Aplastic anemia complicated with ulcerative colitis

A 37-year-old female who presented with pancytopenia in April 2008 was diagnosed with aplastic anemia stage 2 with a normal karyotype. She had a PNH phenotype in her red blood cells (RBC) and granulocytes, and HLA DR15. Her aplastic anemia was deteriorated from stage 2 to stage 3, and she required periodic RBC transfusions. Four months after cyclosporine therapy, the pancytopenia improved and she did not need RBC transfusion. However, three months thereafter, she again required RBC transfusions after developing severe ulcerative colitis. Although mesalazine and steroid pulse therapy improved her ulcerative colitis, her transfusion dependency persisted. Eleven months after the diagnosis of aplastic anemia, equine anti-thymocyte globulin (ATG) and cyclosporine were administered, but no hematological improvement was obtained. Six months after the administration of ATG and cyclosporine, transformation to refractory cytopenia with multilineage dysplasia (RCMD) with 7-monosomy was observed. An allogeneic bone marrow transplant (BMT) from a HLA-identical sibling was performed 23 months after the diagnosis of aplastic anemia. Complete remission of both the aplastic anemia and ulcerative colitis was obtained without medication. Although the relationship between aplastic anemia and ulcerative colitis remains unclear, immunological abnormalities might be involved in the pathogenesis of both disorders because she had PNH phenotype in RBC and HLA DR15 and because allogeneic BMT improved both disorders.     

Autoimmune hemolytic anemia associated with ulcerative colitis

Three patients are reported in whom autoimmune hemolytic anemia developed during the course of ulcerative colitis. A review of the literature yielded 11 additional cases, and the clinical, immunologic, and therapeutic characteristics are summarized. The results of steroid therapy and splenectomy are similar to those for idiopathic autoimmune hemolytic anemia. The cause and effect relationship between these two diseases is not clear, but colectomy appears to produce remission in hemolysis when the latter is refractory to both steroids and splenectomy.     

Budd-Chiari syndrome in a patient with ulcerative colitis: association with anticardiolipin antibodies

This is the first reported case of a young patient with ulcerative colitis who developed acute Budd-Chiari syndrome and was found positive for anticardiolipin antibodies. Although an association between ulcerative colitis and Budd-Chiari syndrome, and between the latter and antiphospholipid antibodies, has been shown in the literature, the coexistence of these three conditions has never been reported. The patient we describe did poorly and was considered as a candidate for liver transplantation in spite of prompt heparin therapy, probably because of the presence of multiple risk factors.     

Autoimmune hemolytic anemia in ulcerative colitis

Three cases of autoimmune hemolytic anemia in association with idiopathic ulcerative colitis are reported; the literature is reviewed; and therapeutic modalities are considered. Successful treatment is shown to have resulted with steroids alone, steroids and immunosuppressives, splenectomy, and colectomy. It is concluded that moderate or severe hemolysis should be treated first with high-dose corticosteriods; if unsuccessful, immunosuppressive therapy may be added or a splenectomy performed. Finally, total colectomy should be reserved for fulminant colitis and its complications and is not indicated solely for hemolysis.     

溃疡性结肠炎合并贫血76例临床分析

目的了解溃疡性结肠炎（UC）合并贫血的相关临床特点，探讨不同临床类型、病情严重程度及病变范围与贫血发生的关系。方法收集我院收治的76例溃疡性结肠炎患者的临床病例资料，并分析其临床特点及外周血红细胞相关的生物学指标。结果76例溃疡性结肠炎患者中发生贫血37例（占48．69％），其中轻度贫血19例，中度贫血12例，重度贫血6例；小细胞低色素性贫血21例（占56．76％），正常细胞性贫血9例（占24．32％），大细胞性贫血7例（占18．92％）。贫血以小细胞性和正常细胞性贫血为主；贫血与临床类型、病情严重程度无关，与病变累硬部位相关。结论贫血在溃疡性结肠炎患者中较为常见，其发生可能与消化道急慢性失血、肠道炎症所致铁吸收减少、溶血等因素有关。     

Coombs-positive autoimmune hemolytic anemia in ulcerative colitis

Summary Coombs-positive autoimmune hemolytic anemia is a rare complication of ulcerative colitis, occurring in fewer than 1% of cases. We have found eight patients with autoimmune hemolytic anemia in a series of 1150 hospitalized patients with ulcerative colitis (0.7%). There was a marked preponderance of female patients FM=71, in accord with previous reports. The hemolytic anemia appeared at a mean of 10 years after the onset of colitis, apparently independent of the age of the patient. Although seven of the eight patients had active colitis at the time of diagnosis of anemia, there was no consistent relationship to the extent of the bowel disease.Seven of the eight patients survived. One patient showed a partial response to steroid therapy, two responded to splenectomy, but four required both colectomy and splenectomy. Steroids should be the first line of therapy followed by splenectomy, if necessary. For those patients who have severe colitis, which in itself would merit surgery, a total proctocolectomy combined with splenectomy seems advisable.