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Delayed Presentation of Thymoma-Related Aplastic Anemia: An Unusual Presentation of a Rare Complication.

A 68-year-old Caucasian man presented with gross hematuria and oral mucosal bleeding. The patient was known to have an anterior mediastinal mass, highly suspicious for thymoma, which was incidentally identified on imaging, 8 years prior. The patient then declined treatment and was lost to follow-up. On presentation, imaging re-demonstrated the anterior mediastinal mass and the patient was found to have profound pancytopenia. Bone marrow biopsy confirmed the diagnosis of aplastic anemia (AA). Despite optimal treatment, the patient expired on day 9 of admission. In this case we report an unusual

Plasmapheresis leading to remission of refractory nephrotic syndrome due to fibrillary glomerulonephritis: a case report.

IntroductionFibrillary glomerulonephritis (FibGN) is characterized by extracellular deposition of Congo red-negative microfibrils within the glomerular mesangium and leads to gross proteinuria or nephrotic syndrome. After diagnosis of FibGN, end-stage renal disease occurs within four years in 50% of patients.Case presentationA 36-year-old Caucasian woman with proteinuria and intermittent nephrotic syndrome due to FibGN intermittently received immunosuppressive therapies, including glucocorticoids, mycophenolate mofetil, and rituximab, for 10 years. However, disease remission was not achieved a

AL type renal amyloidosis with cardiac involvement: A case report and literature review.

RATIONALE: Systemic amyloidosis refers to a spectrum of diseases characterized by extracellular deposition of amyloids, with commonrenal involvement; however, simultaneous involvement of cardiac amyloidosis remains rare. The purpose of this report was to enhance the understanding of the diagnosis and treatment of light chain (AL)-type renal amyloidosis with cardiovascular involvement, reduce misdiagnosis and missed diagnosis, and enable timely and effective treatment for such patients. PATIENT CONCERNS: A 74-year-old male patient was admitted to our hospital because of recurrent edema for ove

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… Humoral immunity: immunoglobulin G 10.76 g/L, immunoglobulin A 1.9 g/L, immunoglobulin M 0.86 g/L, complement C3 0.94 g/L, complement C4 0.26 g/L, complement C1q 0.17 g/L. …

Beneath the surface in autoimmune hemolytic anemia: pathogenetic networks, therapeutic advancements and open questions.

In recent years, the pathophysiologic framework of autoimmune hemolytic anemias (AIHAs) has evolved considerably, extending beyond the simplistic paradigm of antibody-mediated red blood cell (RBC) destruction, which is now recognized as a downstream consequence of a broader immune dysregulation. AIHA is fundamentally orchestrated by a complex interplay between innate and adaptive immune components, including autoreactive B and T lymphocytes, macrophages, and the reticuloendothelial system. Central to disease pathogenesis are two interrelated mechanisms: clonal B-cell expansion with autoantibod

Autoimmune Hemolytic Anemias: Challenges in Diagnosis and Therapy.

BackgroundAutoimmune hemolytic anemia (AIHA) is a rare disease due to increased destruction of erythrocytes by autoantibodies, with or without complement activation.SummaryAIHA is usually classified in warm AIHA (wAIHA) and cold agglutinin disease (CAD), based on isotype and thermal amplitude of the autoantibody. The direct antiglobulin test (DAT) or Coombs test is the cornerstone of AIHA diagnosis, as it is positive with anti-IgG in wAIHA, and with anti-C3d/IgM antisera plus high titer cold agglutinins in CAD. Therapy is quite different, as steroids and rituximab are effective in the former,

Progressive renal failure in a patient with lung adenocarcinoma.

We present an interesting case of a young female smoker who was hospitalized for shortness of breath and acute renal insufficiency (serum creatinine = 2.8 mg/dL). Few weeks prior to admission, she was discovered to have a right lung mass, and a biopsy confirmed lung adenocarcinoma. Her work-up revealed an unremarkable urinalysis quantitatively and on microscopic analysis. Renal ultrasound demonstrated enlarged bilateral unobstructed kidneys, while a nuclear scan showed increased activity in both kidneys. Renal biopsy established the diagnosis of diffuse metastatic infiltration of both kidneys

Sarcoidosis With Muscular and Peripheral Nervous System Involvement: An Atypical Presentation of a Rare Disease.

Neuromuscular sarcoidosis is uncommon and may represent a diagnostic challenge, particularly in the absence of more typical sarcoidosis manifestations such as pulmonary involvement. We report a case of a 71-year-old male presenting with constitutional symptoms, weight loss, progressive gait impairment, hypercalcemia, and acute kidney injury. Initial evaluation excluded malignancy, active infection, and monoclonal gammopathy. Advanced imaging showed diffuse muscular involvement, and muscle biopsy confirmed non-caseating granulomatous myositis, establishing the diagnosis of muscular sarcoidosis.

Recent advances in the management of systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease presenting highly heterogeneous clinical manifestations and multi-systemic involvement. Patients are susceptible to relapse- and remission, thus making management challenging. Moreover, a considerable number of side effects may occur with conventional therapies; therefore, there is clearly a need for new therapeutic strategies. Since the pathogenesis of SLE is highly complex, it is far from being fully understood. However, greater understanding of the pathways and of the cellular and molecular mediators involved in SLE is being

Recent advances in renal transplantation: antibody-mediated rejection takes center stage.

Overlooked for decades, antibodies have taken center stage in renal transplantation and are now widely recognized as the first cause of allograft failure. Diagnosis of antibody-mediated rejection has considerably improved with identification of antibody-mediated lesions in graft biopsies and advances made in the detection of circulating donor-specific antibodies. Unfortunately, this progress has not yet translated into better outcomes for patients. Indeed, in the absence of a drug able to suppress antibody generation by plasma cells, available therapies can only slow down graft destruction. Th

Эффективность транскраниальной электрической стимуляции в сочетании с двухзадачной подготовкой по вопросам инсульта, слабого когнитивного расстройства и болезни Паркинсона: систематический обзор и метаанализ случайных контролируемых испытаний.

АИМ: В рамках этого систематического обзора и метаанализа мы провели оценку эффективности транскраниальной непосредственной стимуляции тока (tDCS) и транскраниальной переменной тока (tACS) или транскраниальной случайной стимуляции шума (TRNS) в сочетании с двойной целевой подготовкой (DTT) по вопросам физических и когнитивных функций у взрослых с незначительными когнитивными нарушениями (MCI), болезнью Паркинсона (PD) и инсульта. МЕТОД: Мы провели систематический поиск баз данных сети " Web of Science " , " MedLINE " , " Cochrane Bibrary " , " PubMed " и

Renal Thrombotic Microangiopathy Associated with the Use of Bortezomib in a Patient with Multiple Myeloma.

Bortezomib is a first-generation proteasome inhibitor used in the treatment of multiple myeloma (MM). A few reports have linked bortezomib exposure with the development of thrombotic microangiopathy (TMA). We describe a case of biopsy-proven renal thrombotic microangiopathy associated with the use of bortezomib in a 51-year-old man with IgG lambda MM. To our knowledge, this is the first biopsy-proven case. In addition, reexposure to bortezomib 18 months later was associated with recurrence of TMA. This supports a possible causal role of bortezomib. The exact mechanisms remain to be elucidated.

Назад ходьба как знак мобильности и инвалидности в мультипликационном склерозе: перекрестный анализ.

Справочная информация: Недостатки мобильности при множественном склерозе (МС) существенно влияют на качество жизни. В ходе этого исследования была проведена оценка клинической полезности и чувствительности испытания 25-футовой прогулки в обратном направлении (B-T25FW) и его связей с ключевыми клиническими мерами в MS. Методы: В двух центрах было проведено перекрестное исследование 129 пациентов амбулаторного лечения. Была проведена оценка инвалидности (расширенная шкала инвалидности, EDS), когниции (испытание на симболическую дигитацию, SMT), ручной декситерит (испытание на нижний столб, 9HPT)

Extraglandular Manifestations of Sjögren’s Syndrome (SS): Dermatologic, Arthritic, Endocrine, Pulmonary, Cardiovascular, Gastroenterology, Renal, Urology, and Gynecologic Manifestations

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… A low complement C4 (either as a C4 null patient or due to complement consumption) is common, so disproportionate decreases in C4 levels are commonly found. In contrast to lupus glomerulonephritis, membranoproliferative glomerulonephritis due to cryoglobulinemia is usually a “later” presentation. …

Secondary renal amyloidosis due to primary Sjogren's syndrome: a case report.

UNLABELLED: Amyloidosis is a rare disorder characterized by the deposition of abnormal proteins in extracellular tissues, resulting in the dysfunction of vital organs and, eventually, death. The occurrence of amyloidosis due to primary Sjogren's syndrome (pSS) is a rare finding. This study describes a rare case of pSS complicated by amyloid-associated amyloidosis. CASE PRESENTATION: A 35-year-old male was diagnosed with nephrotic syndrome and secondary amyloidosis caused by pSS. He had microscopic hematuria, a creatinine level of 6.59 mg/dl, and an elevated erythrocyte sedimentation rate of 1

Multiple myeloma presenting as acute tubulointerstitial nephritis.

BACKGROUND: Acute tubulointerstitial nephritis (ATIN) is a very rare paraneoplastic manifestation in patients with multiple myeloma (MM). It is an uncommon pattern of renal disease in such patients. CASE PRESENTATION: We report a case of an 82-year-old male who was admitted with acute kidney injury. Renal biopsy showed typical findings of light chain-associated ATIN with scattered inflammatory cells in the interstitium and associated active tubulitis. No other common manifestations of MM were present at the time of presentation, including hypercalcemia, hyperuricemia, proteinuria, bone pain o

Иммунорегулирующий амфипатический песптид, полученный из молекулы защиты фасиолы гепатики (FhHDM-1.C2), демонстрирует мощную биотерапевтическую активность в мусриновой модели множественного склероза.

Молекулярные защитные молекулы (HDM) — семейство иммунных регуляционных пептидов, которые выражаются исключительно триматодными червями. Ранее мы продемонстрировали, что в vivo FhHDM-1, архетипный член HDM, регулировала макрофагическую реакцию на подстрекательские лиганды, тем самым облегчая процесс повреждения иммунной ткани в нескольких мусриновых моделях воспалительных заболеваний. Соответственно, мы исходили из того, что понимание взаимосвязи между структурой и функцией НДМ облегчило бы идентификацию минимального биоактивного пептида, который представлял бы собой более синтезируемый, эконо

Adeno-associated virus as a delivery vector for gene therapy of human diseases.

Adeno-associated virus (AAV) has emerged as a pivotal delivery tool in clinical gene therapy owing to its minimal pathogenicity and ability to establish long-term gene expression in different tissues. Recombinant AAV (rAAV) has been engineered for enhanced specificity and developed as a tool for treating various diseases. However, as rAAV is being more widely used as a therapy, the increased demand has created challenges for the existing manufacturing methods. Seven rAAV-based gene therapy products have received regulatory approval, but there continue to be concerns about safely using high-dos

Proteasome inhibitors related thrombotic microangiopathy: a systematic and comprehensive review.

Proteasome inhibitors (PIs) are crucial in treating multiple myeloma but carry a risk of thrombotic microangiopathy (TMA), especially with carfilzomib use. This systematic review includes 44 studies with 115 cases of PI-induced TMA, where carfilzomib was implicated in 101 cases. Treatment approaches varied: 28 patients received supportive care, 43 underwent therapeutic plasma exchange (TPE), 9 were treated exclusively with eculizumab (ECU), and 13 received both TPE and ECU. Notably, eculizumab significantly improved outcomes for patients unresponsive to initial TPE, achieving complete remissio

The Scope of Kidney Affection in Monoclonal Gammopathies at All Levels of Clinical Significance.

Multiple myeloma (MM) is one of the most important clonal malignant plasma cell disorders and renal involvement is associated with poor prognosis. Although there are several reasons for renal impairment in MM, the main cause is the toxic effects of monoclonal proteins. Although cast nephropathy is the best known and unchallenged diagnosis for hematologists and pathologists, the renal effects of monoclonal gammopathy can be various. Monoclonal gammopathy of renal significance was proposed by the International Kidney and Monoclonal Gammopathy Research Group for renal lesions in monoclonal gammop

Kidney failure in the course of focal segmental glomerulonephritis in a patient after alloHSCT - a case study and review of the literature.

Nephrotic syndrome is a rare complication of allogeneic haematopoietic stem cell transplantation (alloHSCT). Its pathogenesis is not fully understood. Membranous nephropathy and minimal change disease are the most common causes of nephrotic syndrome. Nephrotic syndrome occurs more frequently in people with chronic graft-versus-host disease (GvHD). Glucocorticosteroids and cyclosporine are the most common treatments, but their use is associated with the risk of side effects and variable responses. We present a case of a patient after alloHSCT with advanced GvHD who developed nephrotic syndrome