| phenytoin hypersensitivity syndrome (PHS) is a rare delayed hypersensitivity reaction which occurs following exposure to phenytoin sodium. Pulmonary involvement is uncommonly described. Herein is reported the first case of histopathologic bronchiolitis obliterans organizing pneumonia (BOOP) found on open-lung biopsy in a patient with severe PHS. New onset, clinically significant, cold agglutinin disease was also documented. Hemodynamic parameters mimicking sepsis were present in the absence of significant clinical infection. Rapid, dramatic improvement followed high-dose steroid therapy. ( info) |
692/718. Misdiagnosis of post-traumatic splenic rupture in a patient with acute cold agglutinin disease due to Mycoplasma infection. We describe a case of cold agglutinin disease, secondary to mycoplasma pneumoniae infection, which presented with anaemia and abdominal pains in apparent succession to a thoraco-abdominal trauma. An exploratory laparotomy, carried out because of suspected post-traumatic rupture of the spleen, was complicated by a transitory cardiorespiratory arrest. The subsequent and correct diagnosis of the mycoplasmal infection and the cold agglutinins led to specific and successful therapy. The previously unknown hypertrophic cardiomyopathy was a contributing factor to the cardiorespiratory arrest. ( info) |
693/718. Evans syndrome after autologous bone marrow transplant for recurrent Hodgkin's disease. The association of immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) with Hodgkin's disease has been known for many years. Autoimmune cytopenia has also been described in patients that have undergone allogeneic or autologous bone marrow transplantation. We report a rare case of Evans syndrome in a patient 3 years after autologous bone marrow transplantation for recurrent Hodgkin's disease. ( info) |
694/718. factor v inhibitor associated with cold agglutinin disease. An 81-year-old man known to have a stable cold agglutinin syndrome presented with a progressive cerebral hemorrhage. Coagulation tests revealed prolonged APTT and prothrombin time and severely decreased factor v activity, which could not be normalized by mixing with normal plasma. The patient appeared refractory to substitution with fresh-frozen plasma, suggesting the presence of a circulating inhibitor specific for factor v. To our knowledge, this is the first case of a lymphoproliferative disease leading to a cold agglutinin syndrome and a putative inhibitor of factor v. In patients with paraproteinemia presenting with bleeding diathesis, the presence of a circulating inhibitor of a specific coagulation factor must be considered. ( info) |
695/718. Mixed-type autoimmune hemolytic anemia following fludarabine treatment in a patient with chronic lymphocytic leukemia/small cell lymphoma. BACKGROUND AND OBJECTIVES: Mixed-type autoimmune hemolytic anemia (AIHA) is a rare complication of chronic lymphocytic leukemia (CLL). We report a patient with small lymphocytic lymphoma (phenotypic CLL) who developed symptomatic anemia 3 weeks after her fifth cycle of fludarabine, a T cell immunosuppressant. MATERIALS AND methods: An antibody screen and panel, direct antiglobulin test, rapid acid eluate, rabbit erythrocyte stroma (rest) adsorption, and autoadsorption were performed. RESULTS: Warm and cold autoantibodies were detected. prompt treatment with corticosteroids and minimal blood transfusions led to marked improvement. CONCLUSION: Normally, T cells suppress polyclonal lymphocytes that produce autoantibodies. Suppression of T cells in this patient, in addition to the underlying disease process, may explain this mixed-type AIHA, the first reported case to occur following fludarabine treatment. ( info) |
696/718. Essential monoclonal gammopathy with an IgM paraprotein that is a cryoglobulin with cold agglutinin and EDTA-dependent platelet antibody properties. A patient with apparent anaemia and thrombocytopenia caused by a monoclonal paraprotein is described. The patient's serum contained a monoclonal IgM kappa, a cryoglobulin and a cold agglutinin. The cryoglobulin, similar to the serum paraprotein, was a monoclonal IgM kappa. serum was studied to determine the relationship of the cryoglobulin with the cold agglutinin. The cryoglobulin and cold agglutinin were found to be the same paraprotein. Moreover, with absorption and elution techniques the reactivity of the autoantibody with both erythrocytes and platelets was demonstrated. Reports of cryoprecipitable cold agglutinins are rare and therefore this case is exceptional given that the IgM kappa paraprotein was found to be a cold agglutinin which was also reactive with platelets. ( info) |
697/718. Paradoxical haemolysis in a patient with cold agglutinin disease. A patient with classical cold agglutinin disease initially experienced haemolytic episodes during cold exposure. However, with advancing disease cold-induced haemolysis ceased and was substituted with a haemolytic disposition at elevated body temperatures. To investigate this paradoxical development of disease manifestations, we performed a clinical and immunological study. Our results indicate that the patient's complement system became exhausted during the later phase of his disease, probably due to continual consumption of complement components. Initially, the patient had slightly decreased C4 concentrations and moderately reduced total haemolytic activity (CH50). Later C4 fell to undetectable levels and CH50 declined to zero. The increased haemolytic activity experienced during febrile episodes is probably due to a cold agglutinin with a high thermal amplitude, combined with enhanced synthesis of complement molecules during the acute phase response. Although C4 concentrations never increased to detectable levels during infections or inflammations an acute phase reaction was determined each time, as evidenced by increased concentrations of CRP. By reconstituting the patient's serum with active complement from donor serum or plasma, increased haemolytic activity was observed. These results indicate that some patients with cold agglutinin disease may experience deleterious haemolytic consequences if transfused with plasma-containing blood products. ( info) |
698/718. hiv-associated autoimmune hemolytic anemia complicated by pulmonary embolism following a red blood cell transfusion: case report and review of the literature. BACKGROUND: Though positive direct antiglobulin tests are common in AIDS patients, overt hemolysis is rare. A hypercoagulable state has recently been recognized in these patients and may contribute to the thromboembotic complications previously reported in three patients with hiv-associated autoimmune hemolytic anemia. CASE REPORT: An AIDS patient with severe warm autoimmune hemolytic anemia developed a pulmonary embolus after a single red blood cell transfusion. CONCLUSION: There may be an increased risk of thromboembolism in AIDS patients with autoimmune hemolytic anemia who receive red blood cell transfusions, a concern we have previously raised. Prophylactic anticoagulation should be considered in this setting. ( info) |
699/718. Hemolytic anemia associated with cold agglutinin during chickenpox and a review of the literature. PURPOSE: To describe the findings and cause of anemia and jaundice in a 2-year-old boy. PATIENT AND methods: This boy was admitted with a hemoglobin of 7.8 g/dL, a bilirubin of 1.8 mg/dL, and increased urobilinogen. RESULTS: On the second day after admission, hemolytic anemia associated with an anti-I cold agglutinin was diagnosed, which was transient and recovered without any treatment. CONCLUSION: This is a very rare complication of chickenpox; only five cases have been previously reported and these were all characterized by anti-Pr cold agglutinin with hemolytic anemia after the onset of chickenpox. We report the first case of hemolytic anemia associated with an anti-I cold agglutinin during the incubation period of chickenpox. ( info) |
700/718. Overproduction of antiplatelet antibody against glycoprotein IIb after splenectomy in a patient with Evans syndrome resulting in acquired thrombasthenia. We treated a 33-year-old female with Evans syndrome. She received high dose gamma globulin, prednisolone, and azathioprine, and her platelet count transiently increased. After splenectomy, the platelet count markedly increased. However, the bleeding tendency worsened and the bleeding time was prolonged. A platelet defect, characteristic of thrombasthenia, was found. Antigen-captured ELISA and Western blotting revealed that the patient's serum had an IgG autoantibody against platelet membrane glycoprotein iib and the patient's plasma inhibited normal platelet aggregation. These findings suggest that overproduction of the antiplatelet antibody is triggered by platelet recovery due to splenectomy and affects platelet function resulting in acquired thrombasthenia. ( info) |