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Antibody-mediated autoimmune diseases are common, can involve any organ system, and pose a large burden for patients and healthcare systems. Most antibody-mediated diseases are mediated by IgG antibodies. Selective targeting of pathogenic antibodies is an attractive treatment option which has already proven to be effective in antibody-positive generalized myasthenia gravis, maternal-fetal alloimmune cytopenias, and immune thrombocytopenic purpura. Warm autoimmune hemolytic anemia (wAIHA) is an autoimmune disorder mediated by pathogenic antibodies mainly of the IgG class with no approved therapy. Current treatment includes non-specific immunosuppression with corticosteroids, rituximab, and other immunosuppressive agents. With most therapies, time to response can be delayed and transfusions may be needed. Neonatal Fc receptor (FcRN) therapies provide rapid and sustained reduction of pathogenic IgG levels providing potential for fast, effective therapy in antibody-mediated autoimmune diseases including warm autoimmune hemolytic anemia. This review focuses on the emerging role of FcRn inhibition in autoimmune hematologic diseases, and their therapeutic potential in wAIHA.

PurposeThe peritumoral region (PTR) in glioblastoma (GBM) represents a combination of infiltrative tumor and vasogenic edema, which are indistinguishable on magnetic resonance imaging (MRI). We developed a radiomic signature by using imaging data from low grade glioma (LGG) (marker of tumor) and PTR of brain metastasis (BM) (marker of edema) and applied it on the GBM PTR to generate probabilistic maps.Methods270 features were extracted from T1-weighted, T2-weighted, and apparent diffusion coefficient maps in over 3.5 million voxels of LGG (36 segments) and BM (45 segments) scanned in a 1.5T MRI. A support vector machine classifier was used to develop the radiomics model from approximately 50% voxels (downsampled to 10%) and validated with the remaining. The model was applied to over 575,000 voxels of the PTR of 10 patients with GBM to generate a quantitative map using Platt scaling (infiltrative tumor vs. edema).ResultsThe radiomics model had an accuracy of 0.92 and 0.79 in the training and test set, respectively (LGG vs. BM). When extrapolated on the GBM PTR, 9 of 10 patients had a higher percentage of voxels with a tumor-like signature over radiological recurrence areas. In 7 of 10 patients, the areas under curves (AUC) were > 0.50 confirming a positive correlation. Including all the voxels from the GBM patients, the infiltration signature had an AUC of 0.61 to predict recurrence.ConclusionA radiomic signature can demarcate areas of microscopic tumors from edema in the PTR of GBM, which correlates with areas of future recurrence.Graphic abstract

Radiation recall dermatitis is an inflammatory reaction of the skin that may infrequently occur in areas of the skin that have been previously treated with radiation therapy. This is thought to be due to a triggering agent administered after radiation therapy which leads to an acute inflammatory reaction, manifesting as a skin rash. We present the case of a 58-year-old male with recurrent invasive squamous cell carcinoma of the tongue, previously treated with chemotherapy and radiation therapy, who presented with progression of his disease. He was treated with pembrolizumab and subsequently developed a new-onset facial rash over the previously treated radiation field. The distribution of the rash was suggestive of radiation recall dermatitis. A biopsy showed dermal necrosis without evidence of dermatitis, vasculitis, or infectious process. This case highlights the incidence of a rare complication of immune checkpoint inhibitor therapy and emphasizes the need for careful monitoring for radiation recall dermatitis.

Chronic lymphocytic leukemia (CLL) is the most common leukemia in adults and is characterized by monoclonal proliferation of B-cell lymphocytes which are morphologically mature, but immunologically dysfunctional. The primary sites of disease involvement include peripheral blood, lymph nodes, spleen, and bone marrow. CLL can also present locally and aggressively at extranodal sites. We describe the case of a 74-year-old gentleman with multiple medical comorbidities who was Foley catheter–dependent at baseline for bladder outlet obstruction. He was detected to have Rai stage I CLL following an inguinal lymph node biopsy and was on regular outpatient surveillance. Later, he underwent a prostate biopsy for evaluation of hematuria, results of which were consistent with CLL involvement in the prostate and urinary bladder. The patient was started on single-agent ibrutinib, and demonstrated an excellent clinical response to bladder outlet obstruction. His long-term Foley catheter was discontinued within 5 days of ibrutinib therapy. Unfortunately, 1 year later, he had disease progression, and therapy was changed to a single-agent rituximab, to which he is responding well. Our case is unique as it brings up the first reported case of prostate and bladder wall CLL.

Chronic pancreatitis presents with epigastric abdominal pain, nausea, vomiting, and weight loss. Acute pancreatitis can also present with a pleural effusion which is typically left-sided, mild in nature, and self-limiting. However, recurrent bouts of pancreatitis may lead to a pancreaticopleural fistula (PPF) with a large, rapidly recurring, unilateral pleural effusion. Among patients with PPF, the most common presenting complaint is dyspnea. We present the case of a 53-year-old man with recurrent bouts of pancreatitis in the setting of alcohol who presented with progressively worsening shortness of breath. A high-resolution computed topography scan of the thorax demonstrated a large right-sided pleural effusion. A thoracentesis was performed with pleural fluid studies showing an exudative effusion with amylase significantly elevated at 18 382 U/L. An endoscopic retrograde cholangiopancreatography was performed which showed a pancreatic duct leak in the tail of the pancreas. A pancreatic sphincterotomy was performed, and a stent was placed into the ventral pancreatic duct. The patient’s shortness of breath improved, and he was discharged home with outpatient follow-up. The aim of this report is to present the diagnosis of a rare complication of chronic pancreatitis and discuss the management and options for treatment.

Ventricular fibrillation can lead to sudden cardiac death. Automatic implantable cardioverter defibrillator (AICD) devices have shown to be highly successful in the termination of these arrhythmias and are a first-line modality of treatment for the prevention of sudden cardiac death. We present the case of a 69-year-old female with a history of paroxysmal atrial fibrillation on anticoagulation with apixaban and rate controlled with metoprolol who presented from home with a chief complaint of hiccups. She had a prior admission to the hospital after she was found to have monomorphic ventricular tachycardia during a nuclear stress test. A cardiac work-up including cardiac catheterization and cardiac magnetic resonance imaging did not show any evidence of significant coronary artery disease or reversible cardiomyopathy. The patient underwent successful placement of a single chamber ICD and was discharged home. Twelve weeks after placement of the AICD, the patient was lifting furniture and experienced sudden onset of hiccups. A chest X-ray showed displacement of the AICD lead from the right ventricular apex to the superior vena cava. The patient underwent lead repositioning with complete resolution of her hiccups. The etiology hiccups was suspected to be secondary to irritation of the right phrenic nerve which travels along the anterolateral border of the superior vena cava. We present the case of hiccups following ICD lead displacement. This serves to highlight a rare complication of ICD displacement that healthcare providers should consider when patients with recently placed ICD devices complain of hiccups.

Testicular teratomas may present in both prepubertal and adult men; however, the prognosis differs greatly between these 2 populations. In children, teratomas (prepubertal type) most often occur before the age of 4, are generally seen in their pure form, and behave in a benign fashion. In adults (postpubertal type), teratomas are usually part of a mixed germ cell tumor, and they have the potential to be found at metastatic sites, especially following chemotherapy for non-teratomatous germ cell tumor. Analyses of metastases from germ cell tumors and teratomas from the same patient have demonstrated a high degree of concordance in the observed genetic abnormalities. In rare cases, testicular teratoma can transform into a malignant germ cell tumor. One such type of transformation is into a primitive neuroectodermal tumor. These tumors are malignant and often metastasize to the retroperitoneum but may also metastasize to other sites. A multimodal treatment approach is needed, including surgery and adjuvant chemotherapy. We describe a rare case of malignant transformation of a testicular teratoma into a primitive neuroectodermal tumor with metastasis to the mediastinum. The patient was treated with radical orchiectomy, retroperitoneal lymph node dissection, and adjuvant vincristine, adriamycin, and cyclofosfamide alternating with ifosfamide and etoposide (VAC/IE therapy).

Penile squamous cell carcinoma is a rare diagnosis in the United States; however, the incidence is significantly higher in developing countries. This cancer is categorized into human papilloma virus (HPV)-associated and independent disease. In this article, we present a rare case of HPV-independent penile squamous cell carcinoma. Our patient was a 75-year-old Caucasian male who initially presented with a penile ulcer which was managed with partial penectomy and adjuvant chemotherapy. The patient was monitored on surveillance and did not pursue lymph node dissection. He was noted to have recurrence with metastatic disease 5 years after his initial presentation. Due to the aggressive nature of his disease, the patient was admitted to the hospital and treated with chemotherapy and immunotherapy.

Head and neck tumors account for roughly 3% of malignancies in the United States and about 90% of these tumors are squamous cell cancers. Neuroendocrine neoplasms arise from neural crest cells and are commonly found in the gastrointestinal tract. Neuroendocrine neoplasms arising from the head and neck tend to be rare. In this article, we present a rare case of human papilloma virus–associated poorly differentiated small cell neuroendocrine carcinoma (NEC). Our patient was a 62-year-old African American man who presented with worsening left-sided neck pain and swelling that started 3 months prior to presentation, associated with an unintentional 20-pound weight loss over 6 months, hoarseness in his voice, in addition to dysphagia and odynophagia. Biopsy of left-sided tongue mass revealed poorly differentiated small cell NEC that was positive for HPV (E6/E7) RNA in situ hybridization. Patient was found to have metastatic disease at the time of diagnosis and given the aggressive nature of small cell NECs and the patient’s symptomatic burden, chemotherapy with cisplatin and etoposide was initiated in the hospital. The patient was subsequently discharged from the hospital and is continuing treatment outpatient with cisplatin, etoposide, and atezolizumab.

Pulmonary hypertension (PH) is often a difficult condition to diagnose, since it occurs insidiously and is a diagnosis of exclusion. Patients with neurofibromatosis type 1 (NFT1) have been associated with severe exacerbations of PH. To our knowledge, less than 20 cases of PH in NFT1 patients have been reported. However, the severity of presenting symptoms requires physicians to be aware of this association and pursue the appropriate diagnostic workup. In our report, we present a 54-year-old NFT1 patient who presented with worsening dyspnea secondary to PH, which was being treated with trepostanil and macitetan. She required a right heart catheterization to assess her pulmonary artery pressures (which remained elevated). She was placed on tadalafil in addition to trepostanil and macitetan and noted significant resolution of her symptoms. Further studies are required to explore the association between PH and NFT1 and examine the efficacy of triple therapy with endothelin receptor antagonists, phosphodiesterase 5 inhibitors, and parenteral prostanoids in the initial treatment of PH in the aforementioned patient population.