Giant cell hepatitis with autoimmune hemolytic anemia in a Korean infant

Giant cell hepatitis (GCH) with autoimmune hemolytic anemia (AHA) is a very rare disease characterized by early onset and severe clinical manifestations, including immune hemolytic anemia and hepatitis with cholestasis. The prognosis is poor despite aggressive immunosuppressive therapy. We report here the first case of GCH with AHA in East Asia. A 2‐month‐old boy was admitted with jaundice. Blood test indicated abnormal liver function and low hemoglobin. Direct Coombs test and several autoantibodies associated with liver disease were positive, and liver biopsy was consistent with GCH. He was treated with prednisolone and ursodeoxycholic acid, and at the time of writing was in clinical and biochemical remission after prednisolone was stopped.     

Successful treatment of refractory autoimmune hemolytic anemia with monthly rituximab following nonmyeloablative stem cell transplantation for sickle cell disease

Autoimmune hemolytic anemia (AIHA) can occur following hematopoietic stem cell transplantation (HSCT) and may be associated with other cytopenias. It can also occur in the context of chronic red cell transfusion in patients maintained on hypertransfusion regimens. There are an increasing number of reports on the successful treatment of autoimmune cytopenias with the monoclonal anti-CD20 antibody rituximab, including a few patients in a post-HSCT setting. The authors report the successful treatment with rituximab of refractory AIHA following allogeneic nonmyeloablative bone marrow transplantation in a child with sickle cell disease.     

Rubella infection with autoimmune hemolytic anemia

A 4-yr-old boy developed autoimmune hemolytic anemia after rubella infection and clinical manifestations cleared up after decrease in rubella specific IgM titer without any specific therapy.     

Abdominal tuberculosis with autoimmune hemolytic anemia

An eight-year-old male child presented with progressive distension of abdomen, fever, pallor and jaundice with a history of tubercular contact. Investigations were suggestive of abdominal tuberculosis with autoimmune hemolytic anemia. The child responded well to a course of oral steroids with antitubercular treatment. A literature search did not reveal any previous case report of an association between tuberculosis and autoimmune hemolytic anemia.     

Pernicious anemia associated with autoimmune hemolytic anemia and alopecia areata

We report a 16-year-old male with a combination of pernicious anemia, auto-immune hemolytic anemia and alopecia areata. Autoimmune hemolytic anemia coexisted with pernicious anemia but was diagnosed only when the anemia failed to respond to cobalamin therapy. Alopecia areata occurred 9 years later.     

Severe autoimmune hemolytic anemia with renal neoplasm

Autoimmune hemolytic anemia is a type of hemolytic anemia characterized by autoantibodies directed against red blood cells shortening their survival. When autoimmune hemolytic anemia is secondary to a paraneoplastic process, severe anemia can occur leading to significant morbidity and even mortality. Here we discuss the literature and present the case of a child with autoimmune hemolytic anemia from a paraneoplastic syndrome secondary to a renal tumor.     

Successful treatment of tacrolimus‐related pure red cell aplasia and autoimmune hemolytic anemia with rituximab in a pediatric cardiac transplant patient

Acquired pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) are rare complications of immunosuppression in pediatric solid organ transplant patients. We report a 14‐month‐old female child who developed Coombs positive hemolytic anemia and reticulocytopenia while on tacrolimus after cardiac transplantation. She was successfully treated with rituximab after failing treatment with corticosteroids and intravenous immunoglobulins. Clinicians should consider PRCA differential diagnosis in a patient presenting with reticulocytopenia and hemolysis. In addition, the coexistence of PRCA with AIHA, and the response to therapy with rituximab, supports a common immune‐mediated pathogenesis for both disorders.     

Interstitial nephritis in autoimmune hemolytic anemia

A case is reported of a 15-year-old boy with chronic autoimmune hemolytic anemia who developed renal insufficiency 3 years after splenectomy. An interstitial nephritis with striking lymphocytic infiltrates and sclerosed glomeruli could be demonstrated by percutaneous renal biopsy. Renal symptoms disappeared promptly after corticosteroid therapy. The renal lesions are thought to have arisen as part of the autoimmune disease.     

Successful treatment of mixed‐type autoimmune hemolytic anemia with rituximab in a child following liver transplantation

Haller W, Hind J, Height S, Mitry R, Dhawan A. Successful treatment of mixed‐type autoimmune hemolytic anemia with rituximab in a child following liver transplantation.
Pediatr Transplantation 2010: 14: E20–E25. © 2009 John Wiley & Sons A/S. Abstract: Development of a severe form of mixed‐type AIHA after orthotopic liver transplantation is a rare, but a life‐threatening event. We report a case of mixed‐type AIHA that developed in a child after hepatocyte and living‐related orthotopic liver transplantation for factor VII deficiency.     

IgA-mediated autoimmune hemolytic anemia in an infant

Autoimmune hemolytic anemia (AIHA) is characterized by the presence of autoantibodies, most frequently of the IgG isotype, directed against erythrocyte surface antigens. The direct antiglobulin test (DAT) is the critical laboratory test for the diagnosis of AIHA, but is negative in 3-11% of cases. In these cases of DAT negative AIHA, a wider spectrum of clinical data including more specialized testing for erythrocyte autoantibodies may be required. We describe the unique and challenging case of an infant with corticosteroid-responsive, DAT negative AIHA, in which specialized gel card testing identified an isolated IgA autoantibody on the erythrocyte surface.     

Rituximab for treatment of autoimmune hemolytic anemia

We report the successful use of rituximab as single treatment modality in a five-month-old boy with fulminant warm autoantibody autoimmune hemolytic anemia, resistant to standard treatment. On admission, laboratory tests showed a profound anemia with a hemoglobin of 2.6 g/dL. Indirect and direct antiglobulin tests were strongly positive, and nonspecific IgG autoantibodies were detected. Two days of intravenous corticosteroids (methylprednisolone 4mg/kg) and immunoglobulins (1g/kg) did not halt the hemolysis and the infant was severely transfusion-dependent. Rituximab 375mg/sq m weekly was given for 4 weeks, the hepatosplenomegaly gradually regressed, the lymphocytes normalized and he is free from hemolysis two years after treatment.