‘B’ Is for Bacteria: Fever, Night Sweats, and Unintentional Weight Loss

Case Presentation:

A 48‐year‐old Hispanic man presented with 2 weeks of spiking fevers, night sweats, and unintentional loss of 10 pounds in 30 days. He denied diarrhea, abdominal pain, hematemesis, and melena. He endorsed one year of bilateral hip osteoarthritis. Physical exam was remarkable for bilateral inguinal and left axillary lymphadenopathy. The patient was found to be anemic and leukopenic with Hgb of 9.0 and WBC of 2.7. Abdominal CT showed mesenteric and retroperitoneal lymphadenopathy.

The patient was admitted for workup. Throughout hospitalization the patient spiked periodic fevers and was transfused for hemoglobin of 7.6 on hospital day 3. Nutrition was consulted due to the patient’s weight loss and poor oral intake. HIV, CMV, EBV, hepatitis, and tuberculosis were all negative. Upper endoscopy showed Candida esophagitis which was treated with nystatin. The patient was discharged on day 5 with improved energy and appetite.

Duodenal biopsy pathology revealed numerous PAS‐positive AFB‐negative rod‐shaped microorganisms engulfed by foamy histiocytes consistent with Whipple’s disease, confirmed by PCR assay of axillary lymph node biopsy. One month of daily intravenous ceftriaxone was initiated, achieving a dramatic clinical response within 2 weeks. This was followed by one year of oral antibiotics.

Discussion:

First described by George Hoyt Whipple in 1907, Whipple’s disease is caused by the bacteria Trophyrema whipplei and has an estimated worldwide incidence of 12 new cases per year.[1] It is described as presenting in stages, with a prodrome of oligoarthralgia preceding diarrhea and malabsorption.[2],[3] It is diagnosed by histopathology showing PAS‐positive, AFB‐negative stain of infected tissues, and PCR assay of biopsy specimens.[4] Although it is predominantly a gastrointestinal disease, infection with the causative organism can also result in cardiac [5],[6] and neuropsychiatric[7],[8]manifestations.

Based on his clinical presentation of “B” symptoms and lymphadenopathy, our patient was worked up for infectious and neoplastic processes, with non‐Hodgkins lymphoma and HIV highest on the differential.

Conclusions:

This case illustrates the potential for systemic symptoms to predominate over gastrointestinal manifestations of Whipple’s disease, creating a presentation consistent with hematologic malignancy or immune deficiency. We recommend that physicians be aware of the variable presentation of Whipple’s disease. We also recommend consideration of a prodrome of oligo‐ or polyarthritis in raising suspicion for Whipple’s disease in a patient presenting with “B” symptoms and lymphadenopathy.

[1] Dutly and Altwegg. Whipple’s disease and “Tropheryma whippelii.” Clin Microbiol Rev 2001, 14(3):561.

[2] Schwartzman. Whipple’s disease. Rheum Dis Clin N Am 2013, 39:313‐231

[3] Jones et al. A stain in time. N Eng J of Med 2007, 356:68‐74.

[4] Zaidan et al. Whipple disease mimicking adult‐onset Still’s disease and treated by anakinra: diagnosis using PCR. Scand J Rheumatol 2012, 41(4):321‐323.

[5] Algin et al. Tropheryma whipplei aortic valve endocarditis, cured without surgical treatment. BMC Research Notes 2012, 5:600.

[6] Fenollar et al. Whipple’s disease. N Eng J of Med 2007, 356:55‐66.

[7] Uryu et al. Central nervous system relapse of Whipple’s disease. Intern Med 2012, 51: 2045‐2050.

[8] Amendolara et al. Whipple’s disease infection surgical treatment: presentation of a rare case and literature review. G Chir 2013, 34(4):117‐121.