Abstract
A statistical study of 128 cases of autoimmune hemolytic anemias, serologically followed up in the same laboratory, led to some conclusions on classification, prognosis and treatment.
Five forms were distinguished: 1. Idiopathic autoimmune hemolytic anemia with warm autoantibodies (IAHA-wa) was the most frequent form (65 per cent of the cases). It was observed in all peroids of life. A slight predominance among females was noted. This form was characterized clinically by a generalized or conjunctival icterus and a moderate splenomegaly. Hematologically a macrocytic normochromic anemia was present and serologically warm incomplete autoagglutinins, often nonspecific, or sometimes specific for a group antigen, were detected. Hemolysins were not found. The average course was 13 months followed by recovery (54 per cent) and 16 months followed by death (46 per cent). These two groups of patients were compared extensively. No differences in the age, sex, blood group and severity of the initial anemia were noted. A low reticulocyte count, leukopenia and association with thrombocytopenic purpura were more frequent in fatal cases. Tile persistence of a positive indirect Coombs test was unfavorable. Those with free antibodies in the plasma were the most serious. Fifty-two per cent of fatal cases had a positive indirect Coombs test. Of those who recovered, 18.5 per cent had this serologic finding. Transfusions were usually done at the begining of the disease. The efficacy of corticosteroid hormones was confirmed; the percentage of recoveries has risen since this therapy has been used fully (37.5 to 70 per cent). Early or late splenectomy had no influence on final desensitization (long-term effect), but led in 58 per cent of the cases to good clinical results (immediate effect). The spleen destroys red cells coated with noncomplement-fixing antibodies, so that splenectomy leads to compensation for the anemia. One must also describe the acute autoimmune hemolytic anemia observed especially in children, in which warm hemolysins could be detected at the very early stage of the disease. Complement was diminished or absent and the serum often showed anticomplementary activity. Complete recovery was rapid.
2. Symptomatic autoimmune hemolytic anemia with warm autoantibodies (SAHA-wa) accompanied mostly malignant conditions of the lymphocytic or reticuloendothelial systems as well as more rarely disseminated lupus erythematosus (17.6 per cent of the cases). Except for the causal disease, these cases were not different from IAHA-wa and their prognosis depended on the prognosis of the causal disorder.
3. Idiopathic autoimmune hemolytic anemia with cold antibodies (IAHAca) was less frequent (7.7 per cent of the cases). Clinically it was characterized by the rarity of splenomegaly, the chance of cold paroxysmal hemoglobinuria (1 case out of 10) and of Raynaud’s syndrome (1 case out of 10), and serologically by the presence of a cold acid-hemolysin (7 cases out of 8) along with an increased titer of complete agglutinins. Complement was diminished or absent. A positive Coombs test was possibly due to complement fixation. The course of these forms seemed to be very chronic: Nine cases of the 10 of the series were in progress for an average of 26 months, without any apparent trend to densensitization. The action of hormone therapy was less striking than in the warm variety. Splenectomy was probably not effective (1 case), since the red cells sensitized by complement-fixing antibodies were mainly recovered by the liver.
4. Symptomatic autoimmune hemolytic anemia wiith cold antibodies (SAHA-ca) was divided into two distinct forms: (a) one symptomatic of a malignant condition of the blood of the same type as in SAHA-wa (7 per cent of cases). The serology was identical to that of IAHA-ca. The prognosis was determined by that of the causal disease; (b) one symptomatic of a virus or a presumed virus infection (3.9 per cent of cases). Here an acid-hemolysin usually accompanied a very high complete cold agglutinin titer. Complete recovery occurred rapidly.
In all cases with cold antibodies exposure to cold had to be carefully avoided. In cases of hemolysins, washed red cells had to be used for transfusions.
Autoimmune myelofibrosis associated with autoimmune hemolytic anemia successfully treated with ruxolitinib
