Autoimmune hemolytic anemia associated with anti-Sc1

A case of autoimmune hemolytic anemia (AIHA) in a young child is described. The hemolysis was resistant to steroid therapy but responded to splenectomy and intravenous immunoglobulin. The autoantibody was shown to be anti-Sc1 by both serologic and immunoblotting techniques. This seems to be the first report of an autoanti-Sc1 detected by immunoblotting and the first example of AIHA in a child caused by autoanti-Sc1.     

Autoimmune hemolytic anemia associated with IgG auto anti-N

A second case of autoimmune hemolytic anemia mediated by an IgG auto Anti-N is described. The patient's red blood cells were sensitized with both IgG and complement. The serum antibody was not inactivated by 2- mercaptoethanol treatment, and reacted by indirect antiglobulin test at 37 C with monospecific anti-IgG. The IgG antibody eluted from the red blood cells and in the serum showed anti-N specificity. The patient was thought to have systemic lupus erythematosus. Following steroid therapy, the hemolytic anemia resolved with disappearance of the anti-N.     

Autoimmune hemolytic anemia

Diagnosis of autoimmune hemolytic anemia (AIHA) requires both serologic evidence of an autoantibody and hemolysis. Based on the characteristic temperature reactivity of the autoantibody to red cell membranes, AIHA is classified into warm AIHA or cold AIHA (cold agglutinin disease and paroxysmal cold hemoglobinuria). Sensitized RBCs are destructed by intravascular and/or extravascular hemolysis. On the basis of etiology, AIHA are classified as idiopathic or secondary. The common cause of secondary AIHA is lymphoproliferative disorders, autoimmune diseases, and infections. The first line therapy of patients with warm AIHA is glucocorticoids and primary treatment for cold AIHA is avoiding cold exposure. The other standard treatments include splenectomy and immunosuppressive drugs. Recently, rituximab, a monoclonal anti-CD20 antibody, has been used in refractory AIHA with excellent responses.     

Autoimmune hemolytic anemia associated exclusively with IgA of Rh specificity

A nine-year-old boy had typical clinical, hematologic and blood group serologic findings of autoimmune hemolytic anemia except for one important exception; with most commercially available broad spectrum anti-human sera the direct antiglobulin tests were negative. With reagents prepared in the laboratory which were found to be relatively less potent in and anti-IgG and IgM activity but which utilize a short period of incubation after mixing with the washed cells, the tests were all clearly positive. If incubation were employed with the commercial reagents, the tests were positive. Further studies showed that the patient's cells were coated exclusively with IgA and that a commercial reagent, although potent in anti-IgG and anti-IgM, was relatively deficient in anti-IgA. It is proposed that incubation compensates for the latter and is cautioned that incubation, with reagents potent in anti-IgG, could lead to false negative reactions with weakly IgG sensitized cells due to antibody surplus prozones of inhibition. This case illustrates that the rare instances of "Coombs negative immune hemolytic anemias" may be based on mechanisms such as those reported here and, that to be ideal, a broad spectrum anti-human serum should have balanced anti-immunoglobulin activities in relation to its incubation time.     

Autoimmune hemolytic anemia with predominance of IgA autoantibody

A 71-year-old woman with severe autoimmune hemolytic anemia had negative direct antiglobulin tests using commercial broad spectrum antisera. Her unwashed cells agglutinated spontaneously in the potentiating medium polyvinylpyrrolidone (PVP) solution or in hexadimethrine bromide (Polybrene) solution. A strongly positive direct antiglobulin test was obtained with specific antihuman IgA sea with or without PVP. In PVP solution, small amounts of IgG, IgM, and complement components were also detected on her cells. The findings illustrate the ability of anti-human IgA to detect autoimmune red blood cell sensitization when other immunoglobulin classes of autoantibody are below detectable levels or absent. Also illustrated is the value of PVP and Polybrene in detecting agglutination in the evaluation of "antiglobulin negative" hemolytic anemia.     

Autoimmune hemolytic anemia and rheumatoid arthritis

The incidence of AIHA in patients with rheumatoid arthritis has not been shown to exceed that in the general population. The prevalence of rheumatoid arthritis in patients with AIHA approximates that in the general population. On the basis of these data, it is extremely difficult to establish a relationship between AIHA and rheumatoid arthritis. The patient we have described had the unusual presentation of AIHA with rheumatoid arthritis. We believe the two remain distinct entities.     

Autoimmune hemolytic anemia in a patient with autosomal dominant chronic mucocutaneous candidiasis

Chronic mucocutaneous candidiasis is a heterogeneous immunodeficiency syndrome characterized by recurrent candidal infections of the skin, nails, and mucous membranes. The syndrome can be associated with autoimmune conditions, especially endocrine disorders. Typically, inheritance is autosomal recessive, and abnormal T-cell-mediated immunity is thought to be the underlying deficit. We describe a 27-year-old man with chronic mucocutaneous candidiasis inherited in an autosomal dominant fashion, in whom both lymphocyte blastogenesis and delayed-type skin reactivity to Candida antigens were normal. Notable features of the case include autoimmune hemolytic anemia, probable hypoparathyroidism, and hypogonadal hypogonadism.     

Autoimmune hemolytic anemia with anti Jka specificity in a patient taking aldomet

Autoimmune hemolytic anemia (hemoglobin 5.2 g, reticulocyte count 31.0 per cent) developed in a 53-year-old hypertensive woman who was taking Aldomet. Both the patient's serum and the eluate prepared from her red blood cells contained an antibody with anti-Jka specificity. Rapid sustained improvement in the anemia occurred after cessation of Aldomet and a two week course of prednisone therapy. Eight months later, anti- Jka was no longer detectable in the patient's serum and the direct antiglobulin test was nonreactive.     

Immune hemolytic anemia associated with teicoplanin

BACKGROUND: Several drugs can cause immune hemolytic anemia. Here a patient who developed hemolytic anemia after treatment with teicoplanin is described. CASE REPORT: Owing to a two-vessel disease, a 68-year-old white man underwent coronary artery bypass grafting. He was readmitted for superficial sternal wound infection and sternal instability. Rewiring was required and worsening anemia characterized the course after the reoperation. Drugs used in the second admission were gentamycin, teicoplanin, paracetamol, and codeine. They were considered as a possible cause of drug-induced hemolytic anemia. RESULTS: The DAT was positive for complement and IgG. Autoanti-e was identified in the patient's undiluted serum sample. The eluate was reactive with all RBCs tested only after adding teicoplanin; when diluted 1:4, anti-e specificity was observed in the presence of teicoplanin. CONCLUSION: To our knowledge, this is the first report of immune hemolytic anemia owing to teicoplanin.     

Autoimmune hemolytic anemia in Kawasaki disease: a case report

A 3-year-old boy presented with the fever, conjunctivitis, rash, and lymphadenopathy diagnostic of Kawasaki disease. Treatment with antibiotics, aspirin, and intravenous immunoglobulin was instituted. The hematocrit decreased from 35 percent on admission to 11 percent by hospital Day 10, and the white cell count had increased from 13.7 to 42 × 10(3) per microL, and the patient had a leukoerythroblastic blood smear. The direct antiglobulin test demonstrated IgG but not complement on the red cell (RBC) surface. An acid eluate reacted (titer of 4) with all panel cells in the antiglobulin phase. Intravenous immunoglobulin from the same lot used for treatment did not contain antibody that reacted with the patient's group O RBCs or a panel of group O RBCs, but did contain IgG anti-A and -B (titer of 4). The patient received a transfusion and was given methylprednisone. The direct antiglobulin test and acid eluate were negative 4 days later. The patient had an uneventful recovery. The distinction between antibody-mediated hemolytic anemia and autoimmune hemolytic anemia is important in the treatment of this disease.     

Acute hemolytic anemia associated with a chlorpropamide-induced apparent auto-anti-Jka

A patient with acute hemolytic anemia and a positive direct antiglobulin test was found to be Jk(a + b +) with anti-Jka in her serum. For 2 weeks prior to admission, the patient had taken chlorpropamide, a hypoglycemic agent. The drug was discontinued upon the diagnosis of hemolytic anemia, and the hemoglobin concentration gradually increased. When chlorpropamide was added to the patient's serum in vitro, it enhanced the reactivity of the anti-Jka, and 40 days posttransfusion, the serum would only react with Jk(a+) red cells when chlorpropamide was present. These findings suggest that a chlorpropamide-dependent antibody with Jka specificity had formed. We do not know why the antibody induced by chlorpropamide reacted preferentially with Jk(a+) red cells.     

Acquired hemolytic anemia associated with IgA anti-e

Acquired hemolytic anemia in a Caucasian female patient with direct antiglobulin test positive only with anti-IgA is described. The patient's serum contained 7S IgA anti-e antibody. The significance of this, from the point of view of broad spectrum antiglobulin sera, is discussed. The patient's serum contained 7S IgA anti-e antibody. The significance of this, from the point of view of broad spectrum antiglobulin sera, is discussed.     

IgA型自身抗体导致的自身免疫性溶血性贫血1例

目的通过使用微柱凝胶卡式法进行Coombs试验,确定导致自身免疫性溶血性贫血患者的自身抗体类型,从而在临床输血过程中注意此种情况,减少输血反应。方法用微柱凝胶卡式法检测患者直接抗人球蛋白试验,分别用微柱凝胶检测卡法和凝聚胺法对患者进行主、次侧交叉配血试验,以明确该类患者在交叉配血时所需采用的试验方法。结果患者DAT试验结果为阳性,具体抗体分型为IgA型;微柱凝胶法交叉配血患者主、次侧交叉配血试验结果均为阴性;凝聚胺法主、次侧交叉配血结果均为阳性。结论单独型IgA抗体的AIHA患者,除微柱凝胶检测卡交叉配血外,还需用凝聚胺法进行交叉配血来共同参考配血结果。     

Autoimmune hemolytic anemia caused by a cold agglutinin with a new specificity (anti-Ju)

A cold autoantibody detected in the serum of a patient with chronic idiopathic cold agglutinin disease and hemolytic anemia is described. The antibody reacted with adult as well as with cord red cells, and its reactivity was strongly diminished by treatment of the cells with neuraminidase and to a lesser degree by treatment with protease. Thus, the specificity of the antibody is distinct from those of all cold antibodies yet described.     

自身免疫性溶血性贫血的分型及临床意义

目的 对自身免疫性贫血（AIHA）患者红细胞膜上结合的不完全抗体进行免疫分型,为临床诊断治疗提供依据。方法 采用天津协和干细胞基因工程有限公司生产的4种抗人免疫球蛋白（IgG、IgA、IgM 和 C3）单价兔抗人球蛋白血清,进行抗人球蛋白分型实验,并对结果 进行分析。结果 931例患者中共检出阳性61例（占6．6％）,其中男性16例,女性45例,男、女性比例为1∶3．6,均以青壮年为主;免疫分型以IgG型最多（32例）,其次为IgG＋C3型（23例）、C3型（5例）,IgM型（1例）。结论 抗人球蛋白分型实验诊断价值优于广谱抗人球蛋白实验,运用抗人球蛋白分型实验对AIHA进行免疫分型,可以更有效地判断溶血程度,为临床诊断治疗提供可靠的依据。     

Autoimmune hemolytic anemia. 2. Drug-induced type.

A variety of commonly prescribed drugs can produce a positive direct antiglobulin test, but the incidence of actual drug-induced hemolytic anemia is low. Of the four mechanisms which may be responsible, only one involves autoantibody production; the others include drug-RBC interaction of the hapten type, immune complex formation, and direct alteration of the RBC membrane.     

Immune hemolytic anemia associated with tolmetin and suprofen

This article reports the first case of immune hemolytic anemia possibly associated with the ingestion of suprofen. The patient suffered from massive hemoglobinuria and acute renal failure. Serologic studies of the patient's serum revealed suprofen-dependent red cell antibodies. However, tolmetin-dependent antibodies were also found in the serum, showing the same properties as the suprofen antibodies and an even higher titer. The patient not only had drug-dependent antibodies in the serum, but also had developed autoantibodies, a phenomenon that has been described for several other drugs. The working mechanism by which suprofen and tolmetin caused immune hemolysis had properties of both the immune complex model and the induction of autoimmunity. Although it was unclear whether the immune hemolytic anemia was the result of suprofen, tolmetin, or cross-reacting antibodies, we feel that suprofen should be added to the list of nonsteroidal anti-inflammatory drugs associated with a positive direct antiglobulin test.