INTRODUCTION: Hypereosinophilic Syndrome (HES) is a rare disorder most commonly diagnosed in patients 20-50 years of age. It is defined as a persistent absolute eosinophil count greater than 1500 cells/microL and/or pathologic confirmation of tissue hypereosinophilia, as well as evidence of end-organ dysfunction. Prior to making a diagnosis, allergic, rheumatologic, infectious, and neoplastic etiologies should be excluded. CASE DESCRIPTION/METHODS: This is the case of an 87 -year-old male with past medical history of malaria treated with chloroquine who presented with altered mental status, dry cough and ascites de novo. Associated symptoms included unintentional weight loss of 10-15 lbs and intermittent episodes of non- bloody, watery diarrhea. He denied history of alcohol or drug use. Physical exam was remarkable for a distended abdomen with a positive fluid wave and pitting edema +1 in the lower extremities bilaterally. Labs showed a platelet count of 53,000, hypoalbuminemia (1.9 g/dl), increasing creatinine (2.1 mg/dl), alkaline phosphatase of 444 u/l, total bilirubin of 2.8 mg/dl with indirect predominance, AST of 59 u/l, ALT of 55 u/l, and eosinophilia of 72% (absolute eosinophil count of 6,048). Abdominal sonogram with doppler failed to show obstructive pathology and no evidence of hepatic vein thrombosis. Paracentesis showed absence of malignant cells, SAAG >1.1, 0.79 g/dl of protein and abundant eosinophils. Hepatitis, Iron Saturation levels, AMA, ASMA, Schistosoma and Strongyloides workup were negative. Hematology oncology service was consulted for bone marrow biopsy to asses for myeloid and lymphoid disorders. Results showed a hypercellular bone marrow with marked eosinophilia and 50% nucleated cells but C-KIT, PGFR Alpha, PGFRB, BCR/ABL, JAK 2 and TCR were all negative. A diagnosis of Hypereosinophilic Syndrome with suspected eosinophilic liver infiltration was made and the patient was started on prednisone therapy. All prior described multisystemic symptoms resolved. Eosinophilia, thrombocytopenia and all liver enzymes normalized within 24-48 hours after initiation of therapy. Ascites also resolved for which diuretic therapy was discontinued. DISCUSSION: This case is interesting as HES has an incidence of 0.36 per 100,000, and hepatic involvement accounts solely for an estimated 30% of patients. This serves to illustrate that HES should form part of the differential diagnosis when hepatic involvement and eosinophilia are concerned as prompt evaluation and treatment can lead to rapid resolution.
INTRODUCTION: Neuroendocrine tumors (NETs) of the ampulla of Vater represent less than 0.3% of gastrointestinal NETs and less than 2% of all periampullary malignancies. There are approximately 139 cases in literature, making this rare condition a diagnostic challenge. Jaundice is the predominant symptom followed by abdominal pain. Acute pancreatitis as the initial presentation has seldom been reported. Herein we report a case of a patient who presented with acute pancreatitis and biliary obstruction secondary to an ampullary NET. CASE DESCRIPTION/METHODS: Case of a 74-year-old male with hypertension who reported burning epigastric pain. Denied fever, vomits, weight loss, diarrhea, flushing or dyspnea. Physical examination with epigastric tenderness. Laboratories showed elevated alkaline phosphatase 398 U/L, total bilirubin 3.4 mg/L, lipase 594 U/L and amylase 216 U/L. Contrast abdominopelvic CT showed a duodenal periampullary mass with common bile duct measuring 1.1 cm and intrahepatic and extrahepatic dilatation. Endoscopic retrograde cholangiopancreatography (ERCP) revealed an irregular, ulcerated ampullary mass. Biopsy showed poorly differentiated carcinoma. Immunostains were positive for chromogranin, synaptophysin, CK7, weakly positive for CDX-2. Negative for TTF-1 and CK20. Octreotide scintigraphy with no uptake. Somatostatin, chromogranin, and 5-hydroxy-in-dole acetic acid were normal. Patient underwent surgical treatment with pancreaticoduodenectomy. DISCUSSION: The rarity of ampullary NETs and its proliferation under an intact mucosa makes it difficult to detect in biopsy. Most of the time the diagnosis is based on histology and preoperative diagnosis may be challenging since they clinically present like adenocarcinoma. The usual presentation of jaundice and abdominal pain places this diagnosis low on the differential. In our case, the unusual morphologic appearance likely facilitated obtaining an assertive pre-operative diagnosis. Our patient presented with obstructive jaundice and acute pancreatitis, which occurs in less than 6% of cases. The clinical picture of ampullary NETs is easily confounded given the associated symptoms and biopsy challenges. A high index of suspicion is paramount to ensure this rare diagnosed is not missed. Pancreaticoduodenectomy has been advocated as the treatment of choice regardless of size due to high risk of occult nodal metastases. Considering this diagnosis within the differential is important given the drastically different outcomes for patients if caught early.
INTRODUCTION: TransarteriaL chemoembolization (TACE) is a widely-used treatment for unresectable hepatocellular cancer (HCC). Although TACE is minimally invasive, major complications occur in 5% of cases. These include acute cholecystitis, which is self-limiting and rarely requires surgical intervention. Liver abscess is a rare complication but can increase length-of-stay and morbidity. Our case focuses on multiple uncommon complications of TACE occurring in conjunction; gangrenous cholecystitis requiring emergent cholecystectomy, liver abscess and portal vein thrombosis. CASE DESCRIPTION/METHODS: A 67-year-old male with history of hepatitis C, cirrhosis and HCC status-post recent successful TACE who presented to the ER with right upper quadrant pain associated with fever. Physical examination with right upper quadrant tenderness and positive murphy’s sign. Laboratories showed leukocytosis and elevated alkaline phosphatase. Normal bilirubin, liver and pancreatic enzymes. Abdominopelvic CT with hydropic gallbladder with enhancing walls and pericholecystic inflammatory changes, new ill-defined hypodensities suggestive of abscess and extensive thrombus in the right portal vein. The patient received antibiotics and underwent an uneventful cholecystectomy. Post-operation findings consistent with a gangrenous gallbladder with severe adherent hepatic flexure and omentum to the gallbladder fundus. The patient was discharged on antibiotics and anticoagulation. Follow up MRI showed resolution of fluid filled lesions with almost complete resolution of the portal vein thrombosis. DISCUSSION: Cholecystitis is a complication of TACE due to reflux of the embolic material into the cystic artery. In most cases, management can be conservative. Our case is rare given that the patient developed gangrenous cholecystitis which is a serious complication that requires immediate surgical intervention. Liver abscess occurs infrequently unless there is a history of prior biliary reconstruction unlike our patient. Management includes antibiotics and percutaneous drainage for larger abscesses. In our case, our patient responded well to antibiotics. Because symptoms are non-specific, these complications may be mistaken for post embolization syndrome, which is usually self-limiting and abates by one week. Normal post-TACE CT findings can also confound the diagnosis of hepatic abscess formation. This case highlights the importance of being aware of complications of TACE as failure to recognize these may confer a higher mortality risk.
INTRODUCTION: 48-year-old obese (BMI: 40) female, G0P0A0 LMP 1 year ago, with hypertension and hypercholesterolemia who presented with sudden left lower abdominal cramping pain associated with bloody diarrhea of one day duration. Denied associated systemic symptoms. She was using aspirin, simvastatin, metoprolol and had a progestin-only intrauterine device (IUD) placed 2 years ago. Physical examination with tenderness at the left lower quadrant. Digital rectal examination unremarkable. Laboratories with leukocytosis, lactatemia and positive fecal leukocytes. Abdominopelvic CT with IV contrast revealed left-sided peri-colonic and colonic inflammatory changes. CASE DESCRIPTION/METHODS: Gastroenterology service consulted due to non-specific colitis and there was concern for ischemic colitis as CT revealed inflammation in a watershed area. Colonoscopy showed edematous mucosa from the splenic flexure to the sigmoid colon associated with multiple large longitudinal ulcers. Biopsies revealed mucosal hemorrhage and neutrophil infiltration, suggestive of ischemic injury. Etiology of ischemia not clear given no obvious risk factors. Cardiovascular, myeloproliferative and prothrombotic workups were negative. The last piece of the puzzle rested on the IUD which was removed by the OBGYN service. A few days later, a follow up CT showed marked inflammation improvement. DISCUSSION: Ischemic colitis is generally considered a disease of the elderly. The most common causes include low-flow states due to cardiac dysfunction and hypovolemia. However, in younger patients, hypercoagulable states and drugs are the most common etiologies. In our patient, it was hard to initially find a reasonable cause of ischemia but, after excluding all etiologies, patient's IUD was the most likely cause of bowel ischemia. Combination contraceptives are a well-known risk factor for venous thromboembolism (VTE). However, few data have been published regarding whether the risk of VTE increases with progestin-only contraceptives (1). Mechanism is unclear, although it is hypothesized that protein concentrations influenced by hormones affect coagulation homeostasis (2). This risk appears to increase if additional VTE risk factors are present such in our patient with an elevated BMI (3). This rare case proves the importance of acquiring a detailed medical history including the use of contraceptives. Moreover, awareness of this diagnosis is essential so that early recognition can lead to individualized treatment decision and better patient's outcome.
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