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Image challenge: Unusual fever and diarrhea in an infant

What caused recurrent fever associated with diarrhea in a nine-month-old boy even after being treated with antibiotics?

Gastroenterology clinical image challenge: A nine-month-old boy was admitted for recurrent fever associated with diarrhea that had lasted for more than two months. Physical examination showed a moderate anemic appearance, scattered petechiae at neck and abdomen, the spleen touchable 3 cm under the rib edge, but liver not palpable under the ribs. Laboratory tests revealed white blood cells 10,800/mm3, hemoglobin 6.0 g/dL, platelets 287,000/mm3, albumin 28.5g/L, C-reactive protein 85 mg/dL (normal, <8 mg/dL). Stool for occult blood test was positive. Colonoscopy on days seven and 22 of admission revealed multiple patchy erythema, uplift, erosion and ulcers in rectal mucosa, sigmoid colon and descending colon (figure). Pathologic examination of the colonic mucosa by hematoxylin and eosin staining revealed the presence of histocytic cells with prominent pleomorphism in the lamina propria; this was primarily adjudged as lymphatic hematopoietic system tumor (figure). Bone marrow biopsy was performed on sternum and anterior superior iliac spine, with results indicating signs of infection; however, there was no growth of tumorous cells. On his eighth day in the hospital, coagulation tests showed the following: prothrombin time (PT) 18.1 seconds (normal, 13.1 seconds); activated partial thromboplastin time (APTT) 48.2 seconds (normal range, 26-40 seconds), international normalized ratio (INR) 1.38 (normal range, 0.85-1.15), thrombin time (TT) 14.5 seconds (normal range, 14-21 seconds), d-dimer 3650 μg/L (normal, <550 μg/L). Although he was put on antibiotics (including cefotaxime, cefoperazone and sulbactam), along with administration of plasma infusion and blood transfusion, intravenous prednisone (19 mg/d) for five days and dexamethasone (2 mg/d) afterward for two weeks, the symptoms did not resolve. Fever and diarrhea persisted and progressive hepatosplenomegaly was observed.

Abdominal computed tomography scans showed enlarged lymph nodes in the mediastinum, abdominal, and retroperitoneal regions, as well as pelvic and inguinal regions (figure). A computed tomography-guided needle biopsy of anterior mediastinal lymph nodes was performed on the 36th day of admission. On the 48th day of admission, scattered pinpoint spots were observed on patient’s whole body, and repeated coagulation test evidenced the following: PT, 22.2 seconds; APTT, 78.2 seconds; TT, 18.8 seconds; and INR, 1.90. 

What is the diagnosis on the basis of patient’s clinical feature and laboratory tests?

To find out the diagnosis, read the full case in Gastroenterology or download our Clinical Image Challenge app through AGA App Central, which features new cases each week. Sort and filter by organ, most popular or favorites. AGA App Central is available in both the Apple App Store and Google Play

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