INTESTINAL T-CELL LYMPHOMA PRESENTING AS SMALL BOWEL PERFORATION. P. Hulick 1 , H. Tun 1 ; 1 Mayo Clinic, Jacksonville, FL (Tracking ID #74266) LEARNING OBJECTIVES: 1. Recognize the association of primary gastrointestinal T-cell lymphoma and celiac disease 2. Recognize the poor prognosis, especially with +CD56 marker 3. Treatment must include systemic chemotherapy. CASE INFORMATION: A 73-year-old white female presented to the emergency department following an acute exacerbation of abdominal pain that had progressed over the past two months. The pain was originally diffuse in nature but became sharp in character with the acute presentation. The patient denied radiation to the back while movement exacerbated the pain. The patient reported decreased appetite with a fifteen-pound weight loss over the preceding six months, nausea but no vomiting, and intermittent fevers to a maximum of 105F over the preceding two months. The patient denied other constitutional symptoms. On admission, the patient was afebrile with normal vital signs. Physical exam was notable for diffuse abdominal discomfort on palpation, decreased bowel sounds of normal pitch, and the absence of generalized lymphadenopathy Laboratory studies were Hgb 11.1g/dL, MCV 85 fL, and platelet count of 385,000. Electrolytes, creatinine and liver profile were unremarkable. LDH was 185 u/L. Computed tomography (CT) scan of the abdomen/pelvis revealed a pneumoperitoneum without identification of a specific site of perforation. Exploratory laparotomy revealed a perforated small bowel mass (7.0 Â 4.0 Â 1.0 cm) with infiltrating neoplastic lymphocytes positive for CD 3, 43, and 56 staining. Flow cytometry showed a T-cell phenotype with anomalous expression of CD 103 (T-cell mucosal homing antigen receptor). Staging evaluation did not reveal evidence of metastatic disease. The pathology findings in our case are consistent with enteropathy associated T-cell lymphoma. Our patient presented with the rarer T-cell CD56 positive variant which has a poorer overall prognosis and aggressive course. IMPLICATIONS/DISCUSSION: Primary gastrointestinal lymphomas account for 30±40% of extranodal lymphomas, but they are usually of B-cell lineage. Celiac disease, a malabsorption disorder related to gluten intolerance, is thought to be a risk factor for developing primary intestinal T-cell lymphoma. Intestinal T-cell lymphoma continues to have a poor prognosis. CD56 (neuronal cell-adhesion molecule) positive tumors have been associated with a more aggressive course. Additional poor predictors include nutritional status at presentation and presence of a bowel perforation. Survival data for cases presenting with bowel perforation has been estimated at 28% and 0% six months and one year post diagnosis. Treatment consists of surgical resection of the tumor and adjuvant chemotherapy which consists of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) which our patient received. Systemic chemotherapy is necessary, regardless of stage, because of the malignant clonal population of T-cells that exists in the normal gastrointestinal mucosa. Relapse rate is thus high, even in patients with initial response to chemotherapy. year-old woman with recurrent ovarian cancer was hospitalized and conservatively managed for a partial small bowel obstruction. After a second unsuccessful attempt to anti-coagulate her right su-bclavian central venous catheter, the patient developed acute left-sided weakness associated with slurred speech. She denied chest pain but stated she was increasing short of breath overnight. Vital signs included a heart rate of 117 beats per minute and a respiratory rate of 26 breaths per minute. Physical exam was remarkable only for a left facial droop, left arm and leg weakness, and dysarthria. An emergent CT scan revealed an acute, non-hemorrhagic right-sided cerebral infarct. ECG, duplex scan of the carotid arteries and chest radiograph were normal. Cardiac isoenzymes were negative for myocardial infarction. An arterial blood gas analysis revealed severe hypoxia and an alveolar-arterial oxygen gradient of 42. A ventilation-perfusion scan showed high probability for pulmonary embolism and a transesophageal echocardiogram revealed a patent foramen ovale. IMPLICATIONS/DISCUSSION: Indwelling catheters can serve as a nidus for thrombus formation. Emboli may produce pulmonary sequela with concomitant increased right sided circulatory pressures. This results in paradoxical emboli entering the arterial circulation though a right to left shunt, such as a patent foramen ovale, with resultant cerebral ischemia. Patients who demonstrate symptoms of both a cerebral infarct and a pulmonary embolism should be evaluated with a CT scan, carotid duplex, ventilation-perfusion scan and transesophageal echocardiogram to identify the source and location of the embolus. Treatment involves immediate evaluation for emergent anticoagulation if not contraindicated. Operative closure of a patent foramen is not indicated if the elevation of right sided pressure is related to an acute event. year old Asian female without significant medical history presented with ten days of rash, fever, joint pains and stiffness. Rash, which was erythematous and non-pruritic, began on her thighs and spread to her back and chest. Joint pains spread from her hands and wrists to her shoulders, ankles and knees. Her course of illness included high fevers and a sore throat. Physical exam revealed temperature of 103.9 F and erythematous, salmon colored maculo-papules on her trunk and proximal extremities. Palms and soles were spared. The rash was exacerbated by fever spikes, which usually occurred in the afternoon. Koebner's phenomenon occurred when rubbing caused the rash to appear on previously uninvolved skin. Metacarpophalangial joints, wrists and left shoulder were tender, but without erythema, warmth or effusions. There was no lymphadenopathy or splenomegaly. Labs: WBC 22,000 (85% neutrophils, 5% bands). Blood cultures and tests for HIV, enterovirus, cytomegalovirus, Ebstein-Barr virus, hepatitis, parvovirus were negative. Anti-streptolysin O titers were negative. LFT, ANA, RF, chest xray, CT chest/abdomen/pelvis, echocardiogram were normal. Skin biopsy showed perivascular infiltrate, no vascultis. Patient was started on nonsteroidal anti-inflammatory medications and gradually responded to therapy. IMPLICATIONS/DISCUSSION: Adult Still's disease, although uncommon, should be considered in adults with fever, joint pains and rash. The diagnosis requires atleast 5 criteria, including atleast 2 major ones. The major criteria are temperature of > 39 C for atleast 1 week, arthralgias for atleast 2 weeks, typical rash (salmon, maculopapular, evanescent, koebners phenomenon), WBC > 10,000 (> 80% neutrophils). Minor criteria are sore throat, lymphadenopathy and/or splenomegaly, elevated transaminases or LDH, negative ANA and RF. Diagnosis also requires exclusion of infection, malignancy and other rheumatic diseases. Treatment is aimed at joint symptoms because extent and chronicity of arthritis is best predictor of long term consequences. Nonsteroidals, corticosteroids and immunomodulating drugs are therapeutic options. Choice of therapy depends upon organ involvement and severity of disease. LEARNING OBJECTIVES: 1) To recognize intussusception as a cause of intestinal obstruction, 2) To recognize that the treatment of adult intussusception differs from pediatric cases, 3) To recognize that adult intussusception is often a presentation of underlying pathology. CASE INFORMATION: A 53 year old male with a past history of small cell lymphoma treated three years prior with CHOP chemotherapy and radiation therapy presented to the emergency department with several days of diffuse, unremitting periumbilical abdominal pain. He denied melena or hematochezia, and, until recent constipation, his bowel habits were unchanged. His intake of food was reduced due to bloating, but he denied nausea or vomiting. He did not experience fatigue, night sweats, fevers, or weight loss. On examination, his abdomen was soft and non-distended. He had normal bowel sounds. Despite diffuse nonspecific tenderness, he did not exhibit guarding or rebound. The spleen and liver were normal size, and no masses were palpated. A rectal exam was normal, and stool hemoccult was negative. Because of his medical history, a CT scan of the abdomen was performed which revealed ileocecal intussusception and resulting obstruction. A barium enema confirmed an intussusception but was unable to reduce it. He was taken to the operating room where the surgeons found intra-abdominal lymphadenopathy and ischemia of the proximal colon requiring hemicolectomy. Pathology of the resected tissue revealed follicular B-cell lymphoma within the terminal ileum and cecum. IMPLICATIONS/DISCUSSION: Intussusception is a process of gastrointestinal invagination in which a proximal segment telescopes into a distal portion. While common in children as the second leading cause for acute abdomen, adult intussusception occurs in only 5±10% of all cases. Presentation may be either acute or of a chronic indolent nature. It is rarely idiopathic in adults as it is in children, and underlying pathology is readily identifiable in up to 90% of all cases. Common causes include polyps, Meckel's diverticulum, endometriosis, and inflammatory bowel disease, but 54%±69% of identified pathology are malignancies including lymphomas. The process of intussusception causes gastrointestinal circulatory obstruction which can lead to ischemia and bowel infarct. Avoiding delay in surgical intervention preserves tissue. While barium enema is both diagnostic and therapeutic in children, it is less efficacious in adults, and its role in adult therapy is actually controversial. Because of the likelihood of underlying pathology, primary resection is often the treatment of choice. Whether or not to attempt reduction before resection is arguable; some authors have suggested that time spent on barium enemas compromises the integrity of bowel tissue. While uncommon, intussusception should be readily recognized when it occurs. Treatment of the disease requires timely surgical intervention to preserve bowel tissue. It is paramount to recognize that treatment in adults differs from pediatric cases where barium enemas are often successful. Discovery of adult intussusception must arouse clinical suspicion of underlying pathology which should be addressed. He was taken to the local emergency department, which was unable to run ethylene glycol level assays. At the outside hospital, he had an anion gap of 25, but an osmolar gap was not measured. He was placed on an ethanol drip and transferred to a tertiary care center. A stat ethylene glycol level returned elevated at 432 mg/dL, and the osmolar gap was 140 mOsm/kg. At this time, although stuporous, he was arousable and conversed appropriately. Unfortunately, urine was not sent for analysis, but serum creatinine was 1.2 mg/dL. Instead of initiating hemodialysis, the patient was placed on intravenous fluids with dextrose and saline and given fomepizole every 12 hours. Treatment continued for the next 60 hours until his ethylene glycol level fell within a non-toxic range. Clinically, his mental status returned to baseline, and his serum creatinine improved to 0.8 mg/dL. The patient underwent psychiatric evaluation and after medical clearance was transferred to an inpatient psychiatric facility for treatment of his depression. IMPLICATIONS/DISCUSSION: Ethylene glycol is a sweet liquid found in antifreeze. The taste has led to accidental intoxication in young children, but ingestion also occurs during suicide attempts. It is metabolized into oxalate, which is excreted by the kidneys, through several enzymatic steps beginning with alcohol dehydrogenase. Early toxicity is manifested by mental status changes ranging from stupor to coma. Renal damage from direct toxicity to the kidneys develops later although the rate of occurrence depends on the severity of intoxication. Presumptive diagnosis, based on a history of ingesting solutions likely to contain ethylene glycol, such as antifreeze, is the first critical step in diagnosis. While ethylene glycol can be measured directly, the laboratory equipment may not be available readily in all emergency departments. However, diagnosis can be confirmed indirectly with basic laboratory tests. Ethylene glycol itself causes an increased osmolar gap, and its metabolite glycolic acid creates the anion gap metabolic acidosis. The end product of metabolism, oxalate, can precipitate in urine, and urinalysis may reveal calcium oxalate crystals. Treatment must occur expediently to preserve renal and neurological function. This should involve adequate hydration and inhibition of alcohol dehydrogenase to prevent breakdown of ethylene glycol into its toxic metabolites. Ethylene glycol is dialyzable, and severe intoxication may require hemodialysis. However, in most cases, fomepizole, a more efficacious inhibitor of alcohol dehydrogenase than ethanol, can be used alone without need for dialysis. If both methods are used the dose of fomepizole needs to be increased since it is a dialyzable agent. Fomepizole can preserve renal function without invasive vascular access required for hemodialysis. Recovery without permanent sequelae is possible but requires timely recognition of ethylene glycol intoxication and familiarity with the treatment methods. A 24 year old man with severe hemophilia A and AIDS was admitted to the medical service with new neurological deficits. Eight weeks prior to admission he was started on highly active retroviral therapy (HAART). Six weeks prior to admission he developed left facial droop and left upper extremity weakness. The patient's family described behavioral changes including mood disturbances and aggressive behavior. On the day of admission the patient experienced two witnessed generalized seizures. On physical examination he appeared chronically ill. Neurological examination revealed a left cranial nerve VII upper motor neuron palsy and left upper extremity weakness. There was no papilledema. An MRI of the brain revealed multifocal hyperintense signals in the right frontal, left frontal, and right parietal areas predominately affecting the white matter without enhancement or mass effect. A subsequent spinal tap revealed a normal opening pressure with 1 white blood cell, 0 red blood cells, total protein of 74, and glucose of 54. Evaluation of the cerebrospinal fluid for tuberculosis, bacteria, fungus, toxoplasmosis, syphilis, herpes, CMV, and cryptococcus were negative. JC virus DNA was detected by PCR from the CSF. Based on the clinical presentation, radiographic findings and positive JC virus DNA in the CSF, the diagnosis of progressive multifocal leukoencepholapthy (PML) was made. IMPLICATIONS/DISCUSSION: PML is a disorder characterized by multiple rapidly progressive focal neurological deficits. Hemiparesis, visual field defects, cognitive impairment, and cranial nerve deficits are most common. Intracranial pressure usually remains normal. Ninety-five percent of all patients with PML have an underlying immunosuppressive disorder such as HIV. Conversely, 1±3% of AIDS patients will develop PML. CT or MRI of the brain reveals multiple bilateral, usually asymmetric areas of demyelination. The lesions are usually localized to the periventricular area and subcortical white matter. There is no contrast enhancement or mass effect. The diagnosis is made based on the clinical picture of multiple neurologic deficits, characteristic radiographic findings, and isolation of the JC virus from the CSF. Detection of JC virus from the CSF has a sensitivity of 74±95% and a specificity of 92±100%. There is currently no proven effective therapy for PML. HAART therapy has been shown to slow the progression of the disease. However, most patients deteriorate rapidly within 6 months. LEARNING OBJECTIVES: 1. Recognize the importance of opthomologic exam in the differential diagnosis of aseptic meningitis 2. Understand the role of steroid therapy in Vogt-Koyanagi-Harada Syndrome. CASE INFORMATION: A 24-year-old previously healthy man was admitted to the medical service with progressively worsening generalized headaches, decreased visual acuity, and hearing loss for two weeks. The patient reported malaise, but denied nausea, vomiting and fevers. He had never traveled outside the United States and did not use drugs or alcohol. The patient was afebrile. HEENT examination revealed bilateral papilledema and decreased visual acuity and hearing. Neck examination was remarkable for nuchal rigidity. The neurological exam was otherwise normal. CT scan of the head and orbits confirmed the papilledema but did not reveal any mass effect. A subsequent spinal tap revealed normal opening pressure, 727 white blood cells with 70% lymphocytes, a protein = 81, and glucose = 53. The patient was begun on broad-spectrum antibiotics and anti-tuberculous therapy without significant improvement. RPR, HIV, ESR, and cryptococcal antigen were negative. Ophthalmologic consultation was obtained. Slit lamp examination revealed cells in the anterior chamber consistent with uveitis, choroidal inflammation, and depigmentation. The patient was diagnosed with Vogt-Koyanagi-Harada (VKH) syndrome based on the ophthalmologic findings, aseptic meningitis and hearing loss. The patient was started on high dose steroids with relatively rapid resolution of the headaches and visual and hearing loss. IMPLICATIONS/DISCUSSION: VKH is a uveomeningoencephaltic syndrome and appears to be an autoimmune reaction to melanocytes thus making it more common in Asians, Middle-Easterners, and Hispanics than whites. Melanocytes are located in the skin, uvea, retinal choriod, membrane of the inner ear, and leptomeninges, accounting for the particular pattern of involvement seen in this syndrome. The ocular findings may include cataracts, glucoma, and globe atrophy. Meningeal involvement may lead to encephalopathy, seizures, myelopathy, or other focal signs. CSF findings include a lymphocytic pleocytosis, elevated total protein, and normal opening pressure. Other manifestations of VKH include dysacousia, alopecia, poliosis, and vitiligo. Although the optimal steroid dosage for treating VKH has not yet been defined it is generally accepted to begin therapy with high-dose systemic steroids followed by oral steroids for at least 6 months. Patients typically have a rapid decrease in symptoms. In about half of the patients, disease recurs within six months of discontinuation of steroids.