A case of hemophagocytic syndrome in a patient with fulminant ulcerative colitis superinfected by cytomegalovirus
A 52-year-old female was admitted to hospital with fever, abdominal pain, and hematochezia for 6 days. She had been diagnosed with UC (left-side colitis) 3 years earlier and had been treated with sulfasalazine. She was in remission 5 months earlier. However, she had stopped her medication 4 months earlier on her own. On physical examination, splenomegaly was found. The blood examination showed pancytopenia (hemoglobin 6.7 g/dL, hematocrit 20.3%, total leukocyte count 400/mm3 with an absolute neutrophil count [ANC] of 64/mm3, platelets 24,000/mm3). The erythrocyte sedimentation rate was 120 mm/hr and the C-reactive protein 34.09 mg/dL. The serum ferritin level was elevated (956.1 µg/L). The peripheral smear showed leukopenia (neutropenia and lymphopenia), normocytic, normochromic anemia, and thrombocytopenia. A bone marrow study showed 15% hypocellularity with decreased granulopoiesis and erythropoiesis. In addition, histiocytosis with hemophagocytosis was observed (). Viral testing was positive for CMV serum antigenemia. Sigmoidoscopy revealed continuous hyperemic friable mucosal lesions with spontaneous bleeding () and the histology showed multiple giant cells with intranuclear and cytoplasmic inclusion bodies (). Initially, considering the possibility of a UC flare-up, we started intravenous steroid. However, her condition did not improve. At that time, the bone marrow examination, viral assays, and sigmoidoscopic biopsy indicated CMV infection. Therefore, we started intravenous ganciclovir and tapered the steroid dose. Subsequently, her symptoms improved and the blood count normalized. She was discharged and has not relapsed during follow-up.