Autologous peripheral blood stem cell transplantation and anti-B-cell directed immunotherapy for refractory auto-immune haemolytic anaemia.
ABSTRACT We report the clinical course of a 6.5-year-old boy with refractory auto-immune haemolytic anaemia. Due to failure of conventional immunosuppressive therapy, an autologous peripheral blood stem cell transplantation was performed. The conditioning regimen consisted of cyclophosphamide and anti-thymocyte globulin. The patient was reinfused with 2.6 x 10(6) CD34 positive selected, B- and T-cell-depleted peripheral blood stem cells per kg body weight. He showed a partial response with a reduced demand for red blood cell transfusions. However, due to persistence of the haemolytic process he was started on rituximab therapy on day +40 post-transplant. Following two doses of rituximab, the patient improved rapidly and developed a sustained complete response. After 10 months, haemolysis recurred and responded again to rituximab therapy without the necessity for red blood cell transfusions. 15 months after initial antibody treatment, however, the patient developed a second relapse which was now refractory to rituximab therapy although CD20+ B-lymphocytes were cleared from the peripheral blood. CONCLUSION: Our case report suggests that rituximab and autologous peripheral blood stem cell transplantation are important though not curative elements in the treatment of patients with severe auto-immune haemolytic anaemia who are refractory to conventional immunosuppressive therapy.
- SourceAvailable from: Natalia Maximova[Show abstract] [Hide abstract]
ABSTRACT: The possible role of haematopoietic SCT (HSCT) for the treatment of severe autoimmune diseases was originally supported by animal experiments and remission of concomitant autoimmune diseases in patients undergoing transplantation for haematological disorders. Since 1996, over 100 procedures were performed in children with different severe autoimmune diseases such as juvenile idiopathic arthritis, systemic lupus erythematosus, systemic sclerosis, immune cytopaenias and Crohn's disease. This review tries to summarize the published data on efficacy and toxicity of HSCT in this group of patients.Bone Marrow Transplantation 06/2008; 41 Suppl 2:S96-9. · 3.54 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Autoimmune hemolytic anemia (AIHA) is an immune mediated destruction of erythrocytes, which has a good prognosis in children. It is known to have chronic, remitting or relapsing course, especially in infants and adolescents. Treatment of refractory or relapsing AIHA is a challenge as the other aim of the treatment is to avoid prolonged exposure to steroids or other immunosuppressants in small children. Rituximab is used in patients who are non-responsive to conventional treatment such as steroids, intravenous immunoglobulins and transfusion therapy. It has varying therapeutic success rate. We report a case of AIHA in a 4-month-old infant who had ill-sustained response to conventional therapy, but responded to rituximab.Indian Journal of Pharmacology 01/2013; 45(5):526-527. · 0.58 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: Autoimmune hemolytic anemia (AIHA) is a disease where patients produce antibodies against erythrocytes directed towards membrane glycoproteins adsorbed onto the erythrocyte surface. Drugs and other associations have been implicated. It is described and discussed a case of livedo reticularis associated with AIHA treated with peripheral blood stem cell transplantation (PBSCT) that went into full remission for 10 years. After that period the patient relapsed and was treated with antibody anti-CD20, rituximab, and is now in full remission. The role of PBSCT and rituximab in the treatment of AIHA will be discussed.Revista Brasileira de Reumatologia 02/2012; 52(1):122-4. · 0.86 Impact Factor