Blood, 1951, Vol. 6, No. 2, pp. 101-123.
© 1951 American Society of Hematology, Inc.
Chronic Hemolytic Anemia with Erythrocyte Fragility to
Cold and Acid
I. Clinical and Laboratory Data of Two Cases, with Special
Reference to the Cell Abnormality
S. H. LIU M.D.1
1 Department of Medicine, Peiping Union Medical College, Peiping, China.
Two cases previously diagnosed as aplastic anemia were found to have abnormal susceptibility of the red blood cells to hemolysis after chilling and after
addition of acid in the presence of their own as well as control serum. The serum
of the patients had no such effect on control erythrocytes. This erythrocyte
abnormality was accompanied by a severe anemia of the macrocytic type, slight
reticulocytosis, depression of granulocytes and platelets, and erythropoietic
hyperactivity of the bone marrow in the early stages and hypoactivity in the
late stages. Increased blood destruction was evidenced not by jaundice, but by
persistent hyperhemoglobinemia in the serum and increased iron excretion in the
urine, without nocturnal intensification. Chilling in vivo produced hemoglobinuria in Case 1, but only slight evidence of increased blood destruction in Case
2. Administration of large doses of ammonium chloride failed to produce hemoglobinuria in either case, although evidence of an increase in blood destruction was detectable. Both patients had historical and serologic evidence of
syphilis. Autopsy of Case 1 showed, in addition to syphilitic aortitis, marked
hemosiderosis of the kidneys, liver, spleen and lymph nodes.
These were clearly not cases of paroxysmal hemoglobinuria é frigore (Donath-Landsteiner). They resembled cases of chronic hemolytic anemia with nocturnal
paroxysmal hemoglobinuria (Marchiafava-Micheli), but the erythrocyte fragility to chilling, the absence of nocturnal increase in the hemolytic process and
the presence of syphilis rendered the acceptance of such diagnosis difficult.
