RAPIDLY PROGRESSIVE CARCINOID SYNDROME IN AN IMMUNOSUPPRESSED MALE. J.R. Pederson 1 , J.J. Yium 1 , M. Panda 1 ; 1 University of Tennessee±Chattanooga, Chattanooga, TN LEARNING OBJECTIVES: 1. Recognize the increased risk for malignancy in an immunosuppressed patient 2. Recognize the signs and symptoms of carcinoid syndrome. CASE INFORMATION: A 61 year old male, status post renal transplantation in 1995 for chronic renal insufficiency, was admitted in 9/1999 with complaints of weight loss, diarrhea, anorexia and early satiety. The patient noted a gradual onset of symptoms since 1996, with worsening over a six month period prior to presentation. He was on immunosuppressive therapy since 1995, Physical exam revealed a cachetic white male with redness and telangectasias of the face and neck. His physical exam was otherwise unremarkable. CBC, electrolytes and liver function tests were all within normal limits. Ultrasound of the abdomen, CT pelvis and colonoscopy in 1996 and panendoscopy in 1999 were normal Work up was negative for malabsorption, H. pylori, Giardia and C.difficile. He was readmitted a month later for right hip fracture. He still had diarrhea and weight loss but also noted prominent bilateral facial, neck, and arm flushing. Punch biopsy from the skin of the neck was essentially unremarkable. Post-op CT of the abdomen revealed innumerable hepatic lesions in all lobes of the liver. Ultrasound guided liver biopsy revealed metastatic carcinoid tumors. Subsequent 24 hour urine collection revealed elevated 5-HIAA levels. The patient was treated with sandostatin and antihistamines. He rapidly deteriorated and died within several months of diagnosis. DISCUSSION: Carcinoid accounts for 30 ± 40% of all small intestinal tumors. Over 95% of all GI carcinoids occur in one of 3 sites: the rectum, appendix or small intestine (usually the ileum). Tumors less than 2 cm are less likely to metastasize. These tumors secrete vasoactive materials responsible for the clinical manifestations of carcinoid syndrome which is seen only in patients with hepatic metastasis. With advanced disease, treatment is symptomatic and true cure is seldom achieved. Rapid progression of indolent carcinoid has been reported inpatients who are immunosuppressed. As our patients preliminary extensive GI work-up did not reveal an obvious primary, we postulate that our patient may have primary hepatic carcinoid which rapidly progressed due to his immunosuppressed state. There has been only one case reported in the literature of primary hepatic carcinoid in a renal transplant patient. With the world wide increase in the incidence of tumors among immunosuppressed patients, physicians should have a low threshold for early thorough workup of suspected tumors. A 34 year old black male presented with a 2 week history of low grade fever, night sweats, generalized fatigue, weakness and myalgias. He had a waxing and wanning rash on his face and arms. Past medical history was significant for hypertension since age 26 and a similar rash and fever about 12 years ago. On examination he was febrile (temperature of 103F) and tachycardic. Physical examination was unremarkable except for a 2Â2cm right submandibular lymph node, a few lymph nodes in the right posterior cervical triangle and a hyperpigmented maculopapular skin rash involving the upper extremities and the face. The laboratory data showed WBC: 2th/mm3, Hbg./Hct.À8.4gm/dl 25.8%. The blood cultures, urine cultures, chest x-ray, imaging studies of the head, neck, thorax, and abdomen were all negative. ESR: 98, CRP 8.4mg/dl. Rheumatologic workup, viral and rickettsial serology, RPR and HIV were negative. Bone marrow biopsy and culture was also negative. The right submandibular lymph node biopsy showed histiocytic necrotizing lymphadenitis. He was started on Vioxx for the fever and myalgia. The patient's fever subsided after a total of 5 weeks. He returned with vision problems and was found to have retinal hemorrhages bilaterally with normal disc margins. Steroids were initiated for ocular symptoms with some improvement. DISCUSSION: Kikuchi's disease was first described in Japan in 1972 by Kikuchi and Fujimoto. It is a self limiting disease involving different ages, races and geographic regions. The etiology of this disease is uncertain, although its association with certain viruses like CMV, EBV, HSV, Parvo B-19, HIV, Yersinia and Toxoplasmosis has been described. These viruses or other antigens can induce immunological abnormalities in a susceptible individual with progression to autoimmune diseases like SLE and Still's disease. The most common clinical features of Kikuchi's disease are fever, weakness, fatigue, myalgia, arthralgia and lymphadenopathy (especially cervical). Skin rash is non-characteristic particularly involving the upper regions of the body. Hepatosplenomegly and neurological manifestations like aseptic meningitis and ataxia have also been described. However this is the first case described with retinal changes. Kikuchi's syndrome needs to be differentiated from other diseases like malignant lymphoma, SLE, tuberculosis, sarcoidosis, and cat scratch disease. As the course of Kikuchi's disease is generally self limited, and histopathology is diagnostic it is important to include it in the differential diagnosis of FUO.