A Rare Case of PL-7-Associated Immune-Mediated Necrotizing Myopathy With Isolated Dysphagia as the Presenting Symptom.

Immune-mediated necrotizing myopathy (IMNM) is a rare, progressive disease that accounts for about 19% of all inflammatory myopathies. Dysphagia occurs in about 20%-30% of IMNM patients. This case results in the third presumptive instance of IMNMwith dysphagia as the initial symptom. Given that isolated dysphagia in IMNM is atypical to the conventional symptoms in the late stage of the disease, it is critical for clinicians to have a high degree of suspicion for IMNM due to the aggressive nature of the disease and its refractoriness to treatment. Additionally, this case also highlights an atypical autoantibody, PL-7, being positive in an IMNM patient who presents with dysphagia as an initial symptom.

Endotipsitis in an Immunocompetent Patient With Lactobacillus Bacteremia.

Transjugular intrahepatic portosystemic shunt (TIPS) is a procedure commonly performed to decompress portal venous pressure since the early 1990s. Endotipsitis, which refers to persistent bacteremia caused by endovascular infection of the TIPS stent, is a rare but serious complication of this procedure. Very few cases of endotipsitis have been reported worldwide. We report the case of an immunocompetent patient diagnosed with endotipsitis, an atypical risk factor for Lactobacillus infection. This case report adds to the literature on underreported complications of TIPS, highlighting an urgent need for introducing clinical practice guidelines regarding the definition, diagnosis, and treatment of endotipsitis.

A novel Iowa-Mayo validated composite risk assessment tool for allogeneic stem cell transplantation survival outcome prediction.

Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative option for many hematologic conditions and is associated with considerable morbidity and mortality. Therefore, prognostic tools are essential to navigate the complex patient, disease, donor, and transplant characteristics that differentially influence outcomes. We developed a novel, comprehensive composite prognostic tool. Using a lasso-penalized Cox regression model (n = 273), performance status, HCT-CI, refined disease-risk index (rDRI), donor and recipient CMV status, and donor age were identified as predictors of disease-free survival (DFS). The results for overall survival (OS) were similar except for recipient CMV status not being included in the model. Models were validated in an external dataset (n = 378) and resulted in a c-statistic of 0.61 and 0.62 for DFS and OS, respectively. Importantly, this tool incorporates donor age as a variable, which has an important role in HSCT outcomes. This needs to be further studied in prospective models. An easy-to-use and a web-based nomogram can be accessed here: https://allohsctsurvivalcalc.iowa.uiowa.edu/ .

Repeat Endoscopy Affects Patient Management in Gastrointestinal Graft-Versus-Host Disease.

Graft versus host disease (GVHD) of the gut is associated with significant morbidity and mortality after allogeneic hematopoietic cell transplant (allo-HCT). No guidelines exist regarding repeat endoscopy after failure of first-line treatment with steroids. We aimed to study if repeat endoscopic biopsy can be helpful in these patients to guide treatment decisions. We retrospectively reviewed medical records of all patients who underwent repeat endoscopy for clinical suspicion of gastrointestinal (GI) GVHD after allo-HCT. Of the 318 patients, 24 underwent endoscopy twice after allo-HCT. At first endoscopy, 20 patients (80%) showed abnormal findings: 16 with GVHD alone, 1 with GVHD plus cytomegalovirus (CMV), and 3 with GVHD plus infectious colitis. On repeat endoscopy in these 20 patients with GVHD, 6 showed improvement leading to de-escalation of therapy, 8 showed worsening of GVHD including detection of CMV in 2 patients, and 2 had no histological changes. One patient with simultaneous GVHD and CMV diagnosed on first biopsy, displayed significant improvement leading to de-escalation of therapy. Three patients with GVHD along with infectious colitis on biopsy subsequently showed improvement on repeat biopsy leading to de-escalation of therapy. Among 4 patients with normal findings on first endoscopy, 3 had GVHD and 1 had epstein-barrvirus-associated post-transplant lymphoproliferative disorder (EBV-PTLD) on repeat procedures. This study supports the usefulness of repeat endoscopy in persistently symptomatic patients when there is no improvement after the initial treatment based on the results of the first endoscopy. Repeat endoscopy may guide therapy without significant complications.