Neutropenic Enterocolitis Disclosing an Underlying Cyclic Neutropenia

Abstract

Neutropenic enterocolitis is a syndrome characterized by fever and abdominal pain in a neutropenic patient. It is often reported in children treated for leukemia and rarely reported in patients with other diseases. Herein, we report the case of a 9-year-old patient with a medical history of recurrent fever and mouth ulcers since the age of 4, who presented with neutropenic enterocolitis complicated with intestinal perforation which all leaded to disclose cyclic neutropenia. The patient was successfully treated by aggressive supportive care combined with surgical intervention. Neutropenic enterocolitis with possible complications should be considered and promptly managed in every neutropenic patient and may reveal a rare cause of neutropenia as cyclic neutropenia.

Cyclic Neutropenia Mimicking Crohn’s Disease: Two Case Reports and a Narrative Review

“…Septic shock due to severe infections represents rare complications of both diseases and digestive system infections, abdominal pain, and fatigue are common findings in CD and cyclic neutropenia [2,3,8,9]. Other clinical features such as periodontitis, alveolar bone loss, and cellulitis are characteristics of cyclic neutropenia but not of CD [2,3,8,9]. The overlap of the features may determine the occurrence of misdiagnosis as in our two clinical cases, so specialists should always be aware of the possibility of differential diagnosis [1,8,9,26].…”

“…Pneumonia, perianal abscesses, and impaired fertility affecting women but not men are rarely present [2]; women show a higher rate of abortions and 50% of their children inherit cyclic neutropenia [2,3]. Septic shock, peritonitis, and bone necrosis represent the more serious but rare manifestations of the disease [2,3,9]. Symptoms usually become less severe and less frequent after adolescence and therapy with antibiotics is frequently required, but cyclic neutropenia is not associated with increased risk of malignancy or conversion to leukemia [2,3].…”

“…An important point to focus on is the differential diagnosis of cyclic neutropenia and CD, since they share similar symptoms/signs and similar alteration in the laboratory tests. In fact, neutropenia can be common laboratory evidence in CD, because of infection and some drugs; similarly, neutropenia may show some clinical features common in CD, such as recurrent fever, oral mucosal ulcers, or perianal disease [1][2][3]9].…”

“…Septic shock due to severe infections represents rare complications of both diseases and digestive system infections, abdominal pain, and fatigue are common findings in CD and cyclic neutropenia [2,3,8,9]. Other clinical features such as periodontitis, alveolar bone loss, and cellulitis are characteristics of cyclic neutropenia but not of CD [2,3,8,9].…”

“…Clinical presentation ranges from mild to severe forms of the disease, with an absolute neutrophil count <0.2 × 10 9 /L for a period of 3-5 days, recurrent fever, painful mouth ulcers, gingivitis, and bacterial infections such as sinusitis, otitis, pharyngitis, and cellulitis [1,3,8]. Young patients can also manifest periodontitis and alveolar bone loss; pneumonia, perianal abscesses, impaired fertility, septic shock, and bone necrosis represent rare manifestations of the disease; symptoms usually become less severe and are less frequent after adolescence [1][2][3]9].…”