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The autinglobulin test in autoimmune hemolytic anemia.

The foregoing summarizes what can be learned from a carefully performed antiglobulin test using specific antisera. Clinical syndromes can be more easily considered, and mechanisms of destruction can be more certainly discerned.

Anemia, Hemolytic, Autoimmune↗

A case of primary antiphospholipid antibody syndrome with acute renal failure showing thrombotic microangiopathy.

An 18-year-old woman complained of fever and edema and was admitted to Showa University Hospital for treatment of thrombocytopenia and deteriorating renal function. Laboratory studies demonstrated the presence of lupus anticoagulant (LA), prolongation of prothrombin time, hemolytic anemia, a negative Coombs' test, the absence of antinuclear antibodies, and a normal fibrinogen level. Renal biopsy revealed mesangial hypercellularity, severe endocapillary cell damage, and double contour of the basement membrane walls. Immunofluorescence studies demonstrated focal, peripheral, and finely granular deposits for IgG, IgM, and IgA but were negative for fibrinogen. Electron microscopy showed glomerular capillary loops with subendothelial widening and subendothelial deposits, mesangiolysis, mesangial interposition, and marked luminal narrowing. Biopsy findings were consistent with thrombotic microangiopathy. The patient was treated with hemodialysis, methylprednisolone pulse therapy, and dipyridamole. After treatment, LA disappeared, the prothrombin time became normal, and renal function improved. The renal lesions in this patient were caused by primary antiphospholipid antibody syndrome. This case strongly suggests an important causal relationship between LA and renal lesions in thrombotic microangiopathy. We present this case to promote understanding of the pathogenesis of primary antiphospholipid antibody syndrome.

Acute Kidney Injury↗

Oxaliplatin-induced acute-onset thrombocytopenia, hemorrhage and hemolysis.

BACKGROUND: Oxaliplatin is a novel platinum derivative with established anti-tumor activity in colorectal cancer. Acute-onset hemolytic anemia and thrombocytopenia associated with this drug have rarely been reported and some of these cases have been severe or even fatal. CASE REPORT: This case report describes a patient who developed fever, chills, abdominal and back pain as well as sudden-onset severe thrombocytopenia, upper gastrointestinal bleeding and hemolysis immediately after treatment with oxaliplatin for metastatic colorectal cancer. The reaction appeared during the 14th cycle of chemotherapy. Corticosteroids and antihistamines were administered together with platelet transfusions. Over the next 2 days platelet count improved and the syndrome abated. The patient was discharged 4 days later. Furthermore, the reaction was accompanied by a strongly positive Coombs test and increased TNF-alpha and IL-10 serum levels which returned to normal following anti-inflammatory drug administration. CONCLUSION: Physicians should be aware of the possibility of acute hematological emergencies following oxaliplatin administration.

Acute Disease↗

Worsening of anemia induced by long-term use of captopril in hemodialysis patients.

During long-term captopril administration to hypertensive patients on maintenance hemodialysis, a decrease in hemoglobin, hematocrit, and red blood cell count was observed in 9 out of 12 cases. The maximum decrease in hemoglobin, on the average, was detected after 10.9 months of captopril treatment, when the average decrease was 20.5%. The average daily dose of the drug was 27.6 mg/day throughout the observation period. Mean corpuscular constants, reticulocyte count, serum iron, and total iron-binding capacity did not change significantly. Coombs' test was negative. Neither serum total protein nor body weight changed significantly. Fever, skin rashes, leukopenia, and eosinophilia were not observed. There was no significant correlation between the degree of decrease in hematological indices and the dose of captopril. After discontinuation of captopril administration, anemia improved to pretreatment levels. In 2 of the 3 patients who did not show worsening of anemia, an anabolic steroid was administered in association with captopril. It is suggested that captopril should be used with caution in hemodialysis patients.

Adult↗

Antimitochondrial antibodies after acute myocardial infarction.

The development of anti-heart mitochondrial antibodies (AHMA) after acute myocardial infarction (AMI) has been previously demonstrated in experimental studies. We performed a prospective study to check the incidence, variation curve and specificity of AHMA and AMI by using two different immunological tests: complement fixation and antihuman globulin consumption. Serial venous blood samples were drawn from the patients as follows: for the first 14 days after the event, once every other day; from 14 to 60 days after the acute event, once a week. The development of AHMA was demonstrated in the sera of 15/35 (45.4%) patients with AMI by complement fixation test using mitochondrial antigen obtained from normal human heart tissue compared to 12/33 (36.3%) by using mitochondrial antigen from the infarcted region. The antibodies exhibited cross-reaction with mitochondrial antigens obtained from human skeletal muscle, but did not react with heterologous heart mitochondrial antigens. According to the results of the antihuman globulin consumption test, the AHMA is immunoglobulin M (IgM). None of the patients developed postmyocardial infarction syndrome.

Adult↗

Pure red cell aplasia following autoimmune haemolytic anaemia. Cell-mediated suppression of erythropoiesis as a possible pathogenesis of pure red cell aplasia.

We report here a rare case of pure red cell aplasia (PRCA) following autoimmune haemolytic anaemia (AIHA). After 4 years of AIHA, the patient developed anaemia with severe erythroid hypoplasia and was diagnosed as having PRCA. At this time, Coombs' test was negative and parvovirus infection was not recognized. The patient received azathioprine, and PRCA improved. To determine the pathogenesis of PRCA, in vitro culture studies of erythropoietic and granulopoietic precursors were performed. The patient's serum or IgG did not suppress the colony formation of bone marrow colony-forming units-erythroid (CFU-E) of normal subjects and the patient. In contrast, mononuclear cells in the peripheral blood of the patient significantly suppressed CFU-E of normal subjects. Media conditioned by the patient's mononuclear cells did not significantly suppress CFU-E. The significant production of suppressive cytokine such as tumour necrosis factor or gamma-interferon by the patient's mononuclear cells was not recognized. These findings suggest that the cytotoxic mononuclear cells affected directly the erythropoietic precursors and caused PRCA in this patient. The pathogenesis of PRCA was, therefore, considered to be different from that of the preceding AIHA.

Adult↗

Serological and immunoglobulin studies in autoimmune haemolytic anaemia with emphasis on the nature of biphasic antibodies.

55 cases of autoimmune haemolytic anaemia with positive direct Coombs' test were investigated for the nature of autoantibodies. The studies involved characterization of the autoantibodies on the basis of their thermal amplitude, serological specificity, nature of immunoglobulin and light chain specificity. The findings showed that the autoantibodies could be classified as cold autoantibody (18 cases), warm autoantibody (5 cases) and biphasic autoantibody which reacted at 37 degrees C as well as at 6 degrees C (32 cases). Further classification of these cases was made on the basis of antibody specificity to blood groups, immunoglobulin type in relation to the optimum temperature of their reaction.

Anemia, Hemolytic, Autoimmune↗

Immune response to endotoxin isolated from Bacteroides fragilis in the pregnant guinea pig.

The humoral immune response to endotoxin isolated from Bacteroides fragilis was analyzed in the pregnant guinea pig by means of passive hemagglutination, passive hemolysis, a modified Coombs test, and by crossed immunoelectrophoresis. Pregnant animals were immunized with endotoxin on day 30 of gestation, and antibodies were determined on day 61 in maternal and fetal sera, and in amniotic fluid. The IgG and IgM responses in maternal sera were of the same magnitude as in sera of nonpregnant animals. Fetal sera contained IgG and sometimes IgM, and a higher percentage of incomplete antibodies against endotoxin than maternal sera. Low-titer anti-endotoxin antibodies, partially sensitive to dithiothreitol, were found in amniotic fluid. A statistically significant reduction in the growth of fetuses from endotoxin-immunized females was observed.

Amniotic Fluid↗

Intrauterine exchange transfusion of the fetus under ultrasound guidance: first successful report.

The authors report the first successful case of fetal exchange transfusion performed in Rh disease. The patient was a gravida 3, para 1; a first ultrasound guided intra-funicular transfusion was carried out at 28 gestation weeks (Coombs test: 256, Liley's chart: zone III, fetal hemoglobin: 5.7 g/l). One week later a fetal exchange transfusion was decided because of the appearance of ascites, sinusoidal heart rate pattern, and Manning's score at 4. At 29 weeks of gestation an ultrasound-guided umbilical cord puncture was performed with a 16-gauge Tuohy needle, a catheter for epidural analgesia (Perifix) was inserted through the trocar. A total exchange of +126/-96 cm3 of 50% hematocrit (Hct) concentrated and irradiated blood without leukocyte and platelet was performed increasing fetal hemoglobin concentration from 3.9 to 11.9 g/l. A 2,040-gram girl was delivered by cesarean section at 33 gestation weeks with Apgar score of 7/9/9 and she was given 4 total exchange transfusions in the Neonatal Intensive Care Unit. Two years later her development is normal. This new procedure seems easier than fetoscopy to achieve umbilical cord puncture and avoid excess blood volume while exact correction of anemia is possible. With more concentrated blood (70% Hct) the duration of the procedure is shortened.

Adult↗

Autoimmune hemolytic anemia in Philadelphia positive chronic myeloid leukemia with t(7;14) anomaly after 5 years of interferon alpha treatment.

A 41-year-old woman, Philadelphia positive chronic myeloid leukemia patient, had progressive decline in hemoglobin levels. She had been receiving interferon alpha treatment for five years. Autoimmune hemolytic anemia was diagnosed. Subsequent bone marrow examination revealed translocation t(7;14). She was placed on prednisone treatment. The patient responded to prednisone as treatment for the hemolytic process. The result of a direct Coombs' test remained positive. The patient died shortly after the diagnosis of autoimmune hemolytic anemia. Autoimmune hemolytic anemia should be considered in the evaluation of chronic myeloid leukemia patients with a sudden decrease in hemoglobin levels.

Adult↗