A case of hemophagocytic syndrome in a patient with fulminant ulcerative colitis superinfected by cytomegalovirus

Abstract

Hemophagocytic syndrome (HPS) is an uncommon hematological disorder that manifests as fever, splenomegaly, and jaundice, with hemophagocytosis in the bone marrow and other tissues pathologically. Secondary HPS is associated with malignancy and infection, especially viral infection. The prevalence of cytomegalovirus (CMV) infection in ulcerative colitis (UC) patients is approximately 16%. Nevertheless, HPS in UC superinfected by CMV is very rare. A 52-year-old female visited the hospital complaining of abdominal pain and hematochezia for 6 days. She was diagnosed with UC 3 years earlier and had been treated with sulfasalazine, but had stopped her medication 4 months earlier. On admission, her spleen was enlarged. The peripheral blood count revealed pancytopenia and bone marrow aspiration smears showed hemophagocytosis. Viral studies revealed CMV infection. She was treated successfully with ganciclovir. We report this case with a review of the related literature.

Keywords: Colitis, ulcerative; Cytomegalovirus infections; Inflammatory bowel diseases; Lymphohistiocytosis, hemophagocytic.

Figure 1

Bone marrow biopsy findings. (A) The biopsy section shows hypocellularity (H&E, × 200) and (B) histiocytes containing red blood cells (black arrow), and cellular debris or particles (Wright & Giemsa stain, × 1,000).

Figure 2

Sigmoidoscopic findings. (A) A continuous hyperemic friable mucosal lesion with spontaneous bleeding is observed, and (B) multiple pseudopolyps (black arrow) are noted.

Figure 3

The sigmoidoscopic biopsy shows giant cells with intranuclear and cytoplasmic inclusion bodies (black arrow) (H&E, × 400).