ABSTRACT
BACKGROUND AND OBJECTIVES: Mixed-type autoimmune hemolytic anemia (AIHA) is a rare complication of chronic lymphocytic leukemia (CLL). We report a patient with small lymphocytic lymphoma (phenotypic CLL) who developed symptomatic anemia 3 weeks after her fifth cycle of fludarabine, a T cell immunosuppressant. MATERIALS AND METHODS: An antibody screen and panel, direct antiglobulin test, rapid acid eluate, rabbit erythrocyte stroma (RESt) adsorption, and autoadsorption were performed. RESULTS: Warm and cold autoantibodies were detected. prompt treatment with corticosteroids and minimal blood transfusions led to marked improvement. CONCLUSION: Normally, T cells suppress polyclonal lymphocytes that produce autoantibodies. Suppression of T cells in this patient, in addition to the underlying disease process, may explain this mixed-type AIHA, the first reported case to occur following fludarabine treatment.
Subject(s)
Anemia, Hemolytic, Autoimmune/complications , Antineoplastic Agents/therapeutic use , Leukemia, Lymphocytic, Chronic, B-Cell/complications , Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy , Vidarabine/analogs & derivatives , Autoantibodies/analysis , Coombs Test , Female , Humans , Middle Aged , Temperature , Vidarabine/therapeutic useABSTRACT
BACKGROUND AND OBJECTIVES: The Rh phenotypes hrB- and VS+ are both rare in Whites but more common in Blacks. The high-incidence antigen hrB is present on most red cells that are e+. The presence of VS on red cells is associated with an aberrant expression of e, often called eS. MATERIALS AND METHODS: Using conventional serologic methods, including a monoclonal anti-hrB-like antibody, we studied 65 e+ samples that were apparently hrB-. RESULTS: Of the 65, we found that 59 (91%) were VS+. Recent findings have indicated that in VS+ persons a change from leucine to valine occurs at amino acid 245 of the RHCE-encoded polypeptide. While this residue is predicted to lie within the red cell membrane bilayer, the change presumably affects alanine 226 (that is present when e is expressed) in such a way that eS is seen. CONCLUSIONS: Our findings suggest that the change from e to eS may result in nonexpression or marked depression of expression of hrB that is, perhaps, an epitope of e. While the molecular basis of the hrB-phenotype is not known, it is unlikely that the leucine-to-valine change at residue 245, resulting in the aberrant from of e, explains all hrB-samples. First, hrB-VS+ and hrB- VS- samples must differ. Second, some hrB- VS+ samples are C+, some are C-. Presumably diverse molecular bases are involved in hrB-phenotypes.
Subject(s)
Rh-Hr Blood-Group System/genetics , Black People/genetics , Genome, Human , Haplotypes , Humans , White People/geneticsABSTRACT
A screening program was implemented to identify hrB- donors. D- C+, D-C-, and D+C- samples from African-American donors were typed with multiple examples of anti-hrB and anti-hrB-like, and one example each of anti-V and anti-VS. Of 75 D-C+ donors, 4 (5%) typed as hrB-, and 14 others had weak or variable expression of hrB. Of these 18 individuals, 15 were V-VS+, and 3 were V+VS+. No hrB- sample was found in 90 C- donors, 26 of whom were V+VS+, and 1 was V-VS+. A review of our records of 44 hrB- patients and donors studied earlier revealed that at least 12, and possibly as many as 30, carried r or rs. All hrB- donors found in our screening program had D-C+VS+ RBCs, indicating an overrepresentation of r. Our record review also showed that the presence of r and rs more often results in hrB- RBCs, and that the most effective way to screen for hrB- donors is to type African Americans who have D-C+ RBCs.
