3 FAMILIES WITH UNSTABLE HEMOGLOBINOPATHIES (KOLN OLMSTED AND SANTA ANA) CAUSING HEMOLYTIC ANEMIA WITH INCLUSION BODIES AND PIGMENTURIA

Nine additional cases of hemolytic anemia due to an unstable hemoglobin are recorded. In addition to hemoglobin Köln, two new hemoglobinopathies, due to hemoglobin Olmsted and hemoglobin Santa Ana, are described. The salient features in each are anemia and pigmenturia with onset in the first year of life, a susceptibility of hemoglobin to oxidative, peroxidative and thermal denaturation, hypochromia and altered osmotic fragility of the erythrocytes. The peculiar dark urine noted in the present cases is shown to be primarily due to a water-soluble pigment with a molecular weight of about 1,300. Its genesis remains obscure. © 1969.