Alpha-Interferon for Severe Cold Agglutinin Disease

O’Connor, Brian; Clifford, Jacqueline S.; Lawrence, W. Dwayne; Logue, Gerald L.

doi:10.7326/0003-4819-111-3-255

Cited by 38 publications

(18 citation statements)

References 8 publications

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“…In case of an underlying lymphoma, specific therapy of the lymphoma (cytoreduction, chemotherapy) may improve AIHA, although not all patients may respond [86]. In a few reports [155,156], patients with CAD were found to responded to interferonalpha. A drug with well documented efficacy in CA-AIHA is rituximab.…”

Section: Treatment Of Cold Antibody Aihamentioning

confidence: 99%

Diagnosis and treatment of autoimmune haemolytic anaemias in adults: a clinical review

Valent

Lechner

2008

Wien Klin Wochenschr

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Autoimmune haemolytic anaemia (AIHA) is an immune disorder caused by antibodies directed against unmodified autologous red cells. The disorder may be a primary (idiopathic) or a secondary disease. The diagnosis is based on the presence of anaemia, signs of haemolysis with reticulocytosis, low haptoglobin, increased lactate dehydrogenase, elevated indirect bilirubin, and a positive direct antiglobulin test (Coombs test). Sometimes, not all of these typical features are present. Most AIHA are caused by warm antibodies, whereas cold antibodies are less commonly detected. While half of the warm antibody-based AIHA are idiopathic anaemias, almost all cold antibody AIHA are secondary anaemias. Underlying diseases are Non Hodgkin's lymphomas and systemic autoimmune disorders, and less frequently organ transplantation, infections, or solid tumors. Moreover, AIHA is an important complication of treatment with nucleoside analogs. Most patients with AIHA require therapy. In warm antibody AIHA, standard first line therapy are glucocorticosteroids with or without high dose immunoglobulins, whereas splenectomy is considered second-line therapy. Response rates of primary AIHA to corticosteroid therapy are high. After initial remission, the dose should be tapered down slowly and with caution, and in some cases, low-dose maintenance therapy is required. The efficacy of standard therapy is low in secondary AIHA that develops in lymphoma patients, posttransplant patients, or tumor patients. Among other immunosuppressive treatments, rituximab (anti-CD20) appears to be highly effective in patients with warm antibody AIHA refractory to standard therapy. Mycophenolate mofetil is quite effective in AIHA patients with an underlying autoimmune or lymphoproliferative disease. Patients with cold agglutinins are refractory to steroids and splenectomy. Half of these patients may respond to rituximab, although responses usually are short-lived. Sometimes, AIHA that is associated with malignant lymphomas or tumors, disappears after successful anti-lymphoma or anti-tumor therapy.

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Section: Treatment Of Cold Antibody Aihamentioning

confidence: 99%

Diagnosis and treatment of autoimmune haemolytic anaemias in adults: a clinical review

Valent

Lechner

2008

Wien Klin Wochenschr

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“…[42] Significant responses have also been seen with alpha-interferon. [43] Steroids are found effective only in those patients having either low titer or high thermal amplitude IgM cold agglutinins or IgG cold agglutinins. Generally extravascular hemolysis in CAS occurs in the liver, so splenectomy has only benefited those patients with IgG cold agglutinins.…”

Section: Treatment Of Aihamentioning

confidence: 99%

Autoimmune hemolytic anemia: From lab to bedside

Chaudhary¹,

Das²

2014

Asian J Transfus Sci

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Autoimmune hemolytic anemia (AIHA) is not an uncommon clinical disorder and requires advanced, efficient immunohematological and transfusion support. Many AIHA patients have underlying disorder and therefore, it is incumbent upon the clinician to investigate these patients in detail, as the underlying condition can be of a serious nature such as lymphoproliferative disorder or connective tissue disorder. Despite advances in transfusion medicine, simple immunohematological test such as direct antiglobulin test (DAT) still remains the diagnostic hallmark of AIHA. The sensitive gel technology has enabled the immunohematologist not only to diagnose serologically such patients, but also to characterize red cell bound autoantibodies with regard to their class, subclass and titer in a rapid and simplified way. Detailed characterization of autoantibodies is important, as there is a relationship between in vivo hemolysis and strength of DAT; red cell bound multiple immunoglobulins, immunoglobulin G subclass and titer. Transfusing AIHA patient is a challenge to the immunohematologist as it is encountered with difficulties in ABO grouping and cross matching requiring specialized serological tests such as alloadsorption or autoadsorption. At times, it may be almost impossible to find a fully matched unit to transfuse these patients. However, transfusion should not be withheld in a critically ill patient even in the absence of compatible blood. The “best match” or “least incompatible units” can be transfused to such patients under close supervision without any serious side-effects. All blood banks should have the facilities to perform the necessary investigations required to issue “best match” packed red blood cells in AIHA. Specialized techniques such as elution and adsorption, which at times are helpful in enhancing blood safety in AIHA should be established in all transfusion services.

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“…disease [6]. Considering the exceedingly poor prognosis of patients with this disorder and their lack of response to usual interventions applied, we recommend that this type of therapy be considered in the management of low-titer idiopathic cold agglutinin disease with hemolytic anemia.…”

Section: Discussionmentioning

confidence: 99%