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The anemia is of variable severity but may be severe, with hematocrit of less than 10% Reticulocytosis is usually present, and spherocytes are seen on the peripheral blood smear In cases of severe hemolysis, the stressed bone marrow may also release nucleated red blood cells As with other hemolytic disorders, indirect bilirubin is increased Approximately 10% of patients with autoimmune hemolytic anemia have coincident immune thrombocytopenia (Evans s syndrome) The direct Coombs test is positive, and the indirect Coombs test may or may not be positive A positive indirect Coombs test indicates the presence of a large amount of autoantibody that has saturated binding sites in the red blood cell and consequently appears in the serum Because the patient s serum usually contains the autoantibody, it may be difficult to obtain a compatible crossmatch with donor s cells



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Cold agglutinin disease is an acquired hemolytic anemia due to an IgM autoantibody usually directed against the I antigen on red blood cells These IgM autoantibodies characteristically will not react with cells at 37 C but only at lower temperatures Since the blood temperature (even in the most peripheral parts of the body) rarely goes lower than 20 C, only antibodies active at higher temperatures will produce clinical effects Hemolysis results indirectly from attachment of IgM, which in the cooler parts of the circulation (fingers, nose, ears) binds and fixes complement When the red blood cell returns to a warmer temperature, the IgM antibody dissociates, leaving complement on the cell Lysis of cells rarely occurs Rather, C3b present on the red cells is recognized by Kupffer cells (which have receptors for C3b), and red blood cell sequestration ensues Most cases of chronic cold agglutinin disease are idiopathic Others occur in association with Waldenstr m s macroglobulinemia, in which a monoclonal IgM paraprotein is produced Acute postinfectious cold agglutinin disease occurs following mycoplasmal pneumonia or infectious mononucleosis (with antibody directed against antigen i rather than I)

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Initial treatment consists of prednisone, 1 2 mg/kg/d in divided doses Most transfused blood will survive similarly to the patient s own red blood cells Because of difficulty in performing the cross-match, incompatible blood may be given Decisions regarding transfusions should be made in consultation with a hematologist If prednisone is ineffective or if the disease recurs on tapering the dose, splenectomy should be performed Patients with autoimmune hemolytic anemia refractory to prednisone and splenectomy may be treated with a variety of agents Treatment with rituximab, a monoclonal antibody against the B cell antigen CD20, is effective in some cases The suggested dose is 375 mg/m2 intravenously weekly for 4 weeks Danazol, 600 800 mg/d, is less often effective than in immune thrombocytopenia but is well suited for long-term use because of its low toxicity Immunosuppressive agents, including cyclophosphamide, azathioprine, mycophenolate mofetil, or cyclosporine, may also be used High-dose intravenous immune globulin (1 g daily for 1 or 2 days) may be highly effective in controlling hemolysis The benefit is short-lived (1 3 weeks), and the drug is very expensive The long-term prognosis for patients with this disorder is good, especially if there is no underlying autoimmune disorder or lymphoma Splenectomy is often successful in controlling the disorder

Petz LD A physician s guide to transfusion in autoimmune haemolytic anaemia Br J Haematol 2004 Mar;124(6):712 6 [PMID: 15009058] Robak T Monoclonal antibodies in the treatment of autoimmune cytopenias Eur J Haematol 2004 Feb;72(2):79 88 [PMID: 14962245]

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In chronic cold agglutinin disease, symptoms related to red blood cell agglutination occur on exposure to cold, and patients may complain of mottled or numb fingers or toes Hemolytic anemia is rarely severe, but episodic hemoglobinuria may occur on exposure to cold The hemolytic anemia in acute postinfectious syndromes is rarely severe

Mild anemia is present with reticulocytosis and spherocytes The direct Coombs test will be positive for complement only Occasionally, a micro-Coombs test is necessary to reveal bound complement (low-titer cold agglutinin disease) A bedside cold agglutinin test may be performed by placing a glass slide in ice and then putting a few drops of heparinized blood on it Inspection may reveal small clumps of agglutinated blood

Treatment is largely symptomatic, based on avoiding exposure to cold Splenectomy and prednisone are usually

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Print .js - Javascript library for HTML elements, PDF and image files ...
Print .js is a tiny javascript library to help printing from the web. Print friendly support for HTML elements, image files and JSON data. Print PDF files directly form ...

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Print .js - Javascript library for HTML elements, PDF and image files ...
Print .js is a tiny javascript library to help printing from the web. Print friendly support for HTML elements, image files and JSON data. Print PDF files directly form ...












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