Red cell exchange for rapid leukoreduction in adults with hyperleukocytosis and leukostasis.

ABSTRACT: We show that red cell exchange (RCE) treats hyperleukocytosis in acute leukemia. RCE provided similar leukoreduction to standard therapeutic leukoreduction and could be superior in patients with severe anemia or monocytic leukemias or when requiring rapid treatment.

Impact of trainee experience on shave biopsy specimen size.

BACKGROUND: Skin biopsies are crucial for the diagnosis of many cutaneous pathologies, yet specimen adequacy is essential for definitive diagnosis. Recent literature has noted a trend in decreasing biopsy size over time, which has created concern over implications regarding adequacy for diagnosis. METHODS: This study sought to evaluate if clinician training length or type of residency training impacted the average biopsy size and sample adequacy. Dermatopathology reports for shave biopsies between January 1, 2021, and June 30, 2021, at Penn State Health were queried through PathNet, the software application for pathology reports in this health system's electronic medical record system. Biopsy dimensions, volume, diagnosis, location, clinician training level, and descriptions of evaluation of deeper sections (recuts) and superficial sampling were recorded for each biopsy. Basic statistical calculations were performed to assess the mean and standard deviation for biopsy sizes per clinician group. RESULTS: Differences in biopsy size between training levels were statistically significant despite qualitatively similar biopsy locations and final diagnosis categories for each clinician training group. After evaluating measures for sample adequacy, our data showed significantly smaller biopsies; however, overall frequencies were minimal. Additionally, more inadequate specimens were noted for clinician groups with the least amount of dermatology experience. CONCLUSIONS: The results of this study identify a correlation with decreasing biopsy size amidst increased experience in dermatology training but find no evidence to support that this trend currently threatens sample adequacy.

Novel fusion sarcomas including targetable NTRK and ALK.

BACKGROUND: Challenging emerging entities with distinctive molecular signatures may benefit from algorithms for diagnostic work-up. METHODS: Fusion sarcomas (2020-2021, during pandemic) were diagnosed by clinicoradiology, morphology, phenotype, and next-generation sequencing (NGS). RESULTS: Six fusion sarcomas in two males and four females involved the chest-wall, neck, or extremities; ages ranged 2-73, median 18 years. Sizes ranged 5.3-25.0, median 9.1 cm. These include high grade 1) TPR-NTRK1 of proximal femur with a larger rounded soft tissue mass, previously considered osteosarcoma yet without convincing tumor matrix. A pathologic fracture necessitated emergency hemipelvectomy (NED) and 2) novel KANK1-NTRK2 sarcoma of bone and soft tissue with spindled pleomorphic to epithelioid features (AWD metastases). 3) Novel ERC1-ALK unaligned fusion, a low grade infiltrative deep soft tissue hand sarcoma with prominent-vascularity, myopericytoid/lipofibromatosis-like ovoid cells, and collagenized stroma, was successfully treated with ALK-inhibitor (Crizotinib), avoiding amputation. These NTRK and ALK tumors variably express S100 and CD34 and were negative for SOX10. 4) and 5) CIC-DUX4 round cell tumors (rapid metastases/demise), one with COVID superinfection, were previously treated as Ewing sarcoma. These demonstrated mild pleomorphism and necrosis, variable myxoid change and CD99 reactivity, and a distinctive dot-like-Golgi WT1 immunostaining pattern. 6) A chest wall/thoracic round cell sarcoma, focal CD34/ keratins/CK7, revealed nuclear-STAT6, STAT6-NAB2 by NGS, confirming malignant solitary fibrous tumor, intermediate-risk-stratification (AWD metastases). CONCLUSIONS: Recent fusion sarcomas include new KANK1-NTRK2 and ERC1-ALK, the latter successfully treated by targeted-therapy. ALK/NTRK fusion partners TPR and KANK1 suggest unusual high-grade morphology/behavior. Clinicoradiologic, morphologic, and phenotypic algorithms can prompt molecular-targeted immunostains or NGS for final classification and promising inhibitor therapy.