Acute cytomegalovirus infection-associated hemophagocytic syndrome in a patient treated with azathioprine

Abstract

The authors present the case of a 21-year-old woman with ulcerative colitis. Azathioprine treatment was complicated with pancytopenia and septic shock. Acute cytomegalovirus infection related to the immunosuppressive therapy, resulting in hemophagocytosis syndrome and neutropenic fever was diagnosed. Recovery was achieved by the administration of parenteral ganciclovir, broad spectrum antibiotic and complex intensive care.

Successful treatment of cytomegalovirus associated hemophagocytic lymphohistiocytosis with the interleukin 1 inhibitor – anakinra

“…Viral infection, which is often respiratory, is a common cause of secondary HLH and CMV‐associated HLH appear to be a particular risk in patients with ulcerative colitis treated with azathioprine [2]. In secondary HLH, it is important to treat the underlying cause [1].…”

“…HLH may be primary (genetic etiology) or secondary to malignancies, autoimmune disease, or infections (most commonly viral). There have been multiple previous reports of cytomegalovirus (CMV) associated HLH developing in patients with ulcerative colitis receiving azathioprine [2]. We report the successful management of this syndrome with ganciclovir, corticosteroids and the new interleukin 1 inhibitor anakinra.…”

Chronic active Epstein–Barr virus enteritis: A literature review

“…A retrospective study demonstrated that patients with IBD using azathioprine or 6‐mercaptopurine significantly increased the risk of developing EBV‐associated lymphoma 34 . Hemophagocytic syndrome has also been increasing in IBD patients treated with azathioprine and infliximab 35–38 …”

Cytomegalovirus-Associated Hemophagocytic Syndrome in a 59-Year-Old Woman with Ulcerative Colitis

“…Cytomegalovirus-associated hemophagocytic lymphohistiocytosis in inflammatory bowel disease patients has rarely been reported. 2,3 At least 5 cases have occurred specifically in inflammatory bowel disease patients treated with azathioprine. 3 Theoretically, immunosuppression with azathioprine favors cytomegalovirus reactivation, which, in combination with the underlying inflammatory state, may predispose patients to uncontrolled cytokine activation and thus, hemophagocytic lymphohistiocytosis.…”

“…2,3 At least 5 cases have occurred specifically in inflammatory bowel disease patients treated with azathioprine. 3 Theoretically, immunosuppression with azathioprine favors cytomegalovirus reactivation, which, in combination with the underlying inflammatory state, may predispose patients to uncontrolled cytokine activation and thus, hemophagocytic lymphohistiocytosis. 2 Antiviral therapy in conjunction with corticosteroids, immunoglobulins, and chemotherapy has been used.…”