AJG – September, Suppl., 2003
lymphocytic infiltrate of the lamina propria by small lymphocytes that were CD3⫹, CD4⫹, CD8⫺ and CD20⫺. Results of PCR for TCR gamma, analysis of the T cell receptor beta chain by Southern blotting were indicative of a monoclonal population of CD4⫹ T cells and consistent with a diagnosis of non-Hodgkin’s T-cell lymphoma. Patient’s symptoms improved while he was treated with chemotherapy in 2001. He developed interval prostate carcinoma and thyroid carcinoma but at last follow up in 2003 he had intermittent diarrhea and weight loss but was doing relatively well. Conclusions: We report a rare case of the newly described entity of low-grade, diffusely infiltrating CD4⫹ T-cell lymphoproliferative disorder. The only similar description was recently published as four case reports by Carbonel et al. Our case illustrates the point that some patients with this disorder may have a relatively slow course, and remain undiagnosed for years. This disorder may clinically mimic refractory sprue. Poor response to gluten free diet should lead to suspicion of other disorders, such as diffusely infiltrating low-grade T-cell lymphoma. A careful histopathologic examination with immunohistochemistry and molecular studies for T-cell clonality are required for correct diagnosis.
616 CYTOMEGALOVIRUS: GASTROINTESTINAL INFECTION CAN HAVE MANY PRESENTATIONS Sara H. Mitchell, M.D., Chad E. Potteiger, M.D., Michael J. Komar, M.D., Donald S. Schneider, M.D.*. Geisinger Medical Center, Danville, PA. Cytomegalovirus (CMV) is a herpes virus that infects most of the world’s population and can cause a variety of gastrointestinal processes. It most commonly infects the colon, but can occur in the esophagus, stomach, or small bowel. CMV enteritis usually presents with abdominal pain and chronic diarrhea in an immunocompromised host. We present two atypical cases of CMV infection. Case 1: A 75 year-old white male with history of hypertension and atrial fibrillation developed an acute abdomen several days after extensive cardiothoracic surgery. He underwent an exploratory laparotomy. Adhesions were found and released, along with evidence of mid-jejunal ischemia where two small areas were oversewn. He later developed a gastrointestinal bleed. Repeated endoscopic examinations including EGDs, enteroscopies and colonoscopies were negative for a source of bleeding. He underwent a second operation and blood was found in the distal ileum. Arteriovenous malformations were suspected, but the resected specimen revealed ileitis with numerous ulcerations. The tissue showed viral inclusion bodies and stained positive for CMV antigen. Further serologies for HIV and other immune related diseases were negative. Case 2: A 53 year-old white female with history of hypertension, diabetes mellitus, status post cadaveric renal transplant was admitted with three days of nausea, vomiting and diarrhea. She was unable to keep down food or liquids, including her antirejection medications: prednisone, mycophenolate mofetil, and cyclosporine. She was admitted with acute renal failure. Her diarrhea persisted, but stool samples were negative for C. difficile, Salmonella, Shigella and Campylobacter. Colonoscopy showed friable mucosa from the rectum to descending colon. Random biopsies revealed inclusion bodies that stained positive for CMV antigen. Her serum CMV antigen was also positive. Both patients were treated successfully with three weeks of IV gancyclovir. CMV can cause disease of any portion of the gastrointestinal tract. It is usually seen in patients with T-lymphocyte dysfunction, and thus has become rare with improved HIV therapies. Diagnosis of CMV is made by mucosal biopsy, as viral cultures are not sensitive for active infection. Pathology is often subtle, but on close review can confirm the diagnosis. Clinicians need to have a high suspicion in all patients with immunosuppression, whether chronic or acute, for opportunistic infections that may involve the gastrointestinal tract.
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617 DEVELOPEMENT OF LICHEN PLANUS DURING PEGYLATED INTERFERON ALFA-2B AND RIBAVIRIN THERAPY FOR HEPATITIS C Jasdeep S. Bal, M.D., Helen Wong, M.D., Devang N. Prajapati, M.D.*. VA Central California Health Care System-University of California, San Francisco-Fresno, Fresno, CA. Combination therapy with pegylated interferon and ribavirin (PEG/RIBA) has become the standard treatment for hepatitis C (HCV). While this therapy has demonstrated clinical efficacy, side effects of these medications can lead to their discontinuation. Dermatological reactions to PEG/RIBA therapy for HCV are uncommon, but can be clinically significant and result in discontinuation of therapy. Lichen planus (LP) is an immune mediated, inflammatory skin lesion that manifests as recurrent pruritic, violaceous papules, that can arise in association with certain malignancies, infections and medications. Although LP has been well described in association with HCV, there have been few reports of its association with interferon and ribavirin therapy. We report a case of LP developing in a patient with HCV during treatment with PEG/RIBA. A 54 year-old man with HCV, with no prior history of dermatological abnormalities, was started on standard PEG/RIBA. The patient had no other significant co-morbidity and initially did well on therapy. Six weeks into therapy the patient developed worsening neutropenia and flu-like symptoms, leading to a reduction in the dose of pegylated interferon. At eight weeks of therapy, the patient noted a new onset diffuse eruption of shiny, flat, polygonal, violaceous papules across the upper back, chest, forehead, arms and legs. The patient was referred to the dermatology clinic, where a punch biopsy from a lesion over the right shoulder was performed. Histological examination of the biopsy specimen revealed a chronic perivasculitis in the dermis with features consistent with LP. PEG/RIBA therapy was discontinued as a result of the worsening skin eruption. Following cessation of PEG/RIBA the patient had a dramatic improvement in the LP with relief from pruritus and significant regression in the skin lesions. Although LP has been well described in association with HCV, its development after initiation PEG/RIBA has rarely been reported. We were unable to determine which component of PEG/RIBA may have been responsible for the eruption, however based on previous reports we speculate it may have been related to the interferon. Treating physicians should be aware of the potential for the development of LP as a side effect of PEG/RIBA. Although our patient discontinued HCV therapy, treatments directed at the LP may potentially allow patients to complete PEG/RIBA therapy.
618 MONOLOBAR CAROLI’S DISEASE: A CASE REPORT Sumir Patel, M.D., Abhijit Kulkarni, M.D.*, Rad Agrawal, M.D., Girija Nathan, M.D. Allegheny General Hospital, Pittsburgh, PA. Purpose: Describe the presentation of monolobar Caroli’s disease. A rare clinical entity. Case Presentation: A 54 year-old white male presented with fever, chills and right sided abdominal pain. The pain was intermittent, steady, and dull with no radiation. Physical exam revealed right upper quadrant abdominal pain with palpation. No hepato-splenomegaly or stigmata of chronic liver disease noted. Lab data: Wbc 18,000 with a normal differential. AST 144, ALT 90 and Alk Phos 540. Total bilirubin of 2.7, otherwise normal comprehensive metabolic panel. An abdominal CT showed dilated left intrahepatic bile ducts and an atrophic left lobe of the liver. MRI/MRCP showed focal left sided intrahepatic biliary dilation of unclear etiology. No evidence of a hepatic mass seen. No extra hepatic biliary dilation noted. ERCP showed a dilated (18mm) left intrahepatic bile duct. Multiple filling defects were noted in the dilated left intrahepatic duct. Multiple stone fragments were aspirated from the common bile duct. Brush cytology obtained for histology was negative for malignancy. The patient underwent an exploratory laparotomy with resection of hepatic segments II and III and a cholecystectomy. Grossly the sectioned
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surface of liver was nodular with cystically dilated bile ducts. Microscopically the findings were consistent with Caroli’s Disease. Discussion: Caroli’s disease is a rare congenital disorder characterized by segmental dilatation of intrahepatic bile ducts. The disease may be monolobar or bilobar. The monolobar type is extremely rare with less than fifty reported cases in the English literature. Here we report a case of monolobar Caroli’s disease. Caroli’s disease was first described by Caroli and coworkers in 1958. There are two forms of Caroli’s disease, simple and fibrous type. The simple (classic) type has congenital, segmental dilation of the intrahepatic biliary tree. In the fibrous type congenital hepatic fibrosis accompanies bile duct abnormalities. Common presentations include abdominal pain, recurrent cholangitis, choledocolithiasis and obstructive jaundice. Patients may also present with variceal bleeding from portal hypertension secondary to cirrhosis. Imaging studies including ultrasound, CT scan and MRI/MRCP can be used to demonstrate bile duct ectasia. ERCP is useful as a diagnostic and therapeutic modality. Treatment for bilobar Caroli’s disease is supportive and should be individualized. The treatment for monolobar Caroli’s disease is hepatic resection.
619 ISCHEMIC COLITIS: A RARE COMPLICATION OF HEPATITIS C THERAPY WITH PEGINTERFERON AND RIBAVIRIN M. Raza Anees, M.D.*, Mashood Qadri, M.D., Farah Anees, M.D., Vikas Khurana, M.D. Overton Brooks VAMC/ LSUH-Shreveport, Shreveport, LA; Overton Brooks VAMC, Shreveport, LA and LSUHShreveport, Shreveport, LA. Many of the adverse effects of the Interferon (INF) mono therapy for hepatitis C have been well documented. However there are very few reports (3–5) regarding its adverse effects on the colon. Although Ischemic colitis has been reported in the literature as a potential side effect of interferon mono therapy, our search revealed very few documented cases. Most of this literature is from Japan, none reported from the US. We present an interesting case of ischemic colitis as a potential complication of PEG-INF and Ribavirin therapy. Case: A 49 year-old male with a history of chronic hepatitis C, recently started on 150 mcg of Peginterferon alpha-2b weekly and Ribavirin 1200 mg a day, presented with abdominal pain and hematochezia. He had no history of nausea, vomiting, diarrhea, fever or exposure to infectious agent. He denies alcohol, drug abuse or any other medications. He does smoke a pack a day. On presentation he was hemodynamically stable. His examination revealed left upper quadrant tenderness and maroon stools. X-ray studies, CT scan of abdomen, EKG, and Doppler ultrasound of mesenteric vasculature were noncontributory. Drug screen was negative. A Gastroscopy performed was normal. Colonoscopy revealed ulcerated, hemorrhagic, friable mucosa starting from sigmoid extended to the splenic flexure, consistent with ischemic colitis. Biopsies later confirmed the diagnosis. Hepatitis C medications were held on admission and based on endoscopic findings; he was later taken off the therapy. Since he does not have any further episodes of hematochezia, he was later discharged from the hospital for outpatient follow up. Discussion: Our search has shown very few documented cases of ischemic colitis as a complication of Interferon mono therapy, all from Japan. Our case is unique since this happened in a U.S. patient who was receiving combination therapy with PEG-INF and ribavirin, rather than Interferon mono therapy. We would like to bring this complication to light so more attention could be paid to it in the future. There are reports in the literature that interferon therapy may cause severe vasculopathic/vasospastic complications that usually improve after its discontinuation. This might explain ischemic colitis in this patient. We will discuss about the possible mechanism of ischemic colitis in the setting of interferon treatment and will also present the biopsy slides.
AJG – Vol. 98, No. 9, Suppl., 2003
620 BLEEDING GASTRIC VARICES AND AN ENLARGING NECK MASS AS THE INITAL MANIFESTATIONS OF PANCREATIC CARCINOMA Ronen Arai, M.D.*. Coral Springs Medical Center, Coral Springs, FL. A 60 year-old male with a long history of smoking presented to the ER with complaints of dizzyness and black tarry stool. He was found to be anemic (HgB 6), and rectal exam demonstrated melena. He was admitted and transfused. EGD demonstrated coffee-grounds in the stomach and large gastric varices, without active bleeding. Abdominal CT scan (to assess for possible SMV thrombosis) showed pancreatitis with mild peripancreatic infiltrative changes. No evidence of a pancreatic mass was seen. The patient denied alcohol use and did not complain of abdominal pain or weight loss. Over the next 24 hours there was no further bleeding and he was discharged. Two weeks later he presented to his physician with complaint of a rapidly enlarging neck mass. On exam a left-sided 2cm hard submandibular mass was appreciated. He was immediately referred for ENT consultation and a biopsy was done. Pathologic exam demonstrated poorlydifferentiated adenocarcinoma. ENT search for a head/neck source of the primary tumor was unrewarding. Over the next several weeks, as plans for further oncologic work-up and treatment were being made, the patient began complaining of nausea, diffuse abdominal pain, bloating, anorexia, and weight loss. He was referred for GI consultation. On exam he appeared cachectic and had abdominal distension with shifting dullness. He was admitted to the hospital for further diagnostic testing. Repeat EGD demonstrated diffusely thickened gastric folds and moderate gastric varices. Repeat CT scan showed massive ascites with multiple pancreatic cysts and solid masses, liver masses, and abdominal lymphadenopathy. Chest CT showed several pulmonary metastases as well. Lab testing showed elevated blood levels of CEA (10) and CA 19-9 (450). A liver biopsy was performed and histologic examination demonstrated poorly-differentiated adenocarcinoma. Comparison of both biopsy specimens revealed that they represented the same neoplasm, most likely of pancreatic origin. This was supported by paracentesis findings of a CA 19-9 level of 14,700. A long discussion with the patient and his family ensued. He opted for comfort measures and was referred to hospice. This is a case of an rapidly progressive pancreatic adenocarcinoma presenting in an unusual fashion. It highlights the varied potential clinical manifestations of this aggressive malignancy. Unexplained pancreatitis complicated by gastric varices, along with a distant adenocarcinoma of unknown primary, should raise suspicion of an occult pancreatic malignancy.
621 COIL EMBOLIZATION OF LARGE SPONTANEOUS SPLENORENAL SHUNT FOR CHRONIC REFRACTORY HEPATIC ENCEPHALOPATHY Todd Frederick, M.D., Robert Lin, M.D., Steven Rose, M.D., Ayad Agha, M.D., Tarek I. Hassanein, M.D.*. UCSD Medical Center, San Diego, CA. Background: Hepatic encephalopathy (HE) is classified as acute or chronic. Chronic HE can be persistent or fluctuating. Persistent HE is difficult to treat, causing global disability, aspiration, malnutrition, and death. Despite the disabling nature of persistent HE, it does not enhance candidacy for liver transplantation. One cause of persistent HE is portosystemic shunting. Spontaneous splenorenal shunts can provide portosystemic bypass for large volumes of blood and percutaneous approaches to limit shunted blood flow have been attempted. Case: A 55 year-old male with cirrhosis due to hepatitis C was admitted for grade 3 HE. Common precipitating factors were excluded and he improved to grade 1 HE with oral lactulose and metronidazole. He was discharged home with lactulose to attain 2-3 soft stools/day. Unfortunately HE persisted from grades 1 to 3, requiring admission on 8 occasions over 18 months. An abdominal CT scan to screen for hepatocellular carcinoma revealed a large vascular structure connecting the left renal vein with the