The eosinophilias encompass a broad range of nonhematologic (secondary or reactive) and hematologic (primary or clonal) disorders with potential for end-organ damage. Based on new clinical data and increased understanding of disease molecular genetics, the World Health Organization (WHO) and the international consensus classification (ICC) has provided updated criteria and classifications for eosinophilic disorders in 2022. This guideline represents an update of Chinese expert consensus on the diagnosis and treatment of eosinophilia published in 2017 and aim to provide Chinese hematologist with clear guidance on management for eosinophilic disorders.
嗜酸性粒细胞增多症包括一组非血液系统（继发或反应性）和血液系统（原发或克隆性）疾病，该组疾病可能伴有器官受损。基于新的临床研究数据和对疾病分子遗传学解析结果，2022年世界卫生组织（WHO）和国际共识分型（ICC）对嗜酸性粒细胞增多性疾病的诊断和分型标准进行了更新。本指南是在《嗜酸性粒细胞增多症诊断和治疗中国专家共识（2017年版）》基础上的更新，旨在为我国血液学工作者提供一个规范的嗜酸性粒细胞增多症的诊断程序、实验室检查、诊断标准和治疗原则。.

Acquired clonal chromosomal abnormalities (CAs) are usually considered to be disease-related. However, when a CA of this type is the only abnormality present (and especially in small clones), the clinical significance is unclear. Here, we review the literature on recurrent CAs whose significance is regularly subject to debate. Our objective was to help with their interpretation and develop guidelines for sex chromosome loss, trisomy 15, trisomy 8, deletion 20q and other isolated non-myelodysplastic neoplasm (MDS)-defining CAs. We suggest that non-MDS-defining CAs correspond to clonal hematopoiesis of indeterminate potential (CHIP) in the absence of cytopenia and clonal cytopenia of undetermined significance (CCUS) in the presence of cytopenia. Lastly, we review the literature on persistent polyclonal binucleated B-cell lymphocytosis; although usually benign, this condition may correspond to a premalignant state.

By acclimatizing CCR5-tropic tier 1B SHIV-MK1 to rhesus monkeys, a tier 2 SHIV-MK38 strain with neutralization resistance and high replication ability was generated. In this study, we generated SHIV-MK38C, a monkey-infectious consensus molecular clone of SHIV-MK38. Analysis using pseudotype viruses showed that MK38C was tier 1C because it lacked the N169D mutation, which is the most important mutation for neutralization resistance. MK38C harboring the N169D mutation became tier 2. However, the replication ability of SHIV-MK38C with N169D was low; more than 17 weeks elapsed before its detection in monkeys. Tier 1C MK38C was sensitive to a CD4 mimic. Therefore, SHIV-MK38C could be used to evaluate CD4 mimics in vivo.

Monoclonal gammopathy of renal significance (MGRS) is a new nosology in modern nephrology and oncohematology. MGRS is defined as kidney injury due to nephrotoxic monoclonal immunoglobulin produced by the B-cell line clone which does not reach the hematological criteria for specific treatment initiation. Monoclonal proteins pathological effects on kidney parenchyma result in irreversible decline of kidney function till the end stage renal disease that in line with the position of International Consensus of hematologists and nephrologists determinates critical necessity for clone specific treatment in patients with MGRS despite the absence of hematological indications for treatment initiation. Main challenge of MGRS in Russian Federation is an inaccessibility of an in-time diagnostic and appropriate treatment for the great majority of patients due to the following reasons: 1) limited knowledge about the MGRS among hematologists and nephrologists; 2) lack of necessary diagnostic resources in most health-care facilities; 3) lack of approved clinical recommendations and medical economic standards for treatment of this pathological entity. Consensus document comprises the opinion of experts leading nephrologists and hematologists of Russian Federation on the problem of MGRS including the incoherence in nosology classification, diagnostics approach and rationale for clone specific treatment. Consensus document is based on conclusions and agreements reached during the conference of leading nephrologists and hematologists of Russia which was held in the framework of symposia Plasma cell dyscrasias and lymphoproliferative diseases: modern approaches to therapy, 1516 of March 2019, Pavlov First Saint Petersburg State Medical University. The present Consensus is intended to define the principal practical steps to resolve the problem of MGRS in Russian Federation that are summarized as final clauses.
Моноклональная гаммапатия ренального значения (МГРЗ) представляет собой новую нозологическую группу в современной нефрологии и онкогематологии. Под МГРЗ понимают поражение почек, обусловленное действием нефротоксичного моноклонального иммуноглобулина, продуцируемого клоном В-клеточной линии, который не достигает критериев, необходимых для начала противоопухолевой терапии по онкогематологическим показаниям. Результатом воздействия моноклонального белка на почечную паренхиму является неуклонное прогрессирование дисфункции почек вплоть до утраты функции органа, что в соответствии с единой позицией международного консенсуса гематологов и нефрологов определенно указывает на необходимость клон-ориентированного лечения МГРЗ, несмотря на отсутствие критериальных онкогематологических показаний. Основной проблемой МГРЗ в Российской Федерации является недоступность для большинства пациентов своевременной диагностики и лечения данной патологии, что обусловлено, во-первых, недостаточной осведомленностью гематологов и нефрологов страны в отношении МГРЗ, во-вторых, отсутствием в большинстве медицинских учреждений необходимых диагностических ресурсов, в-третьих, отсутствием утвержденных рекомендаций и медико-экономических стандартов лечения этой болезни. Текст настоящего консенсуса заключает в себе мнение специалистов РФ в отношении нозологической классификации, диагностики и подходов к терапии МГРЗ и основан на итогах проведенного совместного совещания ведущих гематологов и нефрологов страны. Совещание состоялось 1516 марта 2019 г. в рамках симпозиума Плазмоклеточные дискразии и лимфопролиферативные заболевания: новые подходы к терапии, проведенного в ФГБОУ ВО Первый СПбГМУ им. акад. И.П. Павлова. Настоящий консенсус гематологов и нефрологов призван наметить основные практические пути решения проблемы МГРЗ в РФ, которые кратко сформулированы в виде заключительных положений.

Paroxysmal nocturnal hemoglobinuria (PNH) arises as a consequence of the non-malignant clonal expansion of one or more hematopoietic stem cells with an acquired somatic mutation of the PIGA gene (Brodsky RA. Blood 113 (2009) 6522-6527). Progeny of affected stem cells are deficient in glycosyl phosphatidylinositol-anchored proteins (GPI-APs). This deficiency is readily detected by flow cytometry. Though this seems straightforward, the clinical utility of this testing requires that the ordering clinician understand not only the characteristics of the test, but also the biology of the underlying disease, and the clinical and laboratory manifestations in the individual patient. When interpreted correctly, the results from PNH flow cytometry testing, including presence and size of the clonal populations and the cell types involved, can allow the clinician to classify the disease appropriately; evaluate the risk of disease progression; and subsequently monitor response to therapy. In these guidelines, we discuss the evaluation of a patient with suspected PNH or other bone marrow failure disorders, with specific emphasis on the contribution of this testing to the diagnosis, classification, and monitoring of patients. For convenience we will commonly refer to these flow cytometry studies as \"PNH testing\" recognizing that an abnormal result is not diagnostic of PNH; rather both laboratory and clinical features are used to establish this diagnosis. © 2017 International Clinical Cytometry Society.

The accurate distinction of reactive and neoplastic lymphoid proliferations can present challenges. Given the different prognoses and treatment strategies, a correct diagnosis is crucial. Molecular clonality assays assess rearranged lymphocyte antigen receptor gene diversity and can help differentiate reactive from neoplastic lymphoid proliferations. Molecular clonality assays are commonly used to assess atypical, mixed, or mature lymphoid proliferations; small tissue fragments that lack architecture; and fluid samples. In addition, clonality testing can be utilized to track neoplastic clones over time or across anatomic sites. Molecular clonality assays are not stand-alone tests but useful adjuncts that follow clinical, morphologic, and immunophenotypic assessment. Even though clonality testing provides valuable information in a variety of situations, the complexities and pitfalls of this method, as well as its dependency on the experience of the interpreter, are often understated. In addition, a lack of standardized terminology, laboratory practices, and interpretational guidelines hinders the reproducibility of clonality testing across laboratories in veterinary medicine. The objectives of this review are twofold. First, the review is intended to familiarize the diagnostic pathologist or interested clinician with the concepts, potential pitfalls, and limitations of clonality testing. Second, the review strives to provide a basis for future harmonization of clonality testing in veterinary medicine by providing diagnostic guidelines.

Tumors are heterogeneous in composition. They are composed of cancer cells proper, along with stromal elements that collectively form a microenvironment, all of which are necessary to nurture the malignant process. In addition, many of the stromal cells are modified to support the unique needs of the malignant state. Tumors are composed of a variety of clones or subpopulations of cancer cells, which may differ in karyotype, growth rate, expression of cell surface markers, sensitivity to therapeutics, etc. New tools and methods to provide an improved understanding of tumor clonal architecture are needed to guide therapy.

BACKGROUND: Comparison between consensual approaches for the detection of paroxysmal nocturnal hemoglobinuria (PNH) clones by flow cytometry (FCM) following the international clinical cytometry society (ICCS) guidelines has not been widely reported.
METHODS: We determined the performance characteristics of 4, 5, and 6-color protocols for white blood cell (WBC) and one and two-color protocols for red blood cell (RBC) evaluation for different PNH target clones and compared results from PNH patient analysis.
RESULTS: Coefficient of variation (CV) for precision/reproducibility analysis ranged from 0.01%/0.12% to 2.56%/ 3.59% for granulocytes, from 0.07%/0.08% to 3.87%/11.61% for monocytes and from 0.4%/1.02% to 6.53%/ 5.1% for RBCs within different approaches and target PNH clones. Comparison of individual protocols revealed excellent correlation (r = 0.99), Wilcoxon rank tests found no statistically significant differences (p > 0.05), Bland-Altman analysis proved agreement for all PNH clones (mean bias ranging from 0.02 to 2.2).
CONCLUSIONS: Our results confirm good intralaboratory characteristics for precision and reproducibility analysis, excellent correlation and agreement between approaches underlining the primary role of optimally selected glycophosphatidylinositol (GPI)-specific reagents and secondary role of number, type of gating reagents and gating strategy.

BACKGROUND: Evaluation of paroxysmal nocturnal hemoglobinuria (PNH) clones by flow cytometry (FCM) is not standardized and is associated with consistent inter-laboratory variability.
METHODS: In order to rule out the influence of particular approach in generating final results, we analyzed the performance characteristics of individual consensus strategies for small to intermediate (1%-20%) and minor (<1%) PNH clones within the white blood cell (WBC) and red blood cell (RBC) compartments with sensitivity up to 0.1%.
RESULTS: Coefficient of variation (CV) for precision/reproducibility analysis ranged from 0.67%/1.49% to 2.56%/3.09% for granulocytes, from 0.93%/3.09% to 7.76%/12.06% for monocytes and from 0.41%/4.73% to 6.53%/5.1% for RBCs. Coefficient of determination (r2) for linear regression analysis ranged from 0.95 to 0.99, Wilcoxon ranks test showed no statistically significant differences (p>0.05), Bland-Altman analysis demonstrated performance agreement with mean bias ranging from -0.18 to 1.24.
CONCLUSIONS: Our results confirmed very good performance characteristics for precision and reproducibility analysis, excellent correlation and favorable agreement between strategies, suggesting that reported inter-laboratory variability is related mainly to incorrect performance and/or insufficient experience with PNH testing by flow cytometry, rather than to relevant limitations of any particular approach.

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