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Background: Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder that has not been well documented in children, particularly those with acquired bone marrow failure disorders (ABMFD) – acquired aplastic anemia (AAA) and myelodysplastic syndrome (MDS). Therefore, we sought to determine the prevalence of PNH populations in children with ABMFD. Methods: PNH testing was performed in children with an ABMFD diagnosis using high sensitivity (≥0.01%) fluorescent aerolysin (FLAER)-based assay according to 2010 International Clinical Cytometry Society (ICCS) PNH Consensus Guidelines and 2012 Practical PNH Guidelines. FLAER/CD64/CD15/CD24/CD14/CD45 and CD235a/CD59 panels were used for white blood cell and red blood cell testing, respectively. Results: Thirty seven patients with ABMFD (34 AAA, 3 MDS) were included (17 M/20 F, age 2 to 18 years, median 9 years). PNH populations were identified in 17 of 37 (46%) patients. Of the 17 patients with PNH populations identified, 7 were PNH clones (> 1% PNH population) and 10 had minor PNH population or rare cells with PNH phenotype (≤ 1% PNH population). Conclusions: This is the first study to use a standardized high-sensitivity FLAER-based flow cytometry assay and the recommended cutoff of 0.01% to identify cells with PNH phenotype in pediatric patients with ABMFD in the United States. The identification of a PNH population in 46% of ABMFD supports the recommendation for high sensitivity PNH testing in children with these disorders, as a routine assay. Using a cutoff of ≥ 1% PNH population would have missed 10 (27%) patients with minor PNH population or rare cells with PNH phenotype. This article is protected by copyright. All rights reserved.

Small-cell carcinoma of the ovary, hypercalcemic type (SCCOHT) is a rare but highly undifferentiated, aggressive malignancy that primarily affects young women. Due to its early onset, unclear familial history and vague presenting symptoms, most SCCOHT patients present late with advanced disease. The prognosis is extremely poor, with

Introduction: Our study attempts to characterize mesothelin and GPR30 / estrogen receptor (ER) staining in pancreatic pathology. Materials and Methods: Immunohistochemical staining for mesothelin, GPR30, and ER was performed on a variety of pancreatic lesions. Results: 24 of 42 (57%) adenocarcinomas stained for mesothelin, while 0 of 16 non-carcinomas (0%) stained (p = 0.0000784). 35 of 39 (90%) adenocarcinomas stained for GPR30, while only 4 of 15 (27%) non-carcinomas stained (p = 0.0000036). Apart from stromal staining in one case of mucinous cystic neoplasm, no cases stained for ER. 27 of 37 (73%) adenocarcinoma fine needle aspirates were positive for mesothelin. Discussion: GPR30 is more sensitive, but less specific than mesothelin for pancreatic adenocarcinoma. Mesothelin is detected in most adenocarcinoma fine needle aspirates. ER is rarely detected in pancreatic lesions.

Background Flow cytometry has been widely used for the diagnosis of various hematopoietic diseases. Although there have been advances in the number of biomarkers that can be analyzed simultaneously and technologies that enable fast performance, the diagnostic data are still interpreted by a manual gating strategy. The process is labor-intensive, time-consuming, and subject to human error. Results We used 80 sets of flow cytometry data from 44 healthy donors, 21 patients with chronic lymphocytic leukemia (CLL), and 15 patients with follicular lymphoma (FL). Approximately 15% of data from each group were used to build the profiles. Our approach was able to successfully identify 36/37 healthy donor cases, 18/18 CLL cases, and 12/13 FL cases. Conclusions This proof-of-concept study demonstrated that an automated diagnosis of CLL and FL can be obtained by examining the cell capture rates of a test case using the computational method based on the multi-profile detection algorithm. The testing phase of our system is efficient and can facilitate diagnosis of B-lymphocyte neoplasms.