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Transplant-associated thrombotic microangiopathy (TA-TMA) is considered one of the most severe complications after hematopoietic stem cell transplantation (HSCT). Transplant-associated thrombotic microangiopathy (TA-TMA) constitutes a form of microangiopathic haemolytic anemia and thrombocytopenia derived from a generalized endothelial dysfunction with intravascular platelet activation and formation of platelet-rich thrombi within the microcirculation. Since clinical features are common in several post hematopoietic stem cell transplantation (HSCT) complications such as capillary leak syndrome, engraftment syndrome, graft versus host disease (GVHD), diffuse alveolar hemorrhage and veno occlusive disease (VOD), an initial diagnosis of TA-TMA is not always certain, which is a principal reason for the failure of proposed treatments. [ABSTRACT FROM AUTHOR]

Immunodeficiency and autoimmune disease may occur concomitantly in the same individual. Some of the immunodeficiency syndromes, especially humoral defects are associated with autoimmune disorders. Hematological manifestations such as thrombocytopenia and hemolytic anemia are the most common presentations. Persistent antigen stimulation due to an inherent defect in the ability of the immune system to eradicate pathogens is the primary cause leading to autoimmunity in patients with primary immunodeficiency states. We describe a 10 year old Iranian girl with chronic granulomatous disease -the autosomal recessive type with mutation of NCF1 gene P47-associated with selective IgA deficiency, refractory immune thrombocytopenia that showed an excellent response to Rituximab (Anti-CD20 monoclonal antibody). Patients with primary immunodeficiencies may have variable autoimmune manifestations. So for early detection and appropriate treatment, autoimmune diseases should always be suspected in such patients. [ABSTRACT FROM AUTHOR]