AbstractAutoimmune Hemolytic Anemia Research Articles

Rituximab in Steroid Refractory Autoimmune Hemolytic Anemia

Autoimmune hemolytic anemia is rare in children and infants and steroids are the corner stone of therapy. Management of the patients with steroid refractory/dependent disease is difficult .Rituximab is being used in the treatment of a variety of autoimmune diseases including Autoimmune hemolytic anemia (AIHA),especially in adults but there is scarce data regarding the use of this agent in pediatric AIHA patients.The authors report two cases of steroid refractory AIHA, who responded to rituximab with review the literature of its use in pediatrics.

Giant cell hepatitis and autoimmune hemolytic anemia after chickenpox

Autoimmune hemolytic anemia with giant cell hepatitis is a distinct entity in children. It is usually fatal with progressive liver disease. Immunosuppressive treatment with conventional drugs offers some response; however, it is usually only temporary. Alternative therapeutic options with monoclonals have been reported with promising remission of the disease. We report a case with autoimmune hemolytic anemia+giant cell hepatitis after varicella infection. She was resistant to standard immunosuppressive combinations, and rescue therapy with rituximab was used. Remission was not achieved with the drug and the child died with septic complication.

Autoimmune Hemolytic Anemia Associated With Ulcerative Colitis: The Most Important Step Is to Induce Complete Remission

Autoimmune Hemolytic Anemia Associated With Ulcerative Colitis: The Most Important Step Is to Induce Complete Remission

Immune hemolytic anemia—selected topics

Autoimmune hemolytic anemia (AIHA) is most often idiopathic. However, in recent years, AIHA has been noted with increased incidence in patients receiving purine nucleoside analogues for hematologic malignancies; it has also been described as a complication of blood transfusion in patients who have also had alloimmunization. As the technology of hematopoietic stem cell transplantation has become more widespread, immune hemolysis in the recipients of ABO-mismatched products has become better recognized. The syndrome is caused by passenger lymphocytes transferred from the donor and, although transient, can be quite severe. A similar syndrome has been observed in recipients of solid organ transplants when there is ABO-incompatibility between donor and recipient. Venous thromboembolism is a little-recognized, though likely common, complication of AIHA, and may in some instances be related to coexistent antiphospholipid antibodies. While AIHA is a well-documented complication of malignant lymphoproliferative disorders, lymphoproliferative disorders may also paradoxically appear as a consequence of AIHA. A number of newer options are available for treatment of AIHA in patients refractory to corticosteroids and splenectomy. Newer immunosuppressives such as mycophenolate mofetil may have a role in such cases. Considerable experience has been accumulating in the last few years with monoclonal antibody therapy, mainly rituximab, in difficult AIHA cases; it appears to be a safe and effective option.

Autoimmune hemolytic anemia: classification and therapeutic approaches

Autoimmune hemolytic anemia (AIHA) is a relatively uncommon cause of anemia. Classifications of AIHA include warm AIHA, cold AIHA (including mainly chronic cold agglutinin disease and paroxysmal cold hemoglobinuria), mixed-type AIHA and drug-induced AIHA. AIHA may also be further subdivided on the basis of etiology. Management of AIHA is based mainly on empirical data and on small, retrospective, uncontrolled studies. The therapeutic options for treating AIHA are increasing with monoclonal antibodies and, potentially, complement inhibitory drugs. Based on data available in the literature and our experience, we propose algorithms for the treatment of warm AIHA and cold agglutinin disease in adults. Therapeutic trials are needed in order to better stratify treatment, taking into account the promising efficacy of rituximab.

Prompt and sustained response of a steroid-refractory autoimmune hemolytic anemia to a rituximab-based therapy in a chronic lymphocytic leukemia patient

Autoimmune hemolytic anemia (AIHA) is a rare and potentially life-threatening event which may complicate the course of chronic lymphocytic leukemia (CLL) at any time and steroid-refractory AIHA of CLL poses a therapeutic challenge for physicians. Here, we report the safety and efficacy of a rituximab-containing regimen in a CLL patient with steroid- and IVIg-refractory AIHA. A 57-year- old man affected by CLL, presented with fatigue, dyspnoea, tachycardia and jaundice. His physical examination revealed overt jaundice, hepato- and splenomegaly, and enlargement of lymph nodes in all superficial sites. The blood chemistry showed severe anemia (Hb value 3.9 g/dL), high white blood cell count (89 x 10(9)/L), altered hemolysis markers and direct antiglobulin test (DAT) was positive for both complement and IgG. The patient failed to respond to both a 4-day course of high-dose dexamethasone IV (40 mg/day) and intravenous immunoglobulin (IVIg) (1 g/kg/day x 2 days). Thus, a schedule containing rituximab (375 mg/m(2) day +1), cyclophosphamide (750 mg/m(2) day +2) and prednisone (60 mg/m(2) from day +1 to day +7) (R-CP) were administered. Four cycles, repeated every 4 weeks, were administered. After 4 days from the infusion of this schedule, the patient showed a marked reduction of the lymphocytosis, and the hemoglobin level started to increase. No rituximab-related side effects were recorded. At the end of treatment DAT became negative and patient achieved a nodular Partial Remission (nPR). Our data showed the safety and efficacy of a rituximab-containing regimen in a life-threatening CLL-related AIHA, refractory to steroid and IVIg therapy. This schedule has allowed the patient to obtain a prompt and dramatic rise in hemoglobin level and a response to both AIHA and CLL.

A Case of Autoimmune Hemolytic Anemia Treated with Rituximab in a Child

Autoimmune hemolytic anemia (AIHA) in children usually responds well to short-term steroid therapy. However, in some cases, AIHA requires prolonged immunosuppressive therapy, with the subsequent development of severe side effects. Compared with previous conventional immunosuppressive therapy, rituximab, an anti-CD20 chimeric monoclonal antibody, shows good therapeutic efficacy and safety in the treatment of autoimmune disorders. Herein, the case of a 13-year-old male patient, who showed a remarkable and durable response to rituximab, at a dose of 375mg/m2 , is reported. Before this trial, he had been a hepatitis B carrier and steroid dependent AIHA for 4 years, with a cushingoid facial appearance and growth retardation. After 10 months, he experienced a recurrence of hemolysis, which was successfully retreated, and was then taken off the steroid ther apy. The therapy was well tolerated, without serious complications. It is suggested that rituximab could be a new option in the treatment of steroid-dependent AIHA. Therefore, long-term follow-up and studies of the risk factors of a relapse are

Immune Hemolytic Anemia—Selected Topics

Autoimmune hemolytic anemia (AIHA) is most often idiopathic. However, in recent years, AIHA has been noted with increased incidence in patients receiving purine nucleoside analogues for hematologic malignancies; it has also been described as a complication of blood transfusion in patients who have also had alloimmunization. As the technology of hematopoietic stem cell transplantation has become more widespread, immune hemolysis in the recipients of ABO-mismatched products has become better recognized. The syndrome is caused by passenger lymphocytes transferred from the donor, and although transient, can be quite severe. A similar syndrome has been observed in recipients of solid organ transplants when there is ABO-incompatibility between donor and recipient. Venous thromboembolism is a little-recognized, though likely common, complication of autoimmune hemolytic anemia (AIHA), and may in some instances be related to coexistent antiphospholipid antibodies. While AIHA is a well-documented complication of malignant lymphoproliferative disorders, lymphoproliferative disorders may also paradoxically appear as a consequence of AIHA. A number of newer options are available for treatment of AIHA in patients refractory to corticosteroids and splenectomy. Newer immunosuppressives such as mycophenolate may have a role in such cases. Considerable experience has been accumulating in the last few years with monoclonal antibody therapy, specifically rituximab, in difficult AIHA cases; it appears to be a safe and effective option.

Development of Cold Agglutinin Autoimmune Hemolytic Anemia During Treatment for Pediatric Acute Lymphoblastic Leukemia

Autoimmune hemolytic anemia (AIHA) is a potentially fatal complication of many lymphoid malignancies. Those most often associated with AIHA include chronic lymphocytic leukemia, B-cell lymphomas, and Burkitt-type acute lymphoblastic leukemia (ALL) and are clonal populations of mature B cells. There have been no reports of patients with B-cell precursor ALL who developed AIHA while undergoing chemotherapy, but AIHA has been reported in a few patients with ALL after hematopoietic stem cell transplant. The authors describe a child with B-cell precursor ALL who developed cold agglutinin AIHA during maintenance treatment while in remission after infection with influenza B.

Autoimmune hemolytic anemia (AIHA) in an infant rhesus macaque (Macaca mulatta)

Autoimmune hemolytic anemia (AIHA) is a disorder associated with the destruction of red blood cells (RBCs) by autoantibodies. We report a rare case of AIHA in an infant rhesus macaque (Macaca mulatta) which received a continuous administration of four drugs, a dopamine agonist. dopamine receptor inhibitor, and two gamma-aminobutyric acid receptor inhibitors into the brain during the course of neurophysiological experiments. The main clinical findings were severe anemia and splenomegaly. Hematological and serological examinations revealed the appearance of peripheral erythroblasts and autoantibodies against RBCs. Medical treatments, including washed RBC transfusion and corticosteroids, transiently improved the animal's anemia, but euthanasia was decided on 331 days after the start of the experiment. The pathological findings revealed severe anemia, splenomegaly, and extramedullary hematopoiesis in the liver and kidneys. These findings and the clinical course suggest that this anemia was a warm-antibody type of AIHA induced by the administration of the drugs for the neurophysiological experiment.

Leukocyte reduction of red cells when transfusing patients with autoimmune hemolytic anemia: a strategy to decrease the incidence of confounding transfusion reactions

Abstract Autoimmune hemolytic anemia (AIHA) presents a difficult challenge to clinicians and blood bankers alike. Autoantibodies in the serum significantly complicate serologic evaluation, and necessitate performing procedures such as adsorptions to eliminate the possibility of underlying alloantibodies. In many instances the blood that is issued may be phenotypically similar but remains crossmatch incompatible, generating a considerable degree of anxiety among the clinical staff who are responsible for transfusing the patient. We report a case of warm autoimmune hemolytic anemia (WAIHA) in which the transfusion of red cells was complicated by a febrile transfusion reaction. Evaluation of the reaction resulted in a significant delay in transfusion therapy. Subsequent administration of leukocyte-poor red cells resulted in uneventful transfusions with a good therapeutic response. Retrospective analysis of the pretransfusion sample demonstrated significant levels of anti-neutrophil antibodies. This case resulted in the establishment of our policy to administer all red cell transfusions to patients with autoantibodies (warm or cold) as leukocyte-poor red cells. Immunohematology 1996;12:84–86.

Autoimmune hemolytic anemia due to IgA class autoantibodies

Abstract Autoimmune hemolytic anemia clue to warm-reacting autoantibod-ies solely of the IgA class is very rare, and only live cases were identified among 5,177 patients referred during 13.5 years. All were females Cages 21-69 years) and all presented with idiopathic “Coombs negative1' autoimmune hemolytic anemia, a. diagnosis that was confirmed using monospecific anti-human IgA reagents. Red cell-bound IgG was reduced, but in two patients IgM was initially increased, an occurrence that was thought to reflect the developing autoimmune response. The autoantibodies had high affinity for red cells with very little free antibody detectable in the serum; in two instances Rh specificity was evident. Hemolysis was severe in four patients. Two of them had intravascular hemolysis, one of whom also had marked dyserythropoiesis and a transiently positive Ham′s test. Although IgA autoantibodies caused hemolysis predominantly through immune adherence, on occasions they also seemed to be able to induce complement activation, possibly via the alternative pathway. Prednisolone was the mainstay of treatment, and was occasionally augmented with azathioprine and intravenous immunoglobulin. Blood transfusion was required in two patients, both of whom eventually required splenectomy that resulted in full remission. The one patient with mild hemolysis recovered without treatment. Immunohematology 1996;12:14-19.

Ｊｋ｀ａ´自己抗体（ＩｇＧ１）が検出されたＡＩＨＡの１症例

Autoimmune hemolytic anemia (Hb 4.1g/dl, reticurocyte count 18.1%) developed in a 42-year-old woman who took any drug before admission. The supernatant of her red cell's eluate prepared from the procedure of adsorption technique with Jk (a- b-) normal red cells, contained antibody with anti Jka specificity. Her antibody was confirmed to have the characterization of IgG1.

An experimental model of autoimmune hemolytic anemia in mice.

Autoimmune hemolytic anemia was induced in C57Bl/6J mice by multiple injections of syngeneic erythrocytes coated with Influenza A (PR8) virus. Coombs positive erythrocytes and erythrocyte-binding immunoglobulins were demonstrated by the method of antiglobulin agglutination. Viable virus was not required to induce the response, although it was more effective than inactivated preparations, and presensitization to the virus was not a prerequisite. The induced disease was mildly hemolytic. Reactive anti-erythrocyte immunoglobulins were of the IgG class, and unlike those in sera of New Zealand strain mice, they were both strain- and species-specific. These results support the concept of a viral etiology for acquired hemolytic anemia. However, the response seen in the experimental model differs from the natural response in New Zealand mice in the persistence of the anemia and the specificity of the autoantibodies. The Gross leukemia virus, being indigenous to the New Zealand strain, may persist and induce inher...

Autoimmune hemolytic anemia: a review.

Autoimmune hemolytic anemia (AIHA) represents the most elegant model of autoimmunity in humans. It is frequently associated with other diseases of altered immune states. By definition, antibody must be demonstrable in every case. Two types of antibody, cold and warm, are recognized. They vary considerably in chemical structure, serologic expression, extent of damage to erythrocytes, and incidence. Warm antibodies are almost exclusively gamma G globulins, and affected erythrocytes are seqestered by the spleen. Cold antibodies are macroglobulins, and affected erythrocytes are usually destroyed in the entire reticuloendothelial system. Present therapy is based chiefly on the nature of the antibody.

Autoimmune Hemolytic Anemia, Acute Leukemia, and Carcinoma of the Lung

AUTOIMMUNE hemolytic anemia is not uncommonly associated with malignant reticuloendothelial disease. It has been frequently described in association with the chronic leukemias but rarely occurs in the acute types, and only one patient has been reported to have a concomitant carcinoma.1Dausset and Colombani2studied 128 patients with autoimmune hemolytic anemia; none had an underlying carcinoma or acute leukemia. We wish to present an unusual patient with polycythemia vera who was treated with sodium phosphate P 32 and developed acute myelogenous leukemia and adenocarcinoma of the lung complicated by an autoimmune hemolytic anemia. Report of a Case A 62-year-old white woman (04-92-98), entered the National Institutes of Health Clinical Center for the first time in May 1963, with a four-month history of night sweats, occipital headaches, and intense pruritis after bathing. Physical examination revealed a ruddy cyanosis; a blood pressure of 165/95 mm Hg; pulse rate, 100