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Association Between Common Variable Immunodeficiency and Pulmonary Amyloidosis: Review.

Background: Common variable immunodeficiency (CVID) is the most frequent symptomatic primary antibody deficiency, associated with recurrent infections, immune dysregulation, and non-infectious complications. Amyloidosis is a rare but severe complication with pulmonary involvement being exceptional. Objective: The aim of this study was to review reported cases of amyloidosis complicating CVID and present a unique case of pulmonary involvement. Methods: A literature research identified observational studies and case reports linking amyloidosis with CVID. Additionally, we describe a patient with

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… Bone marrow biopsy done to rule out multiple myeloma or other lymphoproliferative disorders showed no signs of plasma cell dyscrasia These findings, together with abnormal serum free light chain levels and a deranged kappa:lambda ratio, raised suspicion for AL amyloidosis. …

Ten Наводки на биопсию почек у больных раком.

Ренальная биопсия — это важный диагностический инструмент, который следует учитывать у больных раком, население которого часто страдает почечной дисфункцией, многофакторной и клинической активностью. Точное выявление основных повреждений часто имеет решающее значение для руководства онкологической терапией, предотвращения дальнейшего упадка почечной железы и улучшения общих результатов. Однако проведение биопсии в этой уязвимой группе пациентов требует тщательной оценки процедурных рисков и клинического контекста. Это...10 Брошюра < < Советы > > обеспечивает практическую основу для

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… In lymphoproliferative disorders, renal biopsy may reveal not only MGRS lesions but also paraneoplastic processes such as minimal change disease or TMA as well as direct lymphomatous infiltration. The latter is often underrecognized owing to its subtle presentation. … In addition to cancer types common in the general population, KTRs are particularly susceptible to transplant-associated malignancies, including nonmelanoma skin cancers [ 74 ], post-transplant lymphoproliferative disorders, Kaposi sarcoma, and native kidney renal cell carcinoma [ 75 ]. …

Реналь-лимитированный амилоидный лайт-чаин (Ламбда) Амилоидоз, представленный как Моноклональная Гаммопатия Ренального Значения: доклад по делу.

Моноклональная гаммопатия почечной значимости (MGRS) охватывает почечные заболевания, вызываемые нефротоксичными моноклональными иммуноглобулинами, производимыми небольшими клонами B-клеток или плазменными клонами, которые не отвечают критериям явной гематологической злокачественности. Ренал амилоидный светоцепный (AL) амилоидоз — признанное повреждение в диапазоне MGRS. Мы сообщаем о случае 66-year-old Женщина с нефротическим синдромом и почечной дисфункцией, связанными с моноклональными лямбдами. Биопсия реала подтвердила амилоидоз AL (ламбда), в то время как анализ костного мозга и системна

Bilateral Proptosis in a Case of Recurring Multiple Myeloma: Uncommon Orbital Presentation of Plasmacytoma.

Multiple myeloma is a malignant plasma cell dyscrasia that may invade the orbits in extramedullary presentations. Common manifestations of orbital involvement include unilateral proptosis, injection, chemosis, diplopia, and occasionally pain. Additionally, the soft-tissue tumors associated with multiple myeloma typically cause bony destruction of adjacent structures. However, in certain patients, bilateral proptosis and a lack of bony destruction may occur. In these instances, it is important for physicians to consider patient history, presentation, and progression in order to ensure that a po

Oral complications in hematopoietic stem cell recipients: the role of inflammation.

Hematopoietic stem cell transplantation (HSCT) is widely used as a potentially curative treatment for patients with various hematological malignancies, bone marrow failure syndromes, and congenital immune deficiencies. The prevalence of oral complications in both autologous and allogeneic HSCT recipients remains high, despite advances in transplant medicine and in supportive care. Frequently encountered oral complications include mucositis, infections, oral dryness, taste changes, and graft versus host disease in allogeneic HSCT. Oral complications are associated with substantial morbidity and

Acute Colonic Perforation with Septic Shock Secondary to Disseminated Histoplasmosis in an Autologous Bone Marrow Transplant Recipient.

Histoplasma capsulatum is an opportunistic pathogen which can lead to a wide variety of clinical presentations in the immunocompromised host. Post-transplant histoplasmosis in hematopoietic cell transplant recipients is exceedingly rare, with an incidence of <1%. We present a case of acute caecal perforation resulting from disseminated histoplasmosis in a patient who had undergone autologous bone marrow transplant for plasma cell dyscrasia. This is a 71-year-old patient who initially presented due to progressive weakness associated with shortness of breath.

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… Patients with plasma cell disorders, lymphoproliferative disorders, and myelodysplastic syndrome pursue hematopoietic stem-cell transplants (HCT) post-chemotherapy or as adjuvant therapy for prolonged remission and improved survival. 5 Although HCT is pivotal in certain cases for transplant-eligible …

Anemia and Iron Deficiency in Cancer Patients: Role of Iron Replacement Therapy.

Anemia in cancer patients is quite common, with remarkable negative impacts on quality of life and overall prognosis. The pathogenesis is complex and typically multifactorial, with iron deficiency (ID) often being a major and potentially treatable contributor. In turn, ID in cancer patients can be due to multiple concurring mechanisms, including bleeding (e.g., in gastrointestinal cancers or after surgery), malnutrition, medications, and hepcidin-driven iron sequestration into macrophages with subsequent iron-restricted erythropoiesis. Indeed, either absolute or functional iron deficiency (AID

Состояние питания, способность к антиоксидантам в рационе питания и разнообразие рациона питания у пациентов с недавно диагностированными гематологическими отклонениями: тематическое исследование.

ППОЗ: Состояние питания и способность к антиоксидантам в рационе питания являются важными факторами, определяющими клиническую устойчивость к раку; однако данные о гематологических заболеваниях по-прежнему ограничены. Мы поставили перед собой цель сравнить состояние питания, общий потенциал рациона питания с антиоксидантами (DTAC) и разнообразие рациона питания между пациентами с недавно диагностированными гематологическими отклонениями и здоровыми средствами контроля. МЕТОДЫ: Это тематическое исследование включало 34 больные с недавно диагностированными гематологическими заболеваниями12 лимфо

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… Cancemi G, Cicero N, Allegra A, Gangemi S (2023) Effect of diet and oxidative stress in the pathogenesis of lymphoproliferative disorders. Antioxidants (Basel) 12(9):1674. 10.3390/antiox12091674 11. …

Воздействие витамина D на герпесвирусные инфекции.

Помимо своей классической роли в регулировании метаболизма кальция и фосфата, витамин D также оказывает значительное влияние на модуляцию иммунитета. Витамин D регулирует иммунную реакцию, переходя от воспламенительного состояния к более толерогенному one путем увеличения выброса антивоспалительных цитокинов при одновременном ослаблении регулирования проинфламаторных цитокинов. Таким образом, низкий уровень витамина D связан с повышенным риском возникновения аутоиммунных заболеваний, таких, как множественный склероз и тип 1 диабет. Кроме того, этот прохормон также способствует высвобождению хо

Treosulfan-Based Conditioning Regimen Prior to Allogeneic Stem Cell Transplantation: Long-Term Results From a Phase 2 Clinical Trial.

INTRODUCTION: Reducing toxicities while preserving efficacy in allogeneic stem cell transplant (allo-HCT) remains a particularly challenging problem. Different strategies to enhance the antitumor activity without increasing early and late adverse toxicities of the conditioning regimens have been investigated. METHODS: The aim of "AlloTreo" prospective phase 2 clinical trial was to evaluate the efficacy and safety of a conditioning regimen based on Treosulfan (42 g/m2) and fludarabine (https://clinicaltrials.gov/ct2/show/NCT00598624). We enrolled 108 patients with hematological diseases who re

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… A single dose of Rituximab 500 mg was added in these cases considering the high risk of posttransplant lymphoproliferative disorders related to the in vivo T-cell depletion. Supportive care and antimicrobial prophylaxis followed institutional guidelines. …

Genetic Risk Surveillance for Invasive Aspergillosis in Hematology Patients: A Prospective Observational Study.

IntroductionThe association between genetic background and the risk of invasive aspergillosis (IA) has not been addressed in Thailand. We conducted genetic risk surveillance for IA among Thai hematologic patients.MethodsWe conducted a prospective observational cohort study including moderate- to high-risk hematology patients at Ramathibodi Hospital. IA occurrence, relevant clinical data, and genetic analyses were assessed. Odds ratios (ORs) of IA were assessed for the presence of the selected single nucleotide polymorphism genotype using logistic regression.ResultsA total of 357 patients were

Navigating the Diagnostic Maze: A Case Report of Immunoglobulin G4-Related Disease.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory condition characterized by significant infiltration of immunoglobulin G4 (IgG4)-positive plasma cells within affected tissues, with or without elevated serum IgG4 levels. The prevalence of IgG4-RD remains largely undetermined due to diagnostic challenges, as the condition is frequently unrecognized or misdiagnosed. This report describes a case of a 63-year-old man who was ultimately diagnosed with this rare condition after an extensive two-year period of elusive symptoms. Initially presenting with intermittent body pai

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… These conditions encompass a wide spectrum of diseases, including malignancies, lymphoproliferative disorders, antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis, sarcoidosis, Sjögren’s syndrome, and Castleman’s disease, among others [ 14 ]. …

Recommendations for the management of the haematological and onco-haematological aspects of Gaucher disease.

Current knowledge of the haematological and onco-haematological complications of type 1 Gaucher disease has been reviewed with the aim of identifying best clinical practice for treatment and disease management. It was concluded that: (i) Awareness of typical patterns of cytopenia can help clinicians distinguish haematological co-morbidities. (ii) Red blood cell studies and complete iron metabolism evaluation at baseline are recommended. (iii) Haemoglobin levels defining anaemia should be raised and used in Gaucher disease treatment and monitoring. (iv) Surgeons should be aware of potential ble

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… Increased white blood cell counts may also occur in Gaucher patients after splenectomy and may signal other pathologies, such as the development of lymphoproliferative disorders. …

Biological Age, Aging Clocks, and the Interplay with Lymphoid Neoplasms: Mechanisms and Clinical Frontiers.

Lymphoid neoplasms (LN), a diverse group of malignancies arising from lymphocytes, exhibit a striking increase in incidence with chronological age, suggesting a deep connection with the aging process. While chronological age remains a primary risk factor, the concept of biological age, reflecting an individual's physiological state and susceptibility to age-related diseases, offers a more nuanced understanding of this relationship. Aging clocks, particularly epigenetic clocks based on DNA methylation, provide quantitative measures of biological age and have revealed associations between accele

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… Oyama T ; Ichimura K ; Suzuki R ; Suzumiya J ; Ohshima K ; Yatabe Y ; Yokoi T ; Kojima M ; Kamiya Y ; Taji H ; Senile EBV+ b-cell lymphoproliferative disorders: A clinicopathologic study of 22 patients . Am. J. Surg. Pathol 2003 , 27 , 16 – 26 . 12502924 10.1097/00000478-200301000-00003 52. … Oyama T ; Yamamoto K ; Asano N ; Oshiro A ; Suzuki R ; Kagami Y ; Morishima Y ; Takeuchi K ; Izumo T ; Mori S ; Age-related EBV-associated b-cell lymphoproliferative disorders constitute a distinct clinicopathologic group: A study of 96 patients . Clin. …

Celiac Disease Emerging After ASCT in Multiple Myeloma: A Rare Diagnostic Intersection and Literature Review.

BackgroundAutologous stem cell transplantation (ASCT) is a standard component of therapy for eligible patients with multiple myeloma. Persistent gastrointestinal symptoms after ASCT are commonly attributed to infection, medication-related toxicity, or disease relapse, whereas autoimmune enteropathies such as celiac disease are rarely considered.Case presentationWe report a 53-year-old woman with lambda light-chain multiple myeloma who achieved complete remission after bortezomib, lenalidomide, and dexamethasone induction followed by ASCT and lenalidomide maintenance. Four months after transpla

Overview of Dual-Acting Drug Methotrexate in Different Neurological Diseases, Autoimmune Pathologies and Cancers.

Methotrexate, a structural analogue of folic acid, is one of the most effective and extensively used drugs for treating many kinds of cancer or severe and resistant forms of autoimmune diseases. In this paper, we take an overview of the present state of knowledge with regards to complex mechanisms of methotrexate action and its applications as immunosuppressive drug or chemotherapeutic agent in oncological combination therapy. In addition, the issue of the potential benefits of methotrexate in the development of neurological disorders in Alzheimer's disease or myasthenia gravis will be discuss

What the Intensivist Needs to Know About Hematopoietic Stem Cell Transplantation?

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A lost race against time-a rare case of AL amyloidosis.

INTRODUCTION: Amyloidosis is a rare multi-system disease that can affect the heart. Recent advances have been made in finding successful treatments for these patients. Nonetheless, cardiac amyloidosis continues to carry high morbidity and mortality. CASE PRESENTATION: This case refers to a 52-year-old man presenting with signs and symptoms of heart failure. Electrocardiogram showed q waves in V1-V3 and T wave inversion in the inferior and lateral leads. Echocardiography revealed moderate concentric left ventricular hypertrophy, with preserved biventricular systolic function and no other chang

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… The risk of progression to MM or other lymphoproliferative disorders in MGUS without cardiac involvement is low, with an annual risk of approximately 1% [ 13 ]. …

Systemic Consequences of Inflammatory Bowel Disease Beyond Immune-Mediated Manifestations.

Inflammatory bowel disease (IBD) management traditionally focuses on intestinal inflammation, yet extraintestinal manifestations can substantially impair patient quality of life. In this perspective, we emphasize the broad systemic impact of IBD-from highly prevalent conditions such as anemia, metabolic dysfunction-associated steatotic liver disease, or fatigue to rare but severe complications like interstitial lung disease and drug-induced glomerulonephritis. We review underlying mechanisms linking gut inflammation to distant organs, including immune dysregulation, microbial translocation, an

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… Lymphoproliferative Disorders: Patients with IBD face increased lymphoma risk, particularly with immunosuppressive therapy. … Lymphoproliferative Disorders in Inflammatory Bowel Disease Patients: Is It the Drugs or the Disease. GE Port. J. Gastroenterol. 2018;25:175–178. doi: 10.1159/000484440. 44. Lagrange J., Lacolley P., Wahl D., Peyrin-Biroulet L., Regnault V. …

An unusual cause of gross hematuria and renal dysfunction in a young male.

Monoclonal immunoglobulin deposition disease (MIDD) is an uncommon disease with a peak incidence between the 5(th) and 6(th) decades of life. It is characterized by non-fibrillar, Congo red negative deposition of monoclonal immunoglobulins in various organs, including in the kidneys. MIDD can be of three types depending on the composition of the deposits, and includes light chain deposition disease (LCDD), heavy chain deposition disease and light and heavy chain deposition disease, of which LCDD is the most common. Renal involvement is a universal finding in MIDD, and is in the form of renal i

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… Of these three types, LCDD is the most common, comprising about 80% of the cases.[ 1 ] LCDD is usually seen in association with multiple myeloma or other lymphoproliferative disorders, and presents with renal involvement in the form of proteinuria and renal insufficiency.[ 2 ] Microscopic hematuria is …