Cases reported "Anemia, Hemolytic"

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1/110. ceftizoxime-induced hemolysis due to immune complexes: case report and determination of the epitope responsible for immune complex-mediated hemolysis.

    BACKGROUND: Several occurrences of immune complex-mediated, cephalosporin-induced intravascular hemolysis have been reported. This report describes the first case of hemolytic anemia caused by an immune-complex mechanism associated with ceftizoxime and delineates the epitope responsible for hemolysis. CASE REPORT: The patient's serum was tested for antibody that reacted with five penicillins and 30 cephems (all types of cephalosporins) by using protocols to detect drug-adsorption and immune-complex mechanisms. The patient's antibody that formed immune complexes with ceftizoxime reacted with 10 of 30 cephems. These 10 drugs were classified as oxime-type cephalosporins, which have a common structural formula consisting of [(Z)-2-(2-amino-4-thiazolyl)-2-methoxyiminoacetoamido] at the C7 position on 7-aminocephalosporinic acid with or without substitution at the C3 position. CONCLUSION: The patient's antibody recognized a common structure in 10 oxime-type cephalosporins, and immune complexes formed by the antibody specifically or nonspecifically bound to red cell membranes. Therefore, when intermittent antibiotic therapy is required, as in this case, care should be taken in antibiotic selection to avoid drug-induced hemolytic anemia. In addition, when this type of hemolysis is observed, tests for antibody that reacts by adsorption and immune-complex mechanisms should be performed against penicillins and cephems to select antibiotics not showing a cross-reaction.
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2/110. infectious mononucleosis.

    infectious mononucleosis is a unique disease in its hematologic aspects; it is different from the frequently occurring acute microbial diseases in that it affects primarily the reticuloendothelial system; and it is interesting serologically because of the heterophil antibody reaction, as well as the multiplicity of antibodies which may be produced. The diagnosis should be suspected clinically before hematology is reported - by remembering the prototypes. In fact, a patient between 16 and 25 years old complains of sore throat and fever is more likely to have infectious mononucleosis than another disease; and if - in addition - he is jaundiced, a diagnosis of infectious mononucleosis is almost certain. Finally, a negative result of treatment with corticosteroid has the diagnostic significance mentioned above. Positive effect of treatment has no diagnostic significance.
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3/110. Severe inclusion body beta-thalassaemia with haemolysis in a patient double heterozygous for beta(0)-thalassaemia and quadruplicated alpha-globin gene arrangement of the anti-4.2 type.

    We describe a new case of an association of alpha-globin gene quadruplication of the anti-4.2 type with beta(0)-thalassaemia. The patient, a young woman of mixed Brazilian-Portuguese origin, suffered from chronic haemolytic anaemia with splenomegaly. bone marrow supravital staining with brilliant cresyl blue and electron microscopy studies showed large inclusion bodies in about 3% of erythroblasts. Upon immunofluorescent staining these inclusions reacted with a monoclonal antibody to alpha- but not to beta-globin. Analysis of alpha-globin cluster by Southern blotting showed the presence of pathologic fragments specific for the anti-4.2 alpha-globin gene quadruplication. Alpha/beta mRNA ratio was higher than in cases combining alpha-globin triplication and beta(0)-thalassaemia or in cases of beta(0)-thalassaemia heterozygous state alone (18, 14.7 and 10.1 respectively). Our data confirmed the hypothesis that the clinically detectable haemolysis in this beta(0)-thalassaemic patient was due to an unusually high amount of precipitated alpha-globin in erythroid precursors. This considerable excess of alpha-globin chains was due partly to the beta-globin deficit caused by the presence of the beta(0)-thalassaemic gene, but also to the presence of 6 active alpha-globin genes resulting from alpha-globin gene quadruplication in one chromosome.
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4/110. Primary adrenal non-Hodgkin's lymphoma associated with autoimmune hemolytic anemia: a case diagnosed by ultrasound-guided fine needle biopsy.

    Primary localized adrenal non-Hodgkin's lymphomas are extremely rare. Only 28 observations have been reported so far, all with a very poor prognosis: a median survival of 12.5 weeks. The authors report the case of a 78-year-old male with primary non-Hodgkin's lymphoma of the right adrenal gland. disease onset was characterized by severe autoimmune hemolytic anemia, and diagnosis was made by echo-guided biopsy. The patient was admitted to our Department with severe autoimmune hemolytic anemia; the hemoglobin value was 6.5 g/dL and both indirect and direct Coombs' tests were positive. Steroid treatment with methylprednisolone 2 mg/kg/day did not improve the hemolytic process. Abdominal ultrasound examination disclosed a right hypoechogenic suprarenal mass of 10 x 9 cm; imaging techniques such as computed tomography and magnetic resonance imaging were not useful in the etiologic diagnosis of the right suprarenal mass; fine needle aspiration and tissue-core biopsy revealed low-grade non-Hodgkin's lymphoma. Staging procedures, including clinical examination, total body computed tomography scan, bone-marrow biopsy, gallium scan, abdominal magnetic resonance imaging, did not disclose other sites of involvement and strongly supported a diagnosis of primary non-Hodgkin's lymphoma of the right adrenal gland. Hormone assays were within normal limits. The patient was treated with chemotherapy, cyclophosphamide-vincristine-prednisone regimen, with good regression of the adrenal mass after 6 courses, and normalization of hemoglobin level and negativity of Coombs' tests. The importance of this case lies in the very rare occurrence of this disease, its association with autoimmune hemolytic anemia, the diagnosis made by ultrasound-guided biopsy, and good response to treatment with respect to cases reported in the literature. The patient remains in clinical remission 12 months after onset of the disease.
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5/110. amyl nitrite induced acute haemolytic anaemia in hiv-antibody positive man.

    Volatile nitrites (amyl and butyl) are popular recreational drugs, especially in the homosexual population. Haemolytic anaemia is a rare complication of nitrite inhalation and occurs when the reducing capacity of the red cell enzymes is overcome by the oxidizing effect of the nitrite. We describe here a patient with hiv infection who developed a profound haemolytic anaemia after repeated inhalation of large quantities of amyl nitrite.
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6/110. Passenger lymphocyte syndrome with severe hemolytic anemia due to an anti-Jk(a) after allogeneic PBPC transplantation.

    BACKGROUND: After allogeneic peripheral blood progenitor cell (PBPC) transplantation, a patient developed a severe hemolytic transfusion reaction due to passenger lymphocyte syndrome. CASE REPORT: A 50-year-old woman with secondary acute myeloid leukemia transforming from a myelodysplastic syndrome received an ABO-compatible PBPC graft from her HLA-identical sister. For prophylaxis of GVHD, the patient was treated with cyclosporine and methotrexate. Eighteen days after the transplant, the patient experienced a severe hemolytic transfusion reaction due to an alloantibody (anti-Jk(a)) produced by donor lymphocytes. RESULTS: The patient was typed as group A, Jk(a ) before transplantation; the donor was typed as group A, Jk(a-). On Day 18 after transplantation, the immunohematologic screening revealed a positive DAT (C3d 3 ) and an alloanti-Jk(a). hemolysis in the patient at that time was indicated by a drop in the Hb and an increase in the LDH level (maximum, 592 IU/L on Day 23). CONCLUSION: The course of hemolysis and the time of appearance of an alloantibody in this patient meet the criteria for passenger lymphocyte syndrome. In most cases, this syndrome is triggered by ABO system antibodies. This is the first reported case of passenger lymphocyte syndrome after PBPC transplantation that was due to an alloantibody that did not belong to the ABO system.
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7/110. Cold agglutinins in low-grade B-cell lymphoma.

    The presence of serum cold agglutinin can be the initial presentation of lymphoproliferative diseases. Conditions with persistent cold agglutinins are a spectrum of diseases that vary from benign lymphoproliferation of the "autoimmune-like chronic cold agglutinin disease" to malignant lymphoma. We report a case of a 72-year-old woman who presented with severe anaemia, hepatosplenomegaly and episodes of peripheral haemagglutination precipitated by cold exposure. The haemoglobin was 5.6 g/dL with a cold agglutinin titer of 1:256 at 4 degrees C and 1:8 at room temperature (30 degrees C). The cold agglutinin showed anti-I specificity and kappa light chain restriction. Peripheral blood showed atypical lymphoid cells with a B-cell immunophenotype. Immunoglobulin gene rearrangement study by polymerase chain reaction (PCR) showed an amplified band at 100 bp, consistent with a clonal proliferation of b-lymphocytes. We believe that our patient had cold antibody haemolytic anaemia as the initial presentation of a low-grade non-Hodgkin's lymphoma. The association of cold antibody haemolytic anaemia with low-grade B-cell lymphoma is unusual.
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8/110. Henna-induced hemolytic anemia and acute renal failure.

    Henna is a traditional cosmetic agent and is used worldwide, especially in the middle east. Its active agent is lawsone (2-hydroxy-1,4-naphthoquinone). Henna is not only applied to hands or hair as a cosmetic agent in traditional ceremonies, but is also applied to the body on lesions in the treatment of seborrheic dermatitis or fungal infections. However, its application over the body or in newborns is rare. Here we report a 27-day-old boy who developed hemolytic anemia and acute renal failure following topical application of henna to his abdomen, intertriginous region and legs to treat diaper rash.
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9/110. Immune hemolysis involving non-ABO/RhD alloantibodies following hematopoietic stem cell transplantation.

    We report two cases of severe alloimmune hemolysis after hematopoietic stem cell (HSC) transplant resulting from an anti-Jk(a). The time course of hemolysis and Jk phenotypes of the donor and recipient in the cases reported suggest that the antibody was produced by donor-derived passenger lymphocytes. Retrospective analysis of the blood bank records of all allogeneic HSC transplant patients at Barnes-Jewish Hospital from 1994 to 1999 suggests that the incidence of alloimmune hemolysis due to incompatibilities involving non-ABO or RhD red cell antigens is very low, approximately 1%. In one patient, the duration of hemolysis was shortened significantly by performing red cell exchange at the first sign of intravascular hemolysis.
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10/110. Haemolytic anaemia in a patient with anorexia nervosa.

    We report on a 17-year-old female patient with severe anorexia nervosa (AN) (body mass index of 9.8 kg/m(2)) who developed hypophosphataemia (serum phosphate 0.4 nmol/l) and subsequent haemolytic anaemia during oral refeeding. Hypophosphataemia due to an increased phosphate uptake may lead to a reduction of erythrocyte adenosine triphosphate. This mechanism is important for the differential diagnosis of haemolytic anaemia in patients with AN. To prevent this complication, phosphate supplementation should be considered in the refeeding of severely malnourished patients.
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