Agglutinins ; immunology ; Anemia, Hemolytic, Autoimmune ; Humans ; Immunoglobulin G ; Infant, Newborn

A 34-year-old lady presenting with features of cold agglutinin disease during the course of systemic lupus erythematosus is described. Cold antibody titer was very high (1 in 4096) with specificity for 'I' antigen. Even though she had poor prognostic factors like high titer of cold antibodies with low thermal amplitude, she responded well to prednisolone.
Adult ; Anemia, Hemolytic, Autoimmune/immunology ; Anemia, Hemolytic, Autoimmune/etiology* ; Anemia, Hemolytic, Autoimmune/drug therapy ; Antibodies/analysis ; Case Report ; Female ; Human ; Lupus Erythematosus, Systemic/complications* ; Prednisolone/therapeutic use ; Prognosis

The irregular antibodies are other than antibodies from ABO blood group system because of pregnancies and blood transfusions, clinical autoimmune, drug-induced etc. The irregular IgG and/or IgM antibodies emerge and lead to the difficult identification of clinical blood type, difficult matching of blood, hemolytic disease of newborn, hemolytic transfusion reaction, and so on. It is very necessary to screen and identify the irregular antibodies before blood transfusion or antepartum. For some difficult identifying samples, some detections on serological level should be done firstly, combining with flow cytometry analysis, the difficult-matching patients' genotypes and fetal genotypes were detected by molecular biology techniques such as PCR and PCR-SSP in order to further predict fetal hemolytic disease of newborn and to provide the right blood to difficult-matching patients, and free fetal DNA extracted from maternal plasma. So that some measures must early be taken for clinical prevention and treatment to reduce immune hemolytic reactions. In this paper, the emergence of irregular antibodies, species, laboratory testing, pathogenesis, clinical symptoms and the current research are reviewed.
Anemia, Hemolytic, Autoimmune ; etiology ; immunology ; Erythroblastosis, Fetal ; etiology ; immunology ; Female ; Humans ; Infant, Newborn ; Isoantibodies ; adverse effects ; immunology ; Pregnancy ; Transfusion Reaction

Anemia, Hemolytic, Autoimmune ; complications ; immunology ; pathology ; Antibodies ; immunology ; Female ; Humans ; Lymphoma, Non-Hodgkin ; etiology ; immunology ; pathology ; Male ; Middle Aged

OBJECTIVETo investigate the quantities of bone marrow CD5+ B lymphocytes in the patients with autoimmune hemocytopenia and the relationship between quantities of CD5+ B lymphocytes and clinical or laboratorial parameters.

METHODSQuantities of CD5+ B lymphocytes in the bone marrow of 14 patients with autoimmune hemolytic anemia (AIHA) or Evans syndrome, 22 immunorelated pancytopenia (IRP) patients, and 10 normal controls were assayed by flow cytometry. The correlation between their clinical or laboratorial parameters and CD5+ B lymphocytes was analyzed.

RESULTSThe quantity of CD5+ B lymphocytes of AIHA/Evans syndrome (34.64% +/- 19.81%) or IRP patients (35.81% +/- 16.83%) was significantly higher than that of normal controls (12.00% +/- 1.97%, P < 0.05). However, there was no significant difference between AIHA/Evans syndrome and IRP patients (P > 0.05). In all hemocytopenic patients, the quantity of bone marrow CD5+ B lymphocytes showed significantly negative correlation with serum complement C3 level (r = -0.416, P < 0.05). In the patients with AIHA/Evans syndrome, the quantity of bone marrow CD5+ B lymphocytes showed significantly positive correlation with serum indirect bilirubin level (r = 1.00, P < 0.05). In Evans syndrome patients, the quantity of CD5+ B lymphocytes in bone marrow showed significantly positive correlation with platelet-associated immunoglobulin G (r = 0.761, P < 0.05) and platelet-associated immunoglobulin M ( r = 0.925, P < 0.05). The quantity of CD5+ B lymphocytes in bone marrow of all hemocytopenic patients showed significantly negative correlation with treatment response (tau-b = -0.289, P < 0.05) , but had no correlation with colony forming unit-erythroid (r = -0.205, P > 0.05) or colony forming unit-granulocyte-macrophage colonies (r = -0.214, P > 0.05).

CONCLUSIONSThe quantity of bone marrow CD5+ B lymphocytes in the patients with autoimmune hemocytopenia significantly increases and is correlated with disease severity and clinical response, which suggest that CD5+ B lymphocytes might play an important role in the pathogenesis of autoimmune hemocytopenia.

Anemia, Hemolytic, Autoimmune ; drug therapy ; immunology ; Autoimmune Diseases ; drug therapy ; immunology ; B-Lymphocytes ; classification ; immunology ; Cyclosporine ; therapeutic use ; Drug Therapy, Combination ; Flow Cytometry ; Glucocorticoids ; therapeutic use ; Humans

OBJECTIVETo study the clinical characteristics of autoimmune hemolytic anemia (AIHA) with both warm and cold autoantibodies.

METHODSClinical and laboratory characteristics of 23 cases of AIHA with both warm and cold autoantibodies admitted to our hospital between January 1994 and April 2004 were analyzed retrospectively.

RESULTSIn comparison with the AIHA patients with both warm and cold autoantibodies in the 1980s, the present patients showed the following features: The proportion of this kind AIHA in all AIHA patients increased from 17.6% to 22.1%. There were more females, more primary cases (73.9%), more mixed subtypes of autoantibodies and more of IgM (56.5%). The hemolysis was related with thermal amplitude of autoantibodies and quantity of complement. The response to cortisone and other immunosuppressive drugs was good. The relapse rate was 77.8% in a median follow-up time of 4 months.

CONCLUSIONSAIHA with both warm and cold autoantibodies is related with the type and thermal amplitude of the autoantibody and the activation of complement. It can be treated effectively with combined immunosuppressive therapy, but the relapse rate is high.

Adolescent ; Adult ; Aged ; Anemia, Hemolytic, Autoimmune ; drug therapy ; immunology ; Autoantibodies ; immunology ; Female ; Follow-Up Studies ; Humans ; Immunoglobulin M ; immunology ; Male ; Middle Aged ; Treatment Outcome

A patient with warm autoimmune hemolytic anemia (AIHA) due to predominance of immunoglobulin A (IgA) with an Rh specificity, considered to be the first case in Korea, is described. A 13-yr-old male patient with severe hemolytic anemia showed a weak reactivity (1+) in the direct antiglobulin test (DAT) by using anti-IgG antiglobulin reagent. This finding, however, could not fully explain the patient's severe AIHA. When anti-IgA reagent was used for the DAT, strong reactivity (4+) was observed and free anti-E and anti-c autoantibodies were also detected by anti-IgA and anti-IgG reagents. The patient's hemoglobin began to rise with the administration of steroids. Because RBCs coated with multiple types of immunoglobulins are associated with more severe hemolysis than those only with IgG, the DATs using anti-IgA and other reagents are needed for the correct diagnosis when the result of DAT is not compatible with patient's clinical manifestations.
Adolescent ; Anemia, Hemolytic, Autoimmune/diagnosis/*immunology ; Antibody Specificity ; Autoantibodies/*blood ; Coombs' Test ; Humans ; Immunoglobulin A/*blood ; Korea ; Male ; Rh-Hr Blood-Group System/immunology

There have been a few reported cases of immune hemolytic anemia induced by ceftriaxone. We encountered a patient with immune hemolytic anemia that seemed to be stimulated by a degradation product of ceftriaxone. The patient's direct antiglobulin test was positive only for C3d, and no ceftriaxone-dependent antibodies were detectable in the patient's serum. To demonstrate the presence of the ceftriaxone-induced antibodies, an ex-vivo antigen in urine was obtained from the patient. In addition, we prepared a 1 mg/mL suspension solution of ceftriaxone, and group AB serum as a complement source. Using several combinations of the above reactants, the indirect antiglobulin test was performed. Only the indirect antiglobulin test using the patient's serum with the ex-vivo urine antigen was found to be positive. Other combinations were not reactive. To our knowledge, this is the first reported case in Korea, in which the causative antibody appeared to be stimulated solely by a degradation product of ceftriaxone.
Anemia, Hemolytic, Autoimmune/*chemically induced/*diagnosis/immunology/urine ; Antigens/urine ; Case Report ; Ceftriaxone/*adverse effects ; Cephalosporins/*adverse effects ; Coombs' Test ; Human ; Male ; Middle Age

There have been a few reported cases of immune hemolytic anemia induced by ceftriaxone. We encountered a patient with immune hemolytic anemia that seemed to be stimulated by a degradation product of ceftriaxone. The patient's direct antiglobulin test was positive only for C3d, and no ceftriaxone-dependent antibodies were detectable in the patient's serum. To demonstrate the presence of the ceftriaxone-induced antibodies, an ex-vivo antigen in urine was obtained from the patient. In addition, we prepared a 1 mg/mL suspension solution of ceftriaxone, and group AB serum as a complement source. Using several combinations of the above reactants, the indirect antiglobulin test was performed. Only the indirect antiglobulin test using the patient's serum with the ex-vivo urine antigen was found to be positive. Other combinations were not reactive. To our knowledge, this is the first reported case in Korea, in which the causative antibody appeared to be stimulated solely by a degradation product of ceftriaxone.
Anemia, Hemolytic, Autoimmune/*chemically induced/*diagnosis/immunology/urine ; Antigens/urine ; Case Report ; Ceftriaxone/*adverse effects ; Cephalosporins/*adverse effects ; Coombs' Test ; Human ; Male ; Middle Age

To observe the therapeutic effect and safety of rituximab (anti-CD20 monoclonal antibody) in the treatment of refractory autoimmune hemolytic anemia (AIHA). One AIHA patient refractory to corticosteroid and splenectomy was treated with rituximab, 375 mg/m(2) weekly for four times. Her hemolytic symptoms, adverse effects, hemoglobin (Hb) concentration and other laboratory data were monitored. The results showed that concentration of lactic dehydrogenase (LDH), total bilirubin (TBIL) and indirect bilirubin (IBIL) began to decrease at 11 days after the first dose of rituximab, and decreased to normal range after 45 days. Concentration of hemoglobin increased up to 95 - 100 g/L. The patient remained disease-free 4 months after treatment. No adverse effect was found during the process of treatment. It is concluded that anti-CD20 monoclonal antibody (rituximab) is both effective and safe for the treatment of refractory autoimmune hemolytic anemia.
Anemia, Hemolytic, Autoimmune ; drug therapy ; Antibodies, Monoclonal ; therapeutic use ; Antibodies, Monoclonal, Murine-Derived ; Antigens, CD20 ; immunology ; Female ; Humans ; Middle Aged ; Rituximab