ANEMIA HEMOLITICA AUTOINMUNE PEDIATRIA PDF

Niño de 3 meses con anemia hemolítica no autoinmune. Article in Anales de Pediatría 55(3)– · December with 6 Reads. Article in Anales de Pediatría 71(3) · September with 56 Reads Anemia hemolítica autoinmune con prueba de antiglobulina positiva a. de Medicina Interna de una institución pediátrica de alto nivel de complejidad. El paciente consultó por un cuadro de anemia hemolítica autoinmune.

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Autoimmune hemolytic anemia AIHA is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. It can be idiopathic or secondary, and classified as warm, cold cold hemagglutinin disease CAD and paroxysmal cold hemoglobinuria or mixed, according to the thermal range of the autoantibody.

AIHA may develop gradually, or have a fulminant onset with life-threatening anemia. The treatment of AIHA is still not evidence-based. Additional therapies are intravenous immunoglobulins, danazol, plasma-exchange, and alemtuzumab and high-dose cyclophosphamide as last resort option. As the experience with rituximab evolves, it is likely that this drug will be located at an earlier point in therapy of warm AIHA, before more toxic immunosuppressants, and in place of splenectomy in some cases.

In CAD, rituximab is now recommended as first-line treatment. Autoimmune hemolytic anemia AIHA is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells, with an estimated incidence in adults of 0. The diagnosis is usually simple, based on the presence of hemolytic anemia and serological evidence of anti-erythrocyte antibodies, detectable by the direct antiglobulin test DAT. For the former two conditions, the use of mono-specific antisera against IgA and low ionic strength solutions or cold washings can overcome the DAT negativity.

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Small amounts of RBC-bound IgG can be detected employing techniques that are more sensitive than the traditional DAT-tube, such autoinjune microcolumn, solid-phase, enzyme-linked, and flow cytometry. These atypical cases, which are identified with increasing frequency, may represent a critical diagnostic problem and cause delays in therapy.

AIHA may develop gradually, with concomitant physiological compensation, or may have a fulminant onset with profound, life-threatening anemia. The traditional treatment of AIHA includes corticosteroids, splenectomy and conventional immunosuppressive drugs.

Over recent years, some new therapies have become available and there has been some evidence of success.

These therapies are primarily used in patients who are not candidates for or fail to respond to splenectomy, those who relapse after splenectomy, and those who cannot maintain stable hemoglobin levels without unacceptably high doses of corticosteroids. There is general agreement that corticosteroids represent the first-line treatment for patients with warm antibody type AIHA, albeit their use is based on experience rather than hard evidence.

In fact, there is little published information on their effectiveness, 11617 and this is not supported by clinical trials. Corticosteroids, usually prednisone, are given at the initial dose of 1. Response occurs mainly during the second week, and if none or minimal improvement is observed in the third week, this therapy is assumed to be ineffective. After stabilization of hemoglobin, prednisone should be gradually and slowly tapered off at 10—15 mg weekly to a daily dose of 20—30 mg, then by 5 mg every 1—2 weeks until a dose of 15 mg, and subsequently by 2.

Once the decision for a second-line treatment has been taken, there are several options, although splenectomy and rituximab are the only second-line treatments with a proven short-term efficacy. Splenectomy is commonly thought to be the most effective conventional second-line treatment of warm AIHA to be proposed to patients unresponsive or intolerant to corticosteroids, in those that require a daily maintenance dose of prednisone greater than 10 mg, and in those with multiple relapses.

The role and efficacy of antibiotic prophylaxis in this setting remains unclear, and not all investigators recommend this approach. Rituximab, a monoclonal antibody directed against the CD20 antigen expressed on B cells, has been shown to be effective in AIHA, although the comparison of response rates in various studies is difficult in the absence of common response criteria.

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Complete remission and good partial responses have been reported in all treated adult patients 9 idiopathic and 2 secondary to systemic lupus erythematosus. It has been suggested that this drug could be included in the treatment arsenal of refractory immune cytopenias, as a steroid-sparing option.

Intravenous immunoglobulins IVIG are frequently used in AIHA, alone or in combination with prednisone, 60 and mostly in children, probably because of their proven effectiveness in primary immune thrombocytopenia, and the relatively low incidence of adverse effects compared with other treatment options. However, their use is controversial, primarily because only small case series have been reported.

In a recent guideline, high-dose immunoglobulin was not recommended for use in AIHA, except under certain life-threatening circumstances. Plasma exchange has been performed in a relatively small number of severely affected warm AIHA patients, both children and adults, in whom the anemia could not be stabilized with steroids and transfusion therapy alone, as a temporizing measure.

Moreover, concomitant therapy with steroids and immunosuppressive drugs often made it difficult to define the contribution of this procedure to the outcome. A retrospective single center case-control study failed to demonstrate that plasma exchange increases red blood cell transfusion efficiency in severe autoimmune hemolytic anemia.

Alemtuzumab, a humanized anti-CD52 monoclonal antibody, has been shown to be effective in small series of patients with idiopathic refractory AIHA, with an overall complete remission rate in 13 of 16, including 3 pediatric cases. Patients with AIHA may often require red blood cell RBC transfusion to maintain clinically acceptable hemoglobin values, at least until specific treatments become effective.

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In less urgent cases, an extended phenotyping is advisable and compatible red cell units may be selected for transfusion. Some authors recommend ignoring the specificity of the autoantibody because it is not extensively proven that antigen-negative RBC transfusions result in an increased erythrocyte survival. As regards the volume to be transfused, it is worth remembering that overtransfusion should be avoided both for hemodynamic reasons particularly in elderly patientsand for the occurrence of hemoglobinemia and hemoglobinuria, which might not be due to alloantibody-induced hemolysis, as generally thought, but rather to the increase of the total mass of RBCs available for destruction.

It is worth mentioning that C1-esterase inhibitor might have potential as a safe therapy to control complement-induced RBC destruction in AIHA patients. In fact, non-severe asymptomatic forms of CAD may require only protection against exposure to cold temperatures and occasional transfusion support in winter.

In particular, the patient and the extremity chosen for infusion should be kept warm, and the use of an in-line blood warmer is recommended.

Moreover, infusion of cold liquids and blood products with a high plasma content should be avoided. Finally, erythropoietin, widely used in the USA but not so often in Western and Northern Europe, has no evidence-based proof of efficacy. Rituximab is now recommended as the first-line treatment of CAD, 84 although complete and sustained remissions are uncommon.

As regards new experimental approaches, improvement of anemia has been observed in 2 patients following monotherapy with bortezomib, an inhibitor of 26S proteasome, 95 and in 2 cases after administration of eculizumab, the monoclonal anti-C5 antibody licensed for paroxysmal nocturnal hemoglobinuria. There is no evidence-based therapy for CAD secondary to malignant or infectious diseases.

Generally, treatment of the underlying disease is accompanied by resolution of the hemolysis, particularly in lymphoproliferative diseases and Mycoplasma pneumonia.

Most antibodies are IgG and directed against the P blood group system. In the past, PCH was mainly associated with syphilis, and now usually follows viral and bacterial infections, including Mycoplasma pneumonia.

Some authors suggest that patients with mixed AIHA have a more severe onset and more chronic course than patients with other categories of AIHA, although a comprehensive comparison of the clinical course in the different forms has not been made. These cases frequently showed reticulocytopenia, which may contribute to the clinical picture.

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Moreover, cases with severe onset were more often refractory to first-line therapy and required 3 or more lines of therapy. The therapeutic arsenal now available for steroid-refractory warm AIHA is certainly broader than in the past. However, no controlled clinical trials have yet been performed that can guide the choice of treatment. As experience with rituximab evolves, it is peviatria that this drug will be used earlier in therapy, before more toxic immunosuppressants, and in some cases in place of splenectomy.

As regards CAD, rituximab is now recommended as first-line treatment. Treatment algorithm for warm AIHA in adults. This review article was originally published in the education book of the 19th congress of EHA June National Center for Biotechnology InformationU.

Journal List Haematologica v. Alberto Zanella and Wilma Barcellini. Author information Article notes Anemoa and License information Disclaimer. Received Jul 23; Accepted Aug This article has been pediahria by other articles in PMC. Abstract Autoimmune hemolytic anemia AIHA is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells. Introduction Autoimmune hemolytic anemia AIHA is a relatively uncommon disorder caused by autoantibodies directed against self red blood cells, with an estimated incidence in adults of 0.

Autoinmuhe therapy Corticosteroids There is general agreement that corticosteroids represent the first-line treatment for patients with warm antibody type AIHA, albeit their use is based on experience rather than hard evidence.

Second-line therapy Once the decision for a second-line treatment has been taken, there are several options, although splenectomy and rituximab are the only second-line treatments with a proven short-term efficacy.

Splenectomy Splenectomy is commonly thought to be the most effective conventional second-line treatment of warm AIHA to be proposed to patients unresponsive or intolerant to corticosteroids, in those that require a daily maintenance dose of prednisone greater than 10 mg, and in those with multiple relapses. Supportive therapy Patients with AIHA may often require red blood cell RBC transfusion to maintain clinically acceptable hemoglobin values, at least until specific treatments become effective.

Conclusions The therapeutic arsenal now available for steroid-refractory warm AIHA is certainly broader than in the past. Open in a separate window. Footnotes This review article was originally published in the education book of the hemoliticca congress of EHA June Petz LD, Garratty G. Lechner K, Jager U. How I treat autoimmune hemolytic anemias in adults. Valent P, Lechner K.

Diagnosis and treatment of autoimmune haemolytic anaemias in adults: Blood Banking and Transfusion Medicine. Basic Principles and Practice. New insights into childhood autoimmune hemolytic anemia: Serologic findings in autoimmune hemolytic anemia associated with immunoglobulin M warm autoantibodies.

Characterization of direct antiglobulin test-negative autoimmune hemolytic anemia: Variability of the erythrocyte response in autoimmune hemolytic anemias: Autoimmune hemolytic anemia with reticulocytopenia. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia.

Rituximab for primary chronic cold agglutinin disease: Rituximab in chronic cold agglutinin disease: Low-dose rituximab and alemtuzumab combination therapy for patients with steroid-refractory autoimmune cytopenias. Low-dose hemokitica in adult patients with idiopathic autoimmune hemolytic anemia: Sustained response to low-dose rituximab in idiopathic autoimmune hemolytic anemia. Autoimmune hemolytic anemia in children. Incidence and risk factors of relapses in idiopathic autoimmune hemolytic anemia.

J Med Assoc Thai. S—S [ PubMed ]. Pulsed high-dose dexamethasone in chronic autoimmune haemolytic anaemia of warm type. Megadose methylprednisolone for the treatment of patients with Evans syndrome. Comparative response to splenectomy in Coombs-positive auto-immune hemolytic anemia with or without associated disease.

Warm autoimmune hemolytic anemia: