Purpose: We present the case of a 71 year old female of Japanese descent who presented with five months of chronic, watery diarrhea of up to 20 bowel movements daily. Her diarrhea was large volume without cramping, pain, or fevers, and persisted during the night and despite fasting. She also had a 20 pound weight loss and dehydration requiring eight hospitalizations for intravenous fluid administration and symptom control. Her past medical history was significant for human T-lymphotropic virus type I-associated adult T cell leukemia/lymphoma (HTLV-1 ATLL) diagnosed two years prior to admission. At that time, she also was diagnosed with Strongyloides stercoralis infection via small bowel biopsies. Though the parasite was successfully eradicated with ivermectin, strongyloides recurred four months prior to presentation, requiring further ivermectin treatment in addition to lifelong suppressive therapy. Past history also was remarkable for Clostridium difficile infection detected six months prior to presentation, successfully treated with a combination of oral metronidazole and vancomycin. A 72 hour stool collection confirmed large volume diarrhea of greater than 5000 grams. Investigation of the diarrhea was largely unremarkable, including evaluation of infectious (negative Clostridium difficile and enteric pathogen cultures, hepatitis serologies, strongyloides culture, ova and parasite studies, and small bowel aspirate), neuroendocrine (normal vasoactive intestinal peptide, thyroid studies, pancreatic polypeptide, calcitonin, 24 hour urine collection of 5-HIAA and histamine, gastrin and gastric pH level), anatomic (normal endoscopies, abdominal CT and MRI scans), and autoimmune (normal TTG, ANA, AMA, serum IgG/IgA levels, SPEP and UPEP) causes. She had little improvement with loperamide, octreotide, cholestyramine, or tincture of opium. Duodenal biopsy revealed intraepithelial lymphocytosis and an atypical CD4 positive T cell infiltrate, consistent with HTLV-1/ATLL infiltration of the small bowel. Chemotherapeutic treatment with alemtuzumab was recommended. HTLV-1 infection is endemic in many parts of the world, including southern Japan. It is a risk factor for strongyloides infection, which can present with diarrhea. Any patient with HTLV-1 infection and chronic diarrhea should be evaluated for strongyloides infection. Though there are few examples describing HTLV-1 lymphomatous infiltration of the small bowel, our case demonstrates it does occur. Refractory diarrhea in patients with HTLV-1 lymphoma requires extensive evaluation, and may even be an indication for chemotherapeutic treatment of ATLL, as in this case.
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