Erythroid hyperplasia of the bone marrow Specific investigations Treatment[ edit ] Plentiful literature exists regarding the treatment of AIHA. Efficacy of treatment depends on the correct diagnosis of either warm- or cold-type AIHA. Warm-type AIHA is usually a more insidious disease, not treatable by simply removing the underlying cause. Corticosteroids are first-line therapy.

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La Habana, Cuba. ABSTRACT The treatment of autoimmune hemolytic anemias AIHA is based on the individual clinical evaluation, immunochemical characteristics of the involved antibodies and the absence or presence of an underlying condition. The treatment of AIHAs varies according to its immunohematological classification. After the initial remission, the doses should be reduced slowly, until the required maintenance level is reached; steroid-dependent or steroid resistant patients often require splenectomy or treatment with Rituximab anti CD20 monoclonal antibody and with less frequency, immunosuppressive drugs as azathioprine or cyclophosphamide.

Patients with cold AIHA agglutinins cAHAI are resistant to steroids and splenectomy; patients with asymptomatic or stable anemia do not require treatment. Rituximab is the most effective treatment for symptomatic cases, although immunosuppressants such as cyclophosphamide and chlorambucil could be used.

Paroxysmal cold hemoglobinuria do not usually require treatment. Drug induced AIHAs usually respond to discontinuing the offending drug and steroids treatment. Transfusion should be avoided in AIHAs, except in life threatening situations; if it is required, small volumes of erytrhocyte concentrates should be administered, slowly, in cAIHA; the blood should be prewarmed at C. Los pilares de la terapia inicial de la AHAI por anticuerpos calientes son el tratamiento de la enfermedad subyacente, si existe, y el uso de esteroides.

Los pacientes refractarios al tratamiento inicial con esteroides deben evaluarse para determinar la existencia de una enfermedad subyacente. Una vez obtenida la respuesta debe reducirse la dosis progresivamente. Las dosis mayores no han mostrado una mayor eficacia. Los dos restantes se mantienen estables con tratamiento con inmunosupresores ciclofosfamida y clorambucil.

El tratamiento de este tipo de AHAI es similar al de la producida por anticuerpos calientes. Valent P, Lechner K. Diagnosis and treatment of autoimmune haemolytic anaemias in adults: a clinical review.

Wien Klin Wochenschr. Petz LD. Diagnostic complexities in autoimmune hemolytic anemias. Cold antibody autoimmune hemolytic anemias. Blood Rev. Pruebas de compatibilidad pretransfusional. How I treat autoimmune hemolytic anemias in adults. Medicina Transfusional. Autoimmune hemolytic anemia in children. Pediatr Hematol Oncol.

Autoimmune hemolytic anemia. Indian J Pediatr. Rev Cubana Hematol Inmunol Hemoter. Michel M. Classification and therapeutic approaches in autoimmune hemolytic anemia: an update.

Expert Rev Hematol. Evidence-based focused review of the treatment of idiopathic warm immune hemolytic anemia in adults Blood. Treatment of autoimmune hemolytic anemias. Curr Opin Hematol. High-dose cyclophosphamide for refractory autoimmune hemolytic anemia. Blood Jul 15; Efficacy of mycophenolate mofetil in adult refractory auto-immune cytopenias: a single center preliminary study.

Eur J Haematol. Effectiveness of cyclosporine and mycophenolate mofetil in a child with refractory evans syndrome. Pediatr Rep. Zwerner J, Fiorentino D. Mycophenolate mofetil. Dermatol Ther. Plasma exchange and rituximab treatment for lenalidomide-associated cold agglutinin disease.

Demonstration of two distinct antibodies in autoimmune hemolytic anemia with reticulocytopenia and red cell aplasia. Exp Hematol. Transfus Med Hemother. Kuzmanovic M, Jurisic V. Rituximab for treatment of autoimmune hemolytic anemia. Indian Pediatr. Allogeneic stem cell transplantation for Evans syndrome.

Bone Marrow Transplant. Rituximab for Refractory Evans Syndrome and other immune-mediated hematologic diseases. Am J Hematol. Treatment of childhood autoimmune haemolytic anaemia with rituximab.

Fozza C, Longinoti M. Use of Rituximab in autoimmune hemolytic anemia associated with non-hodgkin lymphomas Adv Hematol. Rituximab for immune cytopenia in adults: idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia, and Evans syndrome. Mayo Clin Proc. Rituximab therapy for chronic lymphocytic leukemia-associated autoimmune hemolytic anemia. Am J Hematol Aug;81 8 Berentsen S. How I manage cold agglutinin disease. Br J Haematol.

Mease, PJ. B-cell-targeted therapy in autoimmune disease: rationale, mechanisms, and clinical application. J Rheumatol. Monoclonal antibodies: new therapeutic agents for autoimmune hemolytic anemia? Rituximab in auto-immune haemolytic anaemia and immune thrombocytopenic purpura: a Belgian retrospective multicentric study. J Intern Med. Pure red cell aplasia due to parvovirus following treatment with CHOP and rituximab for B-cell lymphoma.

Fatal visceral varicella-zoster infection following rituximab and chemotherapy treatment in a patient with follicular lymphoma.

Rituximab treatment results in impaired secondary humoral immune responsiveness. Severe hematological side effects following Rituximab therapy in children. Alemtuzumab to treat refractory autoimmune hemolytic anemia or thrombocytopenia in chronic lymphocytic leukemia Curr Hematol Malig Rep. Low-dose alemtuzumab-associated immune thrombocytopenia in chronic lymphocytic leukemia. The effect of treatment with Campath-1H in patients with autoimmune cytopenias.

Dacie, J. Treatment and prognosis of cold antibody AIHA. En: Dacie J ed. The Haemolytic Anaemias. London:Churchill Livingstone; Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients. Blood Apr 15; 8 Occurrence of lymphoplasmacytic lymphoma 6 years after amelioration of primary cold agglutinin disease by rituximab therapy.

Int J Hematol. Long-term efficacy of the complement inhibitor eculizumab in cold agglutinin disease. A last resort modality using cryofiltration apheresis for the treatment of cold hemagglutinin disease in a Veterans Administration hospital. Ther Apher Dial. Garratty G. Immune hemolytic anemia caused by drugs. Expert Opin Drug Saf.


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