells, hemosiderosis of liver and other organs, but there was no sign of leukemia. These findings indicated protoporphyria erythropoietica, which association with hemolytic anemia was reported only in two cases: those of Porter et al. and Masuya et al. In addition to that our case had myeloblastoid-cells in peripheral blood and megaloblastoid-cells in bone marrow for the first case on this line.
