A haemolytic anaemia occurring almost exclusively in persons of Negro ancestry, chiefly characterised by the presence of sickle-shaped erythrocytes and a severe degree of anaemia. Women more often affected than men.
Onset takes place early in life after normal haemoglobin is replaced with sickle-cell haemoglobin. Symptoms include increased fatigability with sudden increase in weakness, severe pain in joints and elsewhere in extremities. Abdominal manifestations may resemble an acute abdomen, and occasionally well-marked neurologic features with changes in the spinal fluid. Physical manifestations include jaundice, enlarged liver, spleen, and lymph glands with cardiomegaly and valvular murmurs. Inheritance is autosomal intermediate.
See also Dresbach's anaemia, or elliptocytosis, under Melvin Dresbach, American Physician, 1874-1946.
- J. B. Herrick:
Peculiar elongated and sickle-shaped red corpuscles in a case of severe anemia.
Archives of Internal Medicine, Chicago, 1910, 6: 517-521.
Transactions of Association of American Physicians, 1910, 25: 553-561.