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[Detection of platelet autoantibodies. Comparison of an antiglobulin consumption assay with a radioactive direct Coombs test (author's transl)].

131 patients (124 with idiopathic thrombocytopenic purpura) have been investigated for platelet autoantibodies with an antiglobulin consumption assay (ACA) and a direct Coombs test (DCT) using 125I-antiglobulin. The two assays have been simultaneously performed on 165 platelet-suspensions. The results of ACA (expressed as normal or abnormal amounts of platelet bound IgG or PBlgG) and those of DCT (expressed as positive or negative) were concordant in 128 out of 165 cases (77.6 per cent). In 37 of 165 cases (22.4 per cent) the DCT was negative while PBgG was found to be slightly elevated with ACA. There was a significant correlation (r = 0.44) between PBlgG levels and radioactivity index calculated in DCT. The relationship between these data was graphically expressed as a curve constructed on semilog paper meaning that PBlgG levels corresponding to high radioactivity index were more uncertain than those corresponding to lower index. The quantitative expression of DCT would be improved by refering the results to a standard curve and not only to normal platelets. In the case, results of DCT would be probably equivalent to those of ACA.

Autoantibodies↗

[Development of overt hemolytic anemia after splenectomy for thrombocytopenia in Evans syndrome with negative Coombs test].

A 69-year-old man was diagnosed as having idiopathic thrombocytopenic purpura (ITP) in April 2000, and treated with prednisolone (PSL) without effect. Splenectomy performed in June 2000 had only a transient and marginal influence on his platelet count. Two months later, he developed autoimmune hemolytic anemia (AIHA) without Coombs test positivity, and his diagnosis was changed to Coombs-negative Evans syndrome. Treatment with PSL led to recovery of his hemoglobin level, but not his platelet count. Although the mechanism responsible for development of AIHA after splenectomy in this patient with ITP remains unknown, close observation is required for any association with other autoimmune diseases such as SLE.

Anemia, Hemolytic↗

[Familial autoimmune hemolytic animia (AIHA) with negative Coombs test, lymphocytopenia and hypogammaglobulinemia].

A family with several cases of autoimmune hemolytic anemia and dysgammaglobulinemia is described. Two sibling had documented antierythrocyte antibodies, but a third had repeatedly negative Coombs tests. Diminished immunoglobulin-producing cells and a hypogammaglobulinemia were found, but no functional defect of humoral or cell-mediated immunity could be detected.

Agammaglobulinemia↗

Spider bite hemolytic anemia: positive Coombs' test, erythrophagocytosis, and leukoerythroblastic smear.

The full hematologic profile of spider bite hemolytic anemia has not been documented in the literature. The two patients had brisk intravascular hemolysis with direct Coombs' tests positive for complement and with peripheral blood smears showing spherocytosis, erythrophagocytosis, and leukoerythroblastosis. The author's experience, along with that in the literature reviewed herein, suggests that the hemolytic anemia that can follow the bite of the brown recluse spider could be confused with autoimmune hemolytic anemia.

Adult↗

[Gm allotype HLA antigens and T lymphocyte sub-populations in a group of patients having a positive Coombs test under treatment with alpha-methyldopa].

We analysed HLA-A, B, DR antigens and Gm allotypes in 45 Caucasian patients treated by alpha methyl dopa. 30 had a positive antiglobulin direct Coombs test of the pure IgG type and 15 showed no erythrocyte antibody. We found no difference in the HLA and Gm gene frequencies between the 2 patient groups and the normal control group (nb 104). Furthermore we appreciated the T cell subsets in 11 Coombs positive patients. They showed an increase of the helper/suppressor T cell ratio, due to a significant decrease of the suppressor T cell subset. These data are compatible with Kirtland findings which demonstrated a methyl dopa inhibition of suppressor lymphocyte function. However they do not support the hypothesis that genetic factors may have a role in the susceptibility of patients to methyl dopa induced suppressor-cell disfunction.

Adult↗

Chronic myelogenous leukemia with elevated leukocyte alkaline phosphatase, positive indirect Coombs' test, neutrophilic leukocytosis and unusual cytogenetical findings.

An unusual case of chronic myelogeous leukemia (CML) is reported which was characterized by leukocytosis without a shift to the left, elevated leukocyte alkaline phosphatase, positive indirect Coombs' test, anemia and thrombocytosis, as well as the absence of hepatosplenomegaly. The diagnosis of CML was ascertained by the presence of Philadelphia chromosome with translocation of its deleted arms on the short arms on the short arms of a chromosome No. 6. The possible relationship between the chromosomal aberration and the unusual hematological and clinical features of this case is discussed.

Adult↗

[Suitability of monoclonal test sera for determination of blood group markers in positive direct Coombs test].

BACKGROUND: Monoclonal reagents of the IgM type allow to test some red blood cell antigens (K; Jka; Jkb) by the tube centrifugation method at room temperature, for the examination of which the indirect antiglobulin test was necessary in the past. This permits to test antigens also when the direct antiglobulin test is positive, provided the sera do not contain too much supplement. MATERIALS AND METHODS: We tested a choice of monoclonal reagents of different manufacturers (for the ABO system, A subgroups, the Rhesus, MN, Kell, Kidd and Lewis system) for their ability to examine antibody-coated erythrocytes. To recognize false-positive reactions of the reagents, erythrocytes without the particular antigen were coated with an incomplete antibody, and then the reagent was tested with these self-made Coombs control cells. RESULTS: We found no false-positive reactions for all tested anti-A, anti-B, anti-AB, and anti-A1 reagents. Some anti-C, anti-c, anti-E, anti-e and anti-N reagents showed weak false-positive reactions, some anti-H, anti-D, anti-K, anti-Jka, anti-Jkb, anti-Lea, anti-Leb and anti-M reagents stronger false-positive reactions.

Antibodies, Monoclonal↗

[Primary myelofibrosis with positive coombs' test responding to prednisolone].

Primary myelofibrosis was diagnosed in a 43-year-old female in 1991, who gradually became transfusion dependent. There was no evidence suggesting connective tissue diseases except for biological false positive STS. Because her direct Coombs' test was positive and serum haptoglobin level was extremely low (< 6mg/dl), intermediate dose (30mg/day) prednisolone therapy was started. Her hemoglobin level and platelet count increased dramatically to a normal level within 3 weeks after the initiation of prednisolone therapy. Bone marrow biopsy performed 6 months later revealed marked recovery of hematopoiesis. Though the effects of corticosteroid therapy in primary myelofibrosis still remain unclear, this therapy might be useful in the treatment of some groups of patients via the correction of immunological aberrations and/or the decrease of bone marrow fiber.

Adult↗

[Aortic stenosis and mitral regurgitation complicated by hemolytic anemia and positive Direct Coombs test: a case report].

A 83-year-old man was admitted because of heart failure due to severe aortic stenosis and mitral regurgitation secondary to chordal rupture of the anterior leaflet. Mild anemia and elevated serum lactate dehydrogenase were present with reticulocytosis and haptoglobinemia. Direct Coombs test was positive. Coexistence of autoimmune hemolytic anemia was identified, but the main cause of his hemolysis was thought to be mechanical hemolysis due to stenotic valve and/or ruptured chordae because of the presence of red cell fragmentation. The patient successfully underwent double valve replacement. Improvement of anemia was coupled with reduction of the serum lactate dehydrogenase level. Valvular shear stress on the red cells and reduction of red cell deformability secondary to autoimmune hemolytic anemia were thought to be responsible for his hemolysis.

Aged↗

[Determination of the blood group, Rh-D factor, and indirect Coombs' test using the amniotic fluid].

The number of Rh-D sensibilizations provided by pregnancy and delivery has decreased as the result of the preventive measures introduced to the combat this condition. The problem, however, still exists, and to cope with it, the determination of the blood group, Rh-D factor, and the Coombs' test using the amnionic fluid allows a timely preparation for the therapy of the newborn's hemolytic disease. The method of the neutralization technique employed by the authors has proved reliable.

Amniotic Fluid↗

Positive Coombs' test results in two dogs treated with amiodarone.

Effects of amiodarone, an antiarrhythmic drug that is effective in suppressing severe ventricular arrhythmias that are refractory to other antiarrhythmic drugs, were evaluated in 2 dogs with cardiac disease. One dog was a Doberman Pinscher with cardiomyopathy that developed severe thrombocytopenia after receiving amiodarone for 7 months. The second was a Giant Schnauzer with acquired mitral valve degeneration that developed regenerative anemia after receiving amiodarone for 5 months. Results of direct Coombs' tests were positive in both dogs. Adverse effects of amiodarone are numerous; in dogs, the most common adverse effects are anorexia and hepatotoxicosis. Frequent CBC and serum biochemical analyses should be performed when amiodarone is administered with the intent of continuing the drug indefinitely.

Amiodarone↗