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После-пересадки двойное злодейство: Множественная миелома и папиллярная рентгеновская ячейка Карцинома-А.

Фон и клиническая значимость: получатели почечных трансплантаций имеют 2-4-fold более высокий риск раковых заболеваний по сравнению с населением в целом. Целостное появление карциномы миеломы (ММ) и почечной клетки коренных почек (КЦК) является редким явлением и создает конкурирующие приоритеты между эффективностью борьбы с миломой и сохранением сплава. Представление дела: A 54-year-old с женщиной 2020 Пересадка живой донорской почки 2024 с болью в костях и опухолью плеча. КТ всего тела с низкой дозой выявила множественные остеолитические повреждения. Проверка показала, что М-протеин ИгГ. 38.5

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… Among the most common post-transplant lymphoproliferative disorders (PTLDs), rarer hematologic neoplasms such as multiple myeloma (MM) have also been described [ 14 ]. … Post-transplant Lymphoproliferative Disorder J. Assoc. Physicians India 2017 65 102 103 29327533 14. Caillard S. Lelong C. Pessione F. Moulin B. Post-transplant lymphoproliferative disorders after renal transplantation in adults: Report of 230 cases Am. J. …

Immune effector cell-associated enterocolitis post-BCMA directed CAR T-cell therapy: insights from a multicenter case series.

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Letter from the Editors.

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… In the next paper Dr Jacene discusses the most common indications for autologous stem cell transplant in which FDG-PET/CT has been evaluated, including for lymphoma and multiple myeloma. 20 Post-transplant lymphoproliferative disorders (PTLD) are a spectrum of heterogeneous lymphoproliferative conditions … PET/CT for lymphoma post-therapy response assessment in hodgkin lymphoma and diffuse large B-cell lymphoma Semin Nucl Med 48 2018 28 36 29195615 10.1053/j.semnuclmed.2017.09.003 24 Song H Guja KE Iagaru A. 18F-FDG PET/CT for evaluation of post-transplant lymphoproliferative disorder Sem Nucl Med 51 …

Patterns of geriatric anemia: A hospital-based observational study in North India.

BackgroundGeriatric anemia is a global health problem because of its high prevalence and associated significant morbidity and mortality.AimThe objectives of this study were to estimate the pattern of anemia in the elderly patients and the underlying etiology of anemia.Research design and methodsThis was a hospital-based prospective observational study, conducted in patients aged 60 years and above at PGIMER, Chandigarh, a tertiary care center of North India. Anemia is defined as hemoglobin level less than 13 g/dl in men and 12 g/dl in women.ResultsAmong the 105 older patients with anemia, the

Pulmonary amyloidosis: clinicopathologic spectrum and diagnostic pitfalls in a 12-patient case series.

BACKGROUND: Pulmonary amyloidosis is a rare, underrecognized manifestation of amyloid disease that may present as nodular, tracheobronchial, or diffuse alveolar-septal involvement and can mimic common pulmonary conditions. This study aimed to characterize the clinicopathologic features, diagnostic patterns, and management approaches of patients with pulmonary amyloidosis in a real-world setting. METHODS: We performed a retrospective review of patients with pathology-confirmed pulmonary amyloidosis diagnosed between January 2020 and December 2024 at a tertiary academic medical center in Detroi

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… Interestingly, nodular pulmonary amyloidosis has been associated with indolent B-cell lymphoproliferative disorders, likely due to localized light chain deposition. … Nodular pulmonary amyloidosis is characterized by localized immunoglobulin deposition and is frequently associated with an indolent B-cell lymphoproliferative disorder. Am J Surg Pathol 2013;37:406-12. 10.1097/PAS.0b013e318272fe19 29. Cordier JF, Loire R, Brune J. …

Early-stage IgM kappa proliferative glomerulonephritis with monoclonal immunoglobulin deposition presenting with atypical renal insufficiency: a case report and literature review.

INTRODUCTION: Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a rare subtype of monoclonal gammopathy of renal significance (MGRS). While the IgG subtype has been well characterized, the IgM-κ variant is extremely rare, with a low detection rate of circulating monoclonal proteins and limited real-world data on its clinical course under conservative management. This case report describes a biopsy-proven IgM-κ PGNMID in an elderly patient with negative hematological screening, to expand the current understanding of this rare entity. PATIENT CONCERNS AND DIAG

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… Second, whole-body PET-CT and peripheral blood flow cytometry were not performed at initial presentation, which may have missed potential underlying B-cell lymphoproliferative disorders. … Kidney diseases associated with monoclonal immunoglobulin M-secreting B-cell lymphoproliferative disorders: a case series of 35 patients . Am J Kidney Dis . ( 2015 ) 66 : 756 – 67 . doi: 10.1053/j.ajkd.2015.03.035 , 25987261 16. …

Biological Approaches to Aggressive Cutaneous B-Cell Lymphomas.

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… The presence of PDL1 positive tumor cells in PCDLBCL-LT could explain both the poor prognosis of this tumor and the efficacy of anti PD1/PDL1 therapy in this lymphoproliferative disorder ( 16 , 17 ). … The infusion of CD19-specific CAR-T cells can be efficacious in B-lymphoproliferative disorders ( 22 ). …

Необычные образования, необычные задачи: обзор нарушений ячеек из реалистической плазмы.

Редкие расстройства плазменных клеток, включая IgD, IgE и IgM, множественные миеломы, несекретарские миэломы (NSMM), лейкемию плазмы (PCL) и тяжелые цепные заболевания (HCD), биологически неоднородны и часто присутствуют с атипичными характеристиками и агрессивным поведением. В этом обзоре обобщаются текущие данные об их эпидемиологии, патофизиологии, диагностике и лечении. Достижения в протеиновых ингибиторах, иммуномудуляторных агентах и аутологической трансплантации улучшили результаты в отдельных подтипах. Вместе с тем сохраняются проблемы, связанные с проведением различия между миэломой И

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… Management depends on the associated lymphoproliferative disorder. Indolent cases may be monitored without immediate therapy [ 66 , 69 ]. DLBCL-type γ-HCD is treated using R-CHOP or other anthracycline-based regimens [ 73 ]. … Gamma heavy-chain disease: Defining the spectrum of associated lymphoproliferative disorders through analysis of 13 cases Am. J. Surg. Pathol. 2012 36 534 543 10.1097/PAS.0b013e318240590a 22301495 PMC3715127 70. Chang L. Zhou D.B. Cao X.X. …

Clinical management of cancer after kidney transplantation.

Advances in immunosuppressive therapy have markedly improved short-term graft survival post-kidney transplantation (KT); however, these advances have also profoundly altered long-term disease patterns in transplant recipients. Cancer remains a major barrier limiting improvements in long-term survival. Kidney transplant recipients are at increased risk of malignancy owing to impaired graft function and immunosuppressive medications. To improve cancer-related outcomes, comprehensive strategies encompassing prevention, early detection through screening, and appropriate treatment are required. Pri

Monoclonal Gammopathy of Undetermined Significance (MGUS)-Not So Asymptomatic after All.

Monoclonal Gammopathy of Undetermined Significance (MGUS) is considered to be a benign precursor condition that may progress to a lymphoproliferative disease or multiple myeloma. Most patients do not progress to an overt condition, but nevertheless, MGUS is associated with a shortened life expectancy and, in a minority of cases, a number of co-morbid conditions that include an increased fracture risk, renal impairment, peripheral neuropathy, secondary immunodeficiency, and cardiovascular disease. This review aims to consolidate current evidence for the significance of these co-morbidities befo

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… Therefore, most patients with MGUS do not progress to symptomatic MM or other lymphoproliferative disorders [ 7 ]. … The association of non-IgM MGUS and peripheral neuropathy first needs consideration of the lymphoproliferative disorders with distinct management of AL amyloidosis and POEMS syndrome (an acronym of Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, and Skin changes). …

Society for Immunotherapy of Cancer (SITC) clinical practice guideline on immunotherapy for the treatment of lymphoma.

The recent development and clinical implementation of novel immunotherapies for the treatment of Hodgkin and non-Hodgkin lymphoma have improved patient outcomes across subgroups. The rapid introduction of immunotherapeutic agents into the clinic, however, has presented significant questions regarding optimal treatment scheduling around existing chemotherapy/radiation options, as well as a need for improved understanding of how to properly manage patients and recognize toxicities. To address these challenges, the Society for Immunotherapy of Cancer (SITC) convened a panel of experts in lymphoma

Двойная автономность: синдром Эванса и множественный склероз.

Синдром Эванса (ES) — редкое аутоиммунное расстройство, связанное с аутоиммунной гемолитической анемией и иммунной тромбоцитопенией (ИТП). Пациенты, как правило, страдают от петехии, пурпуры, паллора и усталости. Диагностические критерии включают в себя положительное прямое антиглобулиновое испытание, ретикулокитоз, снижение уровня гаптоглобина, а также повышенный косвенный уровень билюродина и лактата дегидрогеназы. ЭУ может быть связано с другими аутоиммунными заболеваниями, включая системный волчанки эритематос, ревматоидный артрит и аутоиммунный гепатит. Вместе с тем в научной литературе в

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… Causes of secondary ES include lymphoproliferative disorders, common variable immunodeficiency, and inborn errors of immunity [ 24 , 25 ]. The patient’s normocellular bone marrow biopsy results further ruled out causes of secondary ES [ 24 ]. …

Herpes Zoster in Hematological Disorders: Pathogenesis, Risk Stratification, and Emerging Strategies for Prevention and Immunization.

BACKGROUND: Herpes zoster (HZ), resulting from reactivation of latent varicella-zoster virus (VZV), imposes a significant burden on immunocompromised patients, particularly those with hematological malignancies and recipients of hematopoietic stem cell transplants (HSCT). These populations face markedly increased risks of severe complications, including disseminated disease and postherpetic neuralgia. OBJECTIVE: This review examines the pathogenesis, epidemiology, risk factors, and evolving preventive strategies for HZ in patients with hematological disorders, with an emphasis on antiviral pr

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… Moreover, persistent lymphopenia and hypogammaglobulinemia—common in chronic lymphoproliferative disorders such as chronic lymphocytic leukemia and multiple myeloma—further compound the susceptibility to reactivation risk. … disorders | Yes, during/after fludarabine/bendamustine | At diagnosis or before therapy (elderly) Non‐malignant hematologic disease | Consider in elderly on steroids | At diagnosis (elderly) The emergence of mRNA‐based zoster vaccines introduces an exciting new frontier. …

Evans syndrome: clinical perspectives, biological insights and treatment modalities.

Evans syndrome (ES) is a rare and chronic autoimmune disease characterized by autoimmune hemolytic anemia and immune thrombocytopenic purpura with a positive direct anti-human globulin test. It is classified as primary and secondary, with the frequency in patients with autoimmune hemolytic anemia being 37%-73%. It predominates in children, mainly due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. ES during pregnancy is associated with high fetal morbidity, including severe hemolysis and intracranial bleeding with neurological sequelae and death. The clinical presenta

Decoding Immunodeficiencies with Artificial Intelligence: A New Era of Precision Medicine.

Primary and secondary immunodeficiencies comprise a wide array of illnesses marked by immune system abnormalities, resulting in heightened vulnerability to infections, autoimmunity, and cancers. Notwithstanding progress in diagnostic instruments and an enhanced comprehension of the underlying pathophysiology, delayed diagnosis and underreporting persist as considerable obstacles. The implementation of artificial intelligence into clinical practice has surfaced as a viable method to enhance early detection, risk assessment, and management of immunodeficiencies. Recent advancements illustrate ho

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… In fact, authors presented new indicators of immune dysregulation and IEIs: cancer, cytopenia, lymphoproliferative disorders, and myelodysplastic disorders [ 47 ]. … SID in Lymphoproliferative Disorders Recognizing biomarkers that can distinguish PIs from secondary forms in patients with chronic B-cell lymphoproliferative disorders (B-CLPD) is of fundamental importance to improve the diagnosis, treatment, and prognosis of these patients. …

Therapeutic Antibodies in Hematology: Advances in Malignant and Non-Malignant Disorders.

Therapeutic antibodies have revolutionized hematology, offering targeted and effective treatments for both malignant and non-malignant diseases. In hematologic malignancies, anti-CD20, anti-CD19, anti-CD38, and anti-B-cell maturation antigen (BCMA) antibodies have markedly improved survival outcomes, whereas antibody-drug conjugates and bispecific antibodies continue to expand therapeutic possibilities. Besides cancer, complement inhibitors such as eculizumab, ravulizumab, and the recently approved crovalimab have redefined paroxysmal nocturnal hemoglobinuria and atypical hemolytic uremic synd

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… . † PMDA officially approves rituximab for CD20-positive lymphoproliferative disorders under immunosuppression. … Immunodeficiency-Associated CD20-Positive B-Cell Lymphoproliferative Disorders Immunodeficiency-associated CD20-positive B-cell lymphoproliferative disorders represent a distinct category of Epstein–Barr virus (EBV)-driven proliferation arising from impaired immune surveillance, including methotrexate-associated …

Breakthrough COVID-19 in Vaccinated Patients with Haematologic Malignancies-The First Single-Centre Experience from the Czech Republic.

Vaccination is an important tool in the fight against the COVID-19 pandemic in patients with haematologic malignancies. The paper provides an analysis of the course of breakthrough SARS-CoV-2 infection in a group of vaccinated patients with haematological malignancy and a comparison with a historical cohort of 96 non-vaccinated patients with haematologic malignancies and bone marrow failure syndromes (two patients) in the treatment of COVID-19. A severe or critical course of COVID-19 was significantly less frequent in the group of vaccinated patients (10.2% vs. 31.4%, p = 0.003). The need for

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… Consistent with previously published results, malignant lymphoproliferative disorders represent a predominant group of diagnoses in both cohorts [ 5 ]. …

The evaluation and management of monoclonal gammopathy of renal significance and monoclonal gammopathy of neurological significance.

Despite the benign nature of monoclonal gammopathy of undetermined significance (MGUS), mounting data are associating MGUS with the development of organ dysfunction, specifically monoclonal gammopathy of renal significance (MGRS) and monoclonal gammopathy of neurological significance (MGNS), which could be associated with substantial morbidity. Emerging evidence suggests that patients with MGRS and MGNS could benefit from treatments used for myeloma, Waldenström macroglobulinemia, or chronic lymphocytic leukemia, depending on the underlying pathology. However, the treatment of MGRS and MGNS is

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… If the evaluation above is diagnostic of MM, WM, AL amyloidosis, CLL, or other lymphoproliferative disorders, the patient should be managed following the respective algorithm. … Non‐IgM and FLC‐associated MGRS should be managed as per the treatment algorithm for MM unless another lymphoproliferative disorder is confirmed. …

Castleman Disease and Kaposi Sarcoma: A Review of the Literature and a Case Series.

Castleman disease and Kaposi sarcoma (KS) are both associated with infection by human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus (KSHV). This virus plays a critical role in the pathogenesis of both conditions, particularly in immunocompromised individuals, such as those with HIV/AIDS. Multicentric Castleman disease (MCD) generally presents with systemic inflammatory symptoms, lymphadenopathy, and organ dysfunction, while Kaposi sarcoma typically appears as vascular tumors on the skin, with occasional involvement of mucous membranes or internal organs. We prese

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… Castleman disease is a rare heterogeneous group of lymphoproliferative disorders common in HHV-8-positive patients characterized by excessive cytokine release, particularly IL-6, leading to systemic inflammation, cytopenias, and organ dysfunction. … According to the 2022 International Consensus Classification, UCD is no longer included within the category of lymphoproliferative disorders. Instead, MCD, when associated with HHV-8, is classified as an HHV-8-associated lymphoproliferative disorder. …

Sjögren Disease-B Cells at the Brink: From Autoimmunity to Lymphomagenesis and the Rise of Novel B Cell-Targeted Therapies.

Sjögren disease (SjD) is a common systemic autoimmune disorder characterized by inflammation of the exocrine glands, resulting in dryness. Patients frequently exhibit extraglandular manifestations affecting various organ systems. To date, there are no US Food and Drug Administration (FDA)-approved disease-modifying therapies for SjD. In this review, we explore the expanding field of SjD endotyping as a tool to enhance patient stratification, prognostication, and clinical decision-making. SjD endotypes driven by heightened B cell activity are linked to increased lymphoma risk. B cells play a ce