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Objectives: Primary central nervous system lymphomas (PCNSLs) in patients with human immunodeficiency virus (HIV) are predominantly B-cell lymphomas associated with Epstein-Barr virus (EBV) and rarely CD8-positive T-cell PCNSLs.Methods: Patient history, laboratory results, cerebrospinal fluid (CSF), imaging, and brain biopsy specimens were reviewed and tested for T-cell receptor clonality.Results: A 64-year-old HIV-positive woman sought treatment for lethargy and left-sided weakness. Brain imaging showed regional increased T2 signal with restricted diffusion in cerebral hemispheres. CSF flow cytometry revealed CD4-positive T lymphocytes with loss of CD3, CD5, and CD7. EBV-positive T-cell lymphoma was immunohistochemically confirmed on brain biopsy specimens. Molecular analysis detected clonal T-cell receptor gene rearrangement. The patient received intrathecal methotrexate and whole-brain radiation. She did not respond to treatment and was eventually placed in hospice care.Conclusions: To our knowledge, this is the first report of CD4-positive T-cell PCNSL in an HIV-positive patient and will help to raise clinical awareness of this previously unknown entity. [ABSTRACT FROM AUTHOR]

Objectives: To explore the implications of cervical conization specimens lacking the targeted high-grade squamous intraepithelial lesions (negative cone).Methods: We studied 540 conization procedures: 400 positive cones and 140 negative cones. Clinicopathologic features and 2-year follow-up results were reported.Results: Negative cones comprised 22% of procedures triggered by CIN2 or higher biopsies. Procedures triggered by cytology produced much higher percentages of negative cones (37% high-grade squamous intraepithelial lesion [HSIL], 46% atypical squamous cells-cannot exclude high-grade squamous intraepithelial lesion [ASC-H], and 76% low-grade squamous intraepithelial lesion-cannot exclude high-grade squamous intraepithelial lesion [LSIL-H]). Upon reviewing negative excision-triggering biopsy and cytology, we downgraded 24 (24%) CIN2 biopsies, three (14%) HSIL, five (83%) ASC-H, and 12 (92%) LSIL-H. One-third of our negative cones can be attributed to overdiagnosis either on biopsy or cytology. Patients with negative cones were older and had smaller excisions, negative colposcopic findings, and negative/equivocal high-risk human papillomavirus (HR-HPV). Within 2 years, 35 (25%) women with negative cones experienced ASCUS or LSIL. Only one (0.7%) recurred as CIN3, a significantly lower percentage than women with positive cones (13%).Conclusions: We advocate careful review of all excision-triggering biopsy and cytology, especially in cases of LSIL-H. Patients with negative cones should be surveyed with cytology and HR-HPV testing. [ABSTRACT FROM AUTHOR]

Background: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a glomerular disease defined by non-organized glomerular deposits of heavy and light chain–restricted immunoglobulin and is rarely reported in children. Methods: We characterized a series of nine pediatric patients from two academic centers with biopsy-proven PGNMID and additionally describe two patients with monotypic IgG in the setting of IgM deposition. Results: Each patient presented with hematuria and/or proteinuria; however, only five had elevated serum creatinine. Prodromal or concurrent infection was identified in six patients, low C3 in five, and alternate complement pathway gene variants in two. No monoclonal serum proteins were identified in five tested patients. Seven patients had monotypic deposits composed of IgG3-λ, two showed IgG3-κ, and one each IgG1 and IgG3 with lambda dominance in the setting of IgM deposition. The glomerular pattern was predominantly mesangial proliferative or membranoproliferative glomerulonephritis (MPGN). Treatment and outcomes were variable; four patients have recent PGNMID diagnoses and therefore minimal follow up, one had relatively stable kidney function for over a decade, and six experienced kidney failure, with four receiving transplants. Recurrent deposits of the same isotype were identified in five of six transplanted kidneys, corresponding to three of four transplanted patients. One of these patients developed PGNMID recurrences in three separate kidney allografts over a 20-year disease course. Conclusions: Our study emphasizes the need for upfront IgG subclass investigation in pediatric mesangial or MPGN with IgG deposition and monotypic or biased light-chain staining. Furthermore, this pediatric experience suggests expanded pathogenic considerations in PGNMID. [ABSTRACT FROM AUTHOR]

Leukocyte chemotactic factor 2 amyloidosis (ALECT2) is a recently described form of systemic amyloidosis, which most commonly affects the kidney and liver. The LECT2 protein is produced during inflammatory processes, but its precise function in renal diseases in unclear. ALECT2, however, is known to be a relatively common form of renal amyloidosis, after amyloid light chain and serum amyloid A types and is most often seen in patients of Hispanic ethnicity. ALECT2 can occur de novo or as recurrent disease in kidney transplants. We present the first case, to our knowledge, of de novo ALECT2 in a pediatric kidney transplant patient, 15 years post‐transplant. [ABSTRACT FROM AUTHOR]