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1/44. Solitary hepatic hemangioma in a newborn infant complicated by cardiac failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. Case report and review of the literature.

    A newborn infant with a large hepatic hemangioma developed congestive heart failure, consumption coagulopathy, microangiopathic hemolytic anemia, and obstructive jaundice. The patient was mildly heparinized (250 units per kg and day) and underwent successful resection of the tumor without lobectomy at the age of 3 days. blood volume increased from 93.9 ml/kg at the age of 5 h to 124.2 ml/kg prior to surgery. Red-cell mass simultaneously decreased from 53.8 to 39.4 ml/kg. The increase of blood volume is explained by congestive heart failure, the decrease of red-cell mass by intravascular coagulation within the tumor resulting in formation of thrombi and microangiopathic hemolytic anemia. A review of the literature on infants with symptoms caused by an intrahepatic hemangioma during the first month of life confirms that surgical intervention is the treatment of choice for infants with giant solitary hemangioma of the liver.
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2/44. 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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3/44. Drug-induced hemolysis: cefotetan-dependent hemolytic anemia mimicking an acute intravascular immune transfusion reaction.

    Numerous cases of drug-induced hemolytic anemia have been described in patients treated with penicillin or cephalosporin. Second and third generation cephalosporins are more commonly implicated in hemolytic reactions than first generation cephalosporins. We report a case of severe cefotetan-induced hemolytic anemia in a previously healthy 46-year-old woman undergoing an elective hysterectomy. The patient received 2 g of intravenous cefotetan intraoperatively and subsequently at 12 and 24 h post-operatively. She complained of diarrhea and fever on the third post-operative day and was seen in her gynecologist's office on the fifth post-operative day (hemoglobin = 10.5 g/dL). On the seventh post-operative day, she complained of fever and soreness around the suprapubic catheter site and was given a prescription for 500 mg oral cephalexin four times a day. The next day she was seen in the gynecologist's office and reported feeling better. Ten days after the operation her fatigue worsened and her hemoglobin was 4.8 g/dL. She was transfused with 3 units of packed red blood cells (PRBC) and was given 1 g of cefotetan intravenously. During the transfusion of the second unit of PRBC nursing staff observed gross hemoglobinuria and she subsequently developed acute renal failure. Laboratory chemistry parameters were consistent with severe acute hemolysis. The patient's direct antiglobulin test was reactive and her serum reacted with cefotetan-coated red blood cells (RBCs) and serum plus soluble cefotetan reacted with untreated RBCs. The titration endpoint of the serum against cefotetan-coated RBCs was 40,960, while the serum plus soluble cefotetan against uncoated RBCs was 2,560. This case of severe cefotetan-induced hemolysis was complicated by an acute hemolytic event that occurred during the transfusion of PRBC. Clinical and transfusion service staff must consider drug-induced hemolysis in the differential diagnosis of acute anemia.
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4/44. Neonatal lupus erythematosus with microvascular hemolysis.

    A female, term newborn born to a mother with a history of idiopathic thrombocytopenic purpura and antinuclear antibodies, single-stranded A antibody, and IgM anticardiolipin antibodies presented with immune thrombocytopenia, disseminated intravascular coagulation (DIC), microangiopathic hemolytic anemia, and a characteristic lupus rash in the periorbital areas. She responded to combined treatment with dexamethasone and intravenous immunoglobulin (IVIG). At age 9 months, she was readmitted with severe thrombocytopenia, DIC, and microangiopathic hemolytic anemia. She again responded to IVIG. This suggests that microangiopathic hemolysis can be a presenting symptom in neonatal lupus erythematosus and that reoccurrence of the microangiopathic hemolysis may occur even after the disappearance of lupus antibodies.
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5/44. Cutaneous anthrax associated with microangiopathic hemolytic anemia and coagulopathy in a 7-month-old infant.

    A 7-month-old infant with cutaneous anthrax developed severe systemic illness despite early treatment with antibiotics. The infant displayed severe microangiopathic hemolytic anemia with renal involvement, coagulopathy, and hyponatremia. These findings are unusual with cutaneous anthrax, but have been described in illness resulting from spider toxin and may delay correct diagnosis. The systemic manifestations of the disease persisted for nearly a month despite corticosteroid therapy, but resolved.
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6/44. Diffuse neonatal haemangiomatosis: a rare cause of haemorrhagic shock and refractory coagulopathy in the newborn.

    A term newborn infant developed hypovolaemic shock shortly after birth. She was pale with gross hepatomegaly. She required multiple boluses of intravenous fluids, blood products as well as inotropic support. Blood investigations showed persistent thrombocytopenia, anaemia and disseminated intravascular coagulopathy (DIC). She also developed heart failure. She finally succumbed on the eleventh day of life. autopsy revealed haemangiomatosis involving the liver, lungs, gastrointestinal tract, kidneys and adrenals.
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keywords = anaemia
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7/44. quinine-induced disseminated intravascular coagulation: case report and review of the literature.

    OBJECTIVE: To describe the clinical course of quinine-induced disseminated intravascular coagulation (DIC) and review all previous cases reported in the medical literature. DESIGN: Case report/literature review. SETTING: University teaching hospital medical ICU. patients: One patient in whom thrombocytopenia, coagulopathy, intravascular hemolysis, DIC, and acute renal failure temporally followed the ingestion of quinine. DATA SOURCES: We conducted a computerized free-text medline database search from 1969 to 2000 using the keywords quinine and thrombocytopenia, quinine and hemolytic-uremic syndrome, and quinine and disseminated intravascular coagulation. STUDY SELECTION: All reported cases and reviews of quinine-induced thrombocytopenia, hemolytic-uremic syndrome (HUS), and DIC were reviewed. DIC was distinguished from quinine-induced thrombocytopenia or quinine-induced HUS based on the presence of abnormal clotting times, elevated fibrin degradation products, and/or elevated D-dimer levels. DATA SYNTHESIS: Fifteen previous patients were found to meet the criteria for DIC temporally related to the recent ingestion of quinine. The clinical course and laboratory abnormalities documented for each case are reviewed. CONCLUSIONS: quinine-induced DIC is a distinct clinical entity, which may present as unexplained thrombocytopenia, coagulopathy, or renal failure. In susceptible patients, the immune response to quinine may result in the production of not only anti-platelet antibodies but also antibodies against leukocytes, erythrocytes, and endothelial cells. Furthermore, the varying patterns and specificities of antibody production in an individual patient may result in a spectrum of clinical disease from mild, transient thrombocytopenia to overt intravascular hemolysis, renal failure, coagulopathy, and DIC. Early recognition of quinine-induced DIC is paramount, as this diagnosis affords a better prognosis than other adult forms of HUS or DIC.
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8/44. insulin-dependent diabetes mellitus showing microangiopathic hemolytic anemia and chronic disseminated intravascular coagulation.

    It has been reported that microangiopathic hemolytic anemia occasionally occurs in patients with severe diabetic microangiopathy. We report a case of insulin-dependent diabetes mellitus in whom microangiopathic hemolytic anemia and chronic disseminated intravascular coagulation (DIC) were thought to be complicated. The patient showed fragmentation hemolytic anemia and progressive diabetic microangiopathy, together with a mild elevation of serum fibrin(ogen) degradation products. Considering the state of chronic DIC, heparin therapy was started, but mild hemolysis persisted. It is possible that microangiopathic hemolytic anemia and chronic DIC cause a vicious cycle in patients with severe diabetic microangiopathy, leading to rapid progression of diabetic microangiopathy.
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9/44. Consumption coagulopathy and microangiopathic hemolytic anemia with an axillo-femoral graft.

    Consumption coagulopathy and microangiopathic hemolytic anemia occurred as a complication of insertion of an axillofemoral, preclotted dacron graft. Treatment with heparin followed by dipyridamole and aspirin normalized the hematologic and coagulation abnormalities over a two month period. The mechanism of consumption coagulopathy associated with prosthetic grafts is discussed and a possible role for treatment with antiplatelet agents is suggested.
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keywords = hemolytic
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10/44. hiv infection presenting as severe autoimmune hemolytic anemia with disseminated intravascular coagulation in an infant.

    Autoimmune hemolytic anemia is rare in children. It is generally diagnosed in relation to a viral or bacterial infection but has also been described in association with drugs, autoimmune disease, malignancy, and immunodeficiency. The authors describe a 5-month-old infant who presented with severe autoimmune hemolytic anemia and disseminated intravascular coagulation as his initial manifestation of hiv infection.
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