Clinical Features and Outcomes of Patients with Pancreaticobiliary Malignancies in Los Angeles County and Their Association with CA 19-9 Levels.

Although CA 19-9 is a commonly used tumor marker in the management of PBMs, the literature describing outcomes in patients with PBMs who have undetectable or low (hereinafter "low") CA 19-9 levels remains scarce. In this study, we sought to compare clinical features and outcomes in patients with PBMs and low CA 19-9 levels to those with normal and elevated CA 19-9 levels. METHODS: We retrospectively collected data on patients with biopsy-confirmed PBMs and stratified patients into categories based on their CA 19-9 level at diagnosis. Survival curves were estimated for patients in each of the three aforementioned CA 19-9 groups using the Kaplan-Meier method and compared using a Cox proportional hazards regression model. RESULTS: Of the 283 patients identified, 23 (8.1%) had low, 70 (24.7%) had normal, and 190 (67.1%) had elevated CA 19-9 levels. After controlling for sex, age, BMI, the presence of metastases at the time of diagnosis, and treatment with curative intent, the hazard ratio for death in the elevated CA 19-9 group compared to the low CA 19-9 group was 1.993 (95% CI 1.089-3.648; p = 0.025). CONCLUSION: The elevated CA 19-9 level compared to the low CA 19-9 level and the presence of metastases were associated with an increased hazard of death, while treatment with curative intent was associated with a decreased hazard of death.

Immunotherapy for Acute Myeloid Leukemia: Allogeneic hematopoietic cell transplantation is here to stay.

Acute Myeloid Leukemia (AML) represents 1 % of all new cancer diagnosis made annually in the US and has a five-year survival of 30 %. Traditional treatment includes aggressive induction therapy followed by consolidation therapy that may include a hematopoietic stem cell transplant (HSCT). Thus far, HSCT remains the only potentially curative therapy for many patients with AML owing to the graft-versus-leukemia effect elicited by this treatment. The use of novel therapies, specifically immunotherapy, in the treatment of AML has been limited by the lack of appropriate target antigens, therapy associated toxicities and variable success with treatment. Antigenic variability on leukemia cells and the sharing of antigens by malignant and non-malignant cells makes the identification of appropriate antigens problematic. While studies with immunotherapeutic agents are underway, prior investigations have demonstrated a mixed response with some studies prematurely discontinued due to associated toxicities. This review presents a discussion of the envisioned role of immunotherapy in the treatment of AML in the setting of mixed therapeutic success and potentially lethal toxicities.

Beta-Propeller Protein-Associated Neurodegeneration (BPAN) Detected in a Child with Epileptic Spasms.

This report discusses a 13-year-old girl diagnosed with beta-propeller protein-associated neurodegeneration (BPAN). BPAN is an X-linked neurodegeneration disorder associated with a mutation in the WDR45 gene. It typically presents in childhood with encephalopathy, developmental delay, and seizures. Following an initial static phase, these symptoms then progress to dementia, dystonia, and parkinsonism in early adulthood. Our child initially presented with epileptic spasms, global developmental delay, speech delay, hypotonia, spasticity, scoliosis, and gait disturbance. While these symptoms remained unchanged in early childhood, they depicted accelerated deterioration at age 12-13 rather than in adulthood. Her diagnosis was made based on her clinical presentation and review of imaging that led to specific genetic testing confirming the condition. The imaging findings were of markedly low signal on gradient T2* sequences in the globus pallidus and substantia nigra and T1 hyperintensity in the substantia nigra, with associated diffuse brain volume loss. Unlike other cases reported in the literature, there was no classic area of central hypointensity on T1 imaging in the substantia nigra.

Becoming the Standard: Expanding the Conventional Use of Interventional Embolization for Ruptured Pulmonary Artery Aneurysms.

Behcet's disease is inflammatory vasculitis that has a high incidence of mortality in patients with pulmonary artery aneurysm (PAA) formation. Traditionally, patients with Behcet's disease and PAA rupture undergo invasive surgical management. Surgical intervention; however, has been shown to have high complication, failure, and mortality rates. It has become a more contemporary practice to utilize the interventional embolization of pulmonary artery aneurysms (PAAs) in patients with Behcet's disease and other various etiologies because of its inherent minimally invasive nature and decreased risk for complications. The management paradigm for treating PAAs has shifted toward endovascular embolization even in severe or emergent cases where surgical management was once thought to be the standard. The following case is a testimony to the practicality of interventional embolization in the setting of a symptomatic patient presenting with PAAs.