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Idiopathic multicentric Castleman disease treated with siltuximab for 15 years: a case report.

UNLABELLED: Human herpes virus-8 (HHV8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disorder sustained by pro-inflammatory cytokines, including interleukin-6 (IL-6). According to the international evidence-based criteria developed by the Castleman Disease Collaborative Network (CDCN), siltuximab, which works by inhibiting IL-6, is the recommended choice for iMCD treatment. We report a case of a 63-year-old white male with iMCD who has been on maintenance therapy with siltuximab for 15 years - representing one of the longest treatment periods of any

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… The patient tested negative for antinuclear antibodies and no reticulin fibrosis was present. Evaluation of the patient found no evidence of monoclonal gammopathy. …

Single agent selinexor is active in patients with myelofibrosis refractory or intolerant to JAK inhibitors.

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… At baseline, 10 (58.8%) patients had MF-2 reticulin fibrosis, and 7 (41.2%) MF-3. At EOT, in five of 14 (35.7%) evaluable patients, MF had decreased by one grade, and in one by two. One patient progressed from MF-2 to MF-3 (Supplementary Table 5 ; Suppl. Fig. 5 ). …

Extreme Peripheral Blood Plasmacytosis Mimicking Plasma Cell Leukemia as a Presenting Feature of Angioimmunoblastic T-Cell Lymphoma (AITL).

Angioimmunoblastic T-cell lymphoma (AITL) is one of four major subtypes of nodal peripheral T cell lymphoma, characterized by its cell of origin, the follicular helper T-cell (TFH). Patients typically present with prominent constitutional (B) symptoms, generalized lymphadenopathy, hepatosplenomegaly, cytopenias, and rash. Here we present a case of a 62-year-old male with progressive cervical adenopathy, fevers and weight loss presenting with extreme polyclonal plasmacytosis and high plasma EBV viral load. While the initial presentation appeared to mimic plasma cell leukemia or severe infection

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… Vascular proliferation and reticulin fibrosis have also been reported. Secondary changes include trilineage hyperplasia and polyclonal plasmacytosis. …

Role of IL-5 in eosinophil-associated diseases and prospects for multi-target therapy.

Eosinophil-associated diseases are a group of inflammatory disorders characterized by abnormal eosinophil infiltration, which significantly impacts patients' quality of life. Interleukin-5 (IL-5), a critical cytokine that regulates eosinophil development, activation, chemotaxis, and survival, plays a central role in the pathogenesis of these diseases. This review systematically examines the molecular structure and signaling pathways of IL-5, its mechanisms of action in asthma and chronic obstructive pulmonary disease (COPD), and the development and clinical applications of monoclonal antibodie

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… Certain compounds, such as reticuline, have been shown to attenuate inflammation by inhibiting this pathway ( 28 ). … Anti-inflammatory effects of reticuline on the JAK2/STAT3/SOCS3 and p38 MAPK/NF-κB signaling pathway in a mouse model of obesity-associated asthma. Clin Respir J. (2024) 18:e13729. doi: 10.1111/crj.13729. PMID: 29. Yu H, Huang X, Xie C, Song J, Zhou Y, Shi H, et al. …

Новое применение XPO1 для лечения миэлофиброза.

Myelofibrosis (MF) — новообразование, характеризующееся конституционными симптомами, прогрессивными цитопентиями и селеногамели. В основе патобологии MF лежит активация мутации на пути JAK/STAT и регулирование цитокинового костного мозга. Хотя многие ингибиторы JAK в настоящее время официально утверждены и обеспечивают значительное улучшение симптомов, эти агенты не имеют курса на изменение потенциала. Кроме того, результаты являются неудовлетворительными для пациентов, которые не справляются с ингибиторами JAK, что подчеркивает необходимость применения новых методов лечения, основанных на исп

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… Clonal expansion of a single mutated hematopoietic stem cell (HSC) leads to hyperplasia of varying degrees in 1 or multiple hematopoietic cell lines: predominantly erythroid cells in PV, megakaryocytes in ET, and the deposition of reticulin and collagen fibers in PMF. 4 Expansion of the malignant HSC … are involved in clonal expansion of the malignant HSC in MPNs, many downstream cell signaling pathways have been further implicated as fundamental mediators of bone marrow fibrosis, specifically transforming growth factor β (TGF-β) and NF-κB. 8 , 9 Bone marrow fibroblasts, which deposit collagen and reticulin …

Мультиорганическая неисправность 5 Синдром цитокина у пациента с множественной миеломой после вливания карфилзомиба: доклад о деле.

Фон: Синдром высвобождения цитокина (CRS) представляет собой опасное для жизни системное воспалительное состояние, характеризующееся чрезмерным производством цитокина, что ведет к многоорганической дисфункции. Она обычно связана с Т-комплексными терапиями, такими, как химериковые антигенные рецепторы (CAR) Т клетки, Т-клекторные двуэлементные молекулы и моноклонные антитела. Известно, что карфилзомиб, протеиновый ингибитор, вызывает целый ряд негативных последствий, в первую очередь гематологическое и сердечно-сосудистое. Тем не менее, многоорганический уровень отказа 5 (Смертельная), аналогич

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… HLH—hemophagocytic lymphohistiocytosis; IRIS—immune reconstitution inflammatory syndrome; TAFRO—thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly; TLS—tumor lysis syndrome. …

Lambda light chain-induced monoclonal gammopathy of renal significance, manifesting with Fanconi Syndrome and osteomalacia.

BACKGROUND: Monoclonal gammopathy of renal significance (MGRS) encompasses a heterogeneous group of kidney diseases in which a monoclonal immunoglobulin secreted by a clone of B cells or plasma cells causes kidney damage without meeting the hematological criteria for malignancy. Among the various forms of involvement, MGRS can manifest as a proximal tubule disorder, such as Fanconi syndrome (FS), characterized by urinary loss of phosphate, glucose, amino acids, uric acid and bicarbonate. Few cases of MGRS have been described in the literature, manifesting as FS and monoclonal production of lam

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… Hypoplastic erythroid and granulocytic series with maturation delay, small and hypolobulated megakaryocytes, unaltered reticulin pattern. Screening for amyloid protein was negative and it should be mentioned that bone marrow flow cytometry analysis was not performed. …

Idiopathic Multicentric Castleman Disease with Cutaneous Manifestation: Case Report.

Castleman disease constitutes a rare class of lymphoproliferative disorders, with an estimated incidence of 21 to 25 per million patient years. The idiopathic subtype exhibits a significantly diverse clinical presentation, which can imitate many autoimmune, malignant, and infectious diseases. Cutaneous manifestations are uncommon and require in-depth investigation, especially when concurrent lymphadenopathy is present. A 79-year-old female, with a chronic, complicated erysipelas-like lesion, presented with bilaterally enlarged inguinal lymph nodes; after surgical excision, their histopathologi

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… The latter subtype can be further classified, based on clinical manifestation, into iMCD-TAFRO (thrombocytopenia, ascites, reticulin fibrosis, renal dysfunction, and organomegaly) and iMCD-NOS (not otherwise specified) [ 9 ]. …

Advanced Diagnostic Techniques

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Histiocytoid giant cellulitis-like Sweet syndrome at the site of sternal aspiration: A case report and review of literature.

BackgroundGiant cellulitis-like Sweet syndrome (SS) is a rare subtype of SS, and reports of the combined histiocytoid type of pathology are scarce. Here, we report a case of SS with distinctive clinical presentations and which was difficult to distinguish from cellulitis. By sharing this case and a discussion of the related literature in detail, we aim to provide clinicians with new insights into the characteristics of histiocytoid giant cellulitis-like (HGC)-SS and the pathogenesis of SS.Case summaryA 52-year-old male was admitted after experiencing progressive fatigue for 1 mo and tongue swe

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… The reticulin fibrosis was grade 2 (designated MF-2), according to the World Health Organization grading for myelofibrosis. Flow cytometry and chromosomal and gene mutation testing were not performed due to the difficulties in obtaining bone marrow fluid. …

Клинические особенности и лечение недавно диагностированной миеломы с вторичным миэлофиброзом: ретроспективное исследование.

Вторичный миэлофиброз (СМФ) характеризуется чрезмерным осаждением волокнистых тканей сверху первичной болезни, что часто приводит к затмению клинических проявлений первичным заболеванием. К сожалению, нынешние системы сборки не включают миэлофиброз, что ведет к потенциальным задержкам в лечении SMF. Оценить прогнозы пациентов с множественной миеломой (ММ), сложной с миэлофиброзом.DESIGN: Исследование включало клинические данные и результаты лечения 208 недавно диагностированные несколько пациентов, страдающих миеломой (НДММ), которые с января лечились в аффилированной больнице Университета Цин

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… Myelofibrosis (MF) is a condition characterized by the abnormal deposition of reticulin fibers and collagen in the bone marrow and can arise from various malignant and nonmalignant diseases. …

Clinical characteristics and outcomes of Castleman disease: A multicenter study of 185 Chinese patients.

Castleman disease (CD) is a rare lymphoproliferative disorder. To assess the clinical features, outcomes, and prognostic factors of this disease, we retrospectively analyzed 185 HIV-negative CD patients from four medical centers in southern China. The median age was 37 years. One hundred and twenty-one patients (65.4%) were classified as unicentric CD (UCD) and 64 patients (34.6%) were classified as multicentric CD (MCD). The histology subtype was hyaline-vascular for 132 patients (71.4%), plasma cell for 50 patients (27%), and mixed type for 3 patients (1.6%). The 5-year overall survival (OS)

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… Thrombocytopenia with reticulin fibrosis accompanied by fever, anasarca and hepatosplenomegaly: a clinical report of five cases . J Clin Exp Hematop . 2013 ; 53 : 63 ‐ 68 . 23801136 10.3960/jslrt.53.63 52 Masaki Y , Nakajima A , Iwao H , et al. …

Profile of pomalidomide and its potential in the treatment of myelofibrosis.

Myelofibrosis, a Philadelphia-negative myeloproliferative neoplasm, is in a new treatment era after the discovery of the JAK2V617F mutation in 2005. JAK inhibitors boast improvements in disease-related symptoms, splenomegaly, and overall survival; however, treatment of myelofibrosis remains a challenge, given the lack of improvement in cytopenias with these agents. Second-generation immunomodulatory agents, such as pomalidomide, have shown efficacy in myelofibrosis-associated anemia within multiple clinical trials. Five major pomalido-mide clinical trials have been completed to date, and demon

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… Ten patients had a reduction in reticulin fibrosis and eight patients experienced a reduction in their baseline mutant allele burden. Grade 3 or higher toxicity occurred, including neutropenia (58%), anemia (42%), and thrombocytopenia (13%), but was generally well tolerated. …

Inflammatory Microenvironment and Specific T Cells in Myeloproliferative Neoplasms: Immunopathogenesis and Novel Immunotherapies.

The Philadelphia-negative myeloproliferative neoplasms (MPNs) are malignancies of the hematopoietic stem cell (HSC) arising as a consequence of clonal proliferation driven by somatically acquired driver mutations in discrete genes (JAK2, CALR, MPL). In recent years, along with the advances in molecular characterization, the role of immune dysregulation has been achieving increasing relevance in the pathogenesis and evolution of MPNs. In particular, a growing number of studies have shown that MPNs are often associated with detrimental cytokine milieu, expansion of the monocyte/macrophage compar

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… Although the primary end point (>50% reduction in JAK2V617F allelic burden) was not reached for any of the patients, BM evaluation (available for 20 out of 39 patients) showed an increase of the nestin+ MSCs, a slight decrease in the reticulin fibers and a trend towards a reduction of megakaryocyte clusters …

Spectrum of Pancytopenia in Adults Attending a Clinical Hematology Department: A Four-Year Experience From a Tertiary Care Center of Western India.

Background Pancytopenia is a common hematological condition encountered in clinical practice. Because there is a wide variation in causes of pancytopenia even in different populations of the same geographical region, identifying the pattern of underlying etiologies is crucial for proper management. This study was conducted to evaluate the clinico-hematological profile and different etiologies of pancytopenia among adults presenting to a clinical hematology department at a tertiary care hospital of Western India. Methodology This observational study was conducted over a period of four years on

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… BM fibrosis (MF) grading was done in select patients using reticulin stain on BM biopsy sections. Sixty (11%) patients had marrow fibrosis (MF grade ≥1). Primary myelofibrosis and hairy cell leukemia cases showed the most severe form of MF. …

Clinicopathological Features and Prognostic Differences Between Primary and Relapsed or Refractory Extramedullary Multiple Myeloma.

PURPOSE: This study aims to explore the clinicopathological features related to extramedullary multiple myeloma (EMM) occurrence and evaluate prognosis differences between patients with primary EMM (PEMM) and those with relapsed or refractory EMM (R/REMM). PATIENTS AND METHODS: The laboratory test results, clinical, histopathological and immunophenotypic characteristics, cytogenetic changes and prognosis of 203 patients with MM were obtained. The patients were categorized into EMM+ and EMM- groups. Patients in the EMM+ group were further categorized into the PEMM and R/REMM groups based on th

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… Reticular fiber staining was performed using a silver impregnation kit for reticulin (Artisan Link Pro; Agilent, Santa Clara, CA, USA; Ventana BenchMark Ultra, Ventana Medical Systems) according to the manufacturer’s instructions. …

Advanced Diagnostic Techniques

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Myelodysplasia in VEXAS syndrome.

AbstractVEXAS (vacuoles, E1 enzyme, X linked, autoinflammatory, somatic) syndrome is a clonal hematoinflammatory disorder affecting older, predominantly male patients, characterized by systemic inflammation and progressive bone marrow failure. It is caused by somatic mutations in ubiquitin-activating enzyme E1 (UBA1), an X-linked gene, essential for initiating cellular ubiquitination. Loss-of-function mutations, most commonly M41 missense variants, lead to decreased expression of the cytoplasmic isoform, UBA1b, resulting in accumulation of misfolded proteins and activation of the endoplasmic r

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… differentiation, with the myeloid differentiation demonstrating a neutrophil maturation bias. 4 , 28 Impaired ubiquitination in UBA1 -mutant HSPCs would be expected to result in proteomic stress and cell death; however, a recent study suggests that prosurvival signals through IRE1, PERK, and ATF4 in endoplasmic reticulin …

Long-term treatment outcome of Castleman's disease: A real-world experience.

BACKGROUND: Castleman disease (CD), classified as unicentric CD (UCD) or multicentric CD (MCD), is a rare non-neoplastic lymphoproliferative disorder of unknown origin. Owing to its rarity, the clinical characteristics, therapeutic modalities, treatment outcomes, and prognostic factors related to UCD or MCD are not well defined. METHOD: We retrospectively analyzed 88 patients with CD, including those with hyaline-vascular, plasma-cell, mixed type, hypervascular, and plasmablastic subtypes, for presenting symptoms, physical, laboratory, and radiologic findings, and treatment response in the Ko

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… Thrombocytopenia with reticulin fibrosis accompanied by fever, anasarca and hepatosplenomegaly: A clinical report of five cases . J Clin Exp Hematop ( 2013 ) 53 : 63–8 . doi: 10.3960/jslrt.53.63 23801136 35 Iwaki N Fajgenbaum DC Nabel CS Gion Y Kondo E Kawano M . …

Real-practice management and treatment of idiopathic multicentric Castleman disease with siltuximab: a collection of clinical experiences.

Castleman disease (CD) is a group of lymphoproliferative disorders that share common histopathological features yet have widely different aetiologies, clinical features and grades of severity as well as treatments and outcomes. Siltuximab is currently the only therapy approved by the FDA and EMA for idiopathic multicentric CD and is recommended as first-line therapy in treatment guidelines. Despite the extensive characterization of siltuximab treatment in clinical trials, available evidence from real-world practice is still scant. This collection of clinical experiences focuses on patients tre

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… required. 6 Furthermore, a differential diagnosis should be made between iMCD, POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell neoplasm and skin changes), and TAFRO syndrome (poorer prognosis, with the clinical characteristics being thrombocytopenia, anasarca, fever, reticulin …