Portal Vein Thrombosis in Cirrhosis.

Portal vein thrombosis (PVT) in the cirrhotic population is a challenging clinical phenomenon requiring a nuanced management approach. Uncertainty exists regarding the clinical significance of PVT in the cirrhotic population because the data for PVT are based mainly on retrospective, small cohort studies. Therefore, strong recommendations regarding the optimal timing, modality, and duration of therapy for PVT in the cirrhotic population cannot be made. However, this review aims to summarize the current literature and provide stepwise guidance in diagnosing and managing PVT in patients with cirrhosis.

Bleeding 'downhill' esophageal varices associated with benign superior vena cava obstruction: case report and literature review.

BACKGROUND: Proximal or 'downhill' esophageal varices are a rare cause of upper gastrointestinal hemorrhage. Unlike the much more common distal esophageal varices, which are most commonly a result of portal hypertension, downhill esophageal varices result from vascular obstruction of the superior vena cava (SVC). While SVC obstruction is most commonly secondary to malignant causes, our review of the literature suggests that benign causes of SVC obstruction are the most common cause actual bleeding from downhill varices. Given the alternative pathophysiology of downhill varices, they require a unique approach to management. Variceal band ligation may be used to temporize acute variceal bleeding, and should be applied on the proximal end of the varix. Relief of the underlying SVC obstruction is the cornerstone of definitive treatment of downhill varices. CASE PRESENTATION: A young woman with a benign superior vena cava stenosis due to a tunneled internal jugular vein dialysis catheter presented with hematemesis and melena. Urgent upper endoscopy revealed multiple 'downhill' esophageal varices with stigmata of recent hemorrhage. As there was no active bleeding, no endoscopic intervention was performed. CT angiography demonstrated stenosis of the SVC surrounding the distal tip of her indwelling hemodialysis catheter. The patient underwent balloon angioplasty of the stenotic SVC segment with resolution of her bleeding and clinical stabilization. CONCLUSION: Downhill esophageal varices are a distinct entity from the more common distal esophageal varices. Endoscopic therapies have a role in temporizing active variceal bleeding, but relief of the underlying SVC obstruction is the cornerstone of treatment and should be pursued as rapidly as possible. It is unknown why benign, as opposed to malignant, causes of SVC obstruction result in bleeding from downhill varices at such a high rate, despite being a less common etiology of SVC obstruction.

Endoscopic or Conservative Management of Iatrogenic Duodenal Perforations Caused by Long Plastic Biliary Stent Distal Migration.

Plastic biliary stents are associated with rare but potentially life-threatening distal stent migration. We present 4 patient cases with distal migration, whereas the proximal aspect remained in the bile duct. Time to stent migration ranged from 1 week to 2 months. Stent migration caused contralateral duodenal wall perforation; 2 underwent endoscopic over-the-scope clip placement for defect closure. All required previous stent removal and stent exchange. This case series highlights that proximal stricture and longer stents have higher migration risk, also shown in the literature. We also show that duodenal perforation can successfully be managed endoscopically with an over-the-scope clip.

Coccidioides immitis Cervical Lymphadenitis Complicated by Esophageal Fistula.

Coccidioidomycosis (valley fever) is caused by the dimorphic fungi Coccidioides immitis or Coccidioides posadasii. Most infections are asymptomatic or result in self-limited pneumonia; extrapulmonary dissemination via either hematogenous or lymphatic spread is rare. Here, we present a case of cervical C. immitis lymphadenitis that resulted in fistula formation to the esophagus via mediastinal extension. This case highlights a very unusual extrapulmonary manifestation of coccidioidomycosis, the difficulty in diagnosing coccidioidal infection when it is not suspected, and the importance of obtaining a thorough exposure history to assist with diagnosis.

Rearrangement of CRLF2 in B-progenitor- and Down syndrome-associated acute lymphoblastic leukemia.

Aneuploidy and translocations are hallmarks of B-progenitor acute lymphoblastic leukemia (ALL), but many individuals with this cancer lack recurring chromosomal alterations. Here we report a recurring interstitial deletion of the pseudoautosomal region 1 of chromosomes X and Y in B-progenitor ALL that juxtaposes the first, noncoding exon of P2RY8 with the coding region of CRLF2. We identified the P2RY8-CRLF2 fusion in 7% of individuals with B-progenitor ALL and 53% of individuals with ALL associated with Down syndrome. CRLF2 alteration was associated with activating JAK mutations, and expression of human P2RY8-CRLF2 together with mutated mouse Jak2 resulted in constitutive Jak-Stat activation and cytokine-independent growth of Ba/F3 cells overexpressing interleukin-7 receptor alpha. Our findings indicate that these two genetic lesions together contribute to leukemogenesis in B-progenitor ALL.