Autoimmunity is thought to play a central role in the pathogenesis of inflammatory bowel disease and associated extraintestinal manifestations. Autoimmune hemolytic anemia associated with ulcerative colitis is a rare occurrence. No more than 50 cases have been described in the international literatures, and only 2 cases reported in Korea. A 29-year-old woman who was diagnosed as ulcerative colitis two years ago was complicated with autoimmune hemolytic anemia, and did not respond to steroid therapy. Ultimately, total colectomy and splenectomy were carried out for the treatment of ulcerative colitis and hemolytic anemia. After the operation, anemia was resolved. We present the case with a review of literature.
Adult ; Anemia, Hemolytic, Autoimmune/*diagnosis/drug therapy/etiology ; Colectomy ; Colitis, Ulcerative/complications/*diagnosis/pathology ; Drug Resistance ; Female ; Humans ; Splenectomy ; Steroids/therapeutic use

A 32-yr-old male diagnosed with myelodysplastic syndrome underwent an unmanipulated, unrelated, HLA matched, peripheral blood stem cell transplantation. The patient and donor were both blood type O, CcDEe. Twelve weeks post-transplantation, he developed acute autoimmune hemolytic anemia (AIHA). He was transfused multiple times with washed O red cells. High-dose steroid therapy was initiated and he underwent splenectomy; however, AIHA was refractory to therapy. The patient was further treated with combined treatment modalities including immunosuppressive therapy with mycophenolate mofetil and cyclosporine and three cycles of plasma exchange, and AIHA responded to treatment. This is the third case of AIHA complicating hematopoietic stem cell transplantation reported in Korea. Since AIHA is relatively common after hematopoietic stem cell transplantation, accurate and timely diagnosis of the disease and treatment strategies with multiple modalities are necessary.
Adult ; Anemia, Hemolytic, Autoimmune/*diagnosis/drug therapy/etiology ; Combined Modality Therapy ; Cyclosporine/therapeutic use ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Humans ; Male ; Mycophenolic Acid/analogs & derivatives/therapeutic use ; Myelodysplastic Syndromes/complications/diagnosis/therapy ; Plasma Exchange

A 37-year-old male presented with fever and jaundice was diagnosed as hepatitis A complicated with progressive cholestasis and severe autoimmune hemolytic anemia. He was treated with high-dose prednisolone (1.5 mg/kg), and eventually recovered. His initial serum contained genotype IA hepatitis A virus (HAV), which was subsequently replaced by genotype IIIA HAV. Moreover, at the time of development of hemolytic anemia, he became positive for immunoglobulin M (IgM) anti-hepatitis E virus (HEV). We detected HAV antigens in the liver biopsy specimen, while we detected neither HEV antigen in the liver nor HEV RNA in his serum. This is the first report of hepatitis A coinfected with two different genotypes manifesting with autoimmune hemolytic anemia, prolonged cholestasis, and false-positive IgM anti-HEV.
Adult ; Anemia, Hemolytic, Autoimmune/*diagnosis/drug therapy/etiology ; Anti-Inflammatory Agents/therapeutic use ; Cholestasis/*diagnosis/drug therapy/pathology ; Coinfection/*diagnosis ; Genotype ; Hepatitis A/complications/*diagnosis/genetics ; Hepatitis E/complications/*diagnosis/genetics ; Humans ; Immunoglobulin M/blood ; Liver/pathology/virology ; Male ; Prednisolone/therapeutic use ; RNA, Viral/blood

BACKGROUNDAutoimmune hemolytic anemia (AIHA) is an uncommon complication of allogeneic hematopoietic stem cell transplantation (allo-HSCT) which has only been reported in a few cases. We here aimed to explore its mechanism.

METHODSWe retrospectively analyzed 296 patients who underwent allo-HSCT in our center from July 2010 to July 2012. Clinical manifestations were carefully reviewed and the response to currently available treatment approaches were evaluated. The survival and risk factors of AIHA patients after allo-HSCT were further analyzed.

RESULTSTwelve patients were diagnosed with AIHA at a median time of 100 days (15-720 days) after allo-HSCT. The incidence of AIHA after allo-HSCT was 4.1%. IgG antibody were detected in ten patients and IgM antibody in two patients. The two cold antibody AIHA patients had a better response to steroid corticoid only treatment and the ten warm antibody AIHA patients responded to corticosteroid treatment and adjustment of immunosuppressant therapy. Rituximab was shown to be effective for AIHA patients who failed conventional therapy. Survival analysis showed that the combination of AIHA in allo-HSCT patients hinted at poor survival. Cytomegalovirus (CMV) infection, graft-versus-host disease (GVHD) and histocompatibility leukocyte antigen (HLA) mismatch seemed to increase the risk of developing AIHA.

CONCLUSIONSPatients who develop AIHA after allo-HSCT have poor survival compared to non-AIHA patients. Possible risk factors of AIHA are CMV infection, GVHD, and HLA mismatch. Rituximab is likely to be the effective treatment choice for the refractory patients.

Adolescent ; Adrenal Cortex Hormones ; therapeutic use ; Adult ; Anemia, Hemolytic, Autoimmune ; diagnosis ; drug therapy ; etiology ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Child ; Child, Preschool ; Female ; Graft vs Host Disease ; diagnosis ; drug therapy ; etiology ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Rituximab ; Transplantation, Homologous ; adverse effects ; Young Adult