Characterization of direct antiglobulin test‐negative autoimmune hemolytic anemia: A study of 154 cases

Direct antiglobulin test (DAT)‐negative (DAT‐)autoimmune hemolytic anemia (AIHA) is empirically thought to show the same clinical conditions as DAT‐positive (DAT+)AIHA, with the exception of an adequate amount of red blood cell (RBC)‐bound immunoglobulin (Ig)G. We investigated the clinical characteristics of DAT−AIHA in comparison with DAT+AIHA. Of the 582 patients referred to our laboratory with undiagnosed hemolytic anemia, AIHA was clinically diagnosed in 216 patients (DAT−AIHA, n = 154; DAT+AIHA, n = 62). The percentage of reticulocytes, mean corpuscular volume, RBC‐IgG levels, white blood cell count, and total protein (TP) levels were significantly higher in patients with DAT+AIHA than patients with DAT−AIHA. The hemoglobin level was significantly lower in patients with DAT+AIHA. No significant differences between patients with DAT−AIHA and DAT+AIHA existed with respect to age, gender, idiopathic/secondary nature, complications such as Evans syndrome, effectiveness of steroid treatment, or survival rate at 1 year following diagnosis. Patients with DAT−AIHA required significantly lower doses of steroids for maintenance therapy. Based on multivariate analysis of idiopathic DAT−AIHA (n = 110), TP and Evans syndrome were associated with the effectiveness of steroids (adjusted odds ratio [aOR], 1.36/[0.1 g/dl]; 95% confidence interval [CI], 1.01–1.84) and survival at the 1‐year follow‐up (aOR, 0.1; 95% CI, 0.01–0.88). Our results indicate that patients with DAT−AIHA generally suffer milder anemia and hemolysis than patients with DAT+AIHA, respond equally well to steroids, and have comparable survival at 1‐year. Am. J. Hematol. 88:93–96, 2013. © 2012 Wiley Periodicals, Inc.

[1]  U. Jäger,et al.  How I treat autoimmune hemolytic anemias in adults. , 2010, Blood.

[2]  B. Gerber,et al.  [Autoimmune hemolytic anemia]. , 2010, Therapeutische Umschau. Revue therapeutique.

[3]  H. Nakasone,et al.  Diabetes mellitus is associated with high early-mortality and poor prognosis in patients with autoimmune hemolytic anemia , 2009, Hematology.

[4]  K. Ozawa,et al.  Cut‐off value of red‐blood‐cell‐bound IgG for the diagnosis of Coombs‐negative autoimmune hemolytic anemia , 2009, American journal of hematology.

[5]  L. Petz.  Chapter 11 – Management of Autoimmune Hemolytic Anemias , 2004 .

[6]  G. Garratty,et al.  Unusual Aspects of Acquired Immune Hemolytic Anemias , 2004 .

[7]  C. Hillyer,et al.  An automatable format for accurate immunohematology testing by flow cytometry , 2003, Transfusion.

[8]  D. Stroncek,et al.  A preliminary comparison of flow cytometry and tube agglutination assays in detecting red blood cell‐associated C3d , 2003, Transfusion medicine.

[9]  C. Engelfriet.  The immune destruction of red cells * , 1992, Transfusion Medicine.

[10]  G. Garratty,et al.  Immune destruction of red blood cells , 1989 .

[11]  T. Hallberg,et al.  A Comparison between Fc Receptors for IgG1 and IgG3 on Human Monocytes and Lymphocytes Using Anti‐Rh Antibodies , 1985, Vox sanguinis.

[12]  J. Kelton,et al.  Quantitation of red cell‐associated IgG using an immunoradiometric assay , 1984, Transfusion.

[13]  B. Żupańska,et al.  The results of prolonged treatment of autoimmune haemolytic anaemia. , 1981, Haematologia.

[14]  A. Lobuglio,et al.  Drug therapy of autoimmune hemolytic anemia. , 1976, Seminars in hematology.

[15]  B. C. Gilliland.  Coombs--negative immune hemolytic anemia. , 1976, Seminars in hematology.

[16]  H. Chaplin.  Clinical usefulness of specific antiglobulin reagents in autoimmune hemolytic anemias. , 1973, Progress in hematology.

[17]  J. Dacie,et al.  Autoimmune haemolytic anaemias. , 1970, British medical journal.

[18]  H. Chaplin,et al.  Idiopathic acquired autoimmune hemolytic anemia. A review of forty-seven cases treated from 1955 through 1965. , 1967, The American journal of medicine.

[19]  J. Dausset,et al.  The serology and the prognosis of 128 cases of autoimmune hemolytic anemia. , 1959, Blood.

[20]  R. S. Weiser,et al.  The serology of autoimmune hemolytic disease; observations on forty-one patients. , 1957, A.M.A. archives of internal medicine.

[21]  W. Crosby,et al.  Autoimmune hemolytic anemia. I. Analysis of hematologic observations with particular reference to their prognostic value; a survey of 57 cases. , 1957, Blood.

[22]  W. Dameshek,et al.  The present status of treatment of autoimmune hemolytic anemia with ACTH and cortisone. , 1956, Blood.

[23]  W. Crosby.  The clinical aspects of immunologic hemolytic anemia. , 1955, Le Sang.