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Прогресс в лечении множественной миеломы.

Множественная миелома (ММ) является вторым по распространенности гематологической злокачественности в мире. Несмотря на свою репутацию агрессивного и смертельного заболевания, недавние улучшения в лечении значительно улучшили результаты лечения. За последнее десятилетие появились инновационные методы лечения с меньшим числом побочных эффектов, включая протеиновые ингибиторы (например, борцезомиб, карфилзомиб, ixazomib), моноколональные антитела (например, даратумумаб, изатуксимаб, элотузумаб), иммуномуляторы (например, талидомид, линалидомид, помалидомид) и кортикостероиды (например, дексамета

Mucocutaneous Findings in Hematolymphoid Neoplasms: An Observational Study.

BackgroundCutaneous manifestations of hematological neoplasms can be divided into three broad categories - direct infiltration, paraneoplastic conditions, and those due to the treatment of hematological cancers.ObjectivesTo study the frequency and patterns of mucocutaneous manifestations in patients with hematolymphoid neoplasms and those due to chemotherapy.Materials and methodsThis was an observational study done with 172 patients. Categorization of mucocutaneous manifestations was done into malignancy-associated and chemotherapeutic drugs-associated and data was analyzed.ResultsOut of a tot

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… Introduction Cutaneous manifestations of hematological neoplasms can be divided into three broad categories:[ 1 ] (a) Direct infiltration of skin with malignant cells or their products (e.g., paraproteins) (b) Paraneoplastic conditions or dermatological syndromes associated with underlying malignancy …

Concurrent chronic lymphocytic leukemia and primary hyperparathyroidism in a mule.

A 26-year-old mule gelding was evaluated for chronic weight loss and decreased appetite. The mule had been losing weight and intermittently hypophagic for approximately 7 months. Laboratory analysis of whole blood and plasma identified severe total hypercalcemia, marked hypophosphatemia, markedly increased parathyroid hormone concentration, and marked lymphocytosis. A sestimibi scan intended to identify parathyroid gland tissue was nondiagnostic. Results of flow cytometry and PCR for antigen receptor rearrangement (PARR) were consistent with a B cell lymphoproliferative disorder, likely chroni

Outcomes of VDPACE with an immunomodulatory agent as a salvage therapy in relapsed/refractory multiple myeloma with extramedullary disease.

Extramedullary disease (EMD) is an aggressive form of multiple myeloma (MM). Confirming the presence of plasma cells outside the bone marrow makes the diagnosis of EMD. There is no clear consensus on the management of EMD in MM, and this entity continues to remain an unmet need. Rapidly controlling EMD to prevent end-organ damage is a priority. Retrospectively, we reviewed our database for patients with EMD that received treatment with bortezomib, dexamethasone, cisplatin, doxorubicin, cyclophosphamide, etoposide (VDPACE) plus an immune modulator (IMiD) regimen. We identified 21 patients with

C-Reactive Protein: Pathophysiology, Diagnosis, False Test Results and a Novel Diagnostic Algorithm for Clinicians.

The current literature provides a body of evidence on C-Reactive Protein (CRP) and its potential role in inflammation. However, most pieces of evidence are sparse and controversial. This critical state-of-the-art monography provides all the crucial data on the potential biochemical properties of the protein, along with further evidence on its potential pathobiology, both for its pentameric and monomeric forms, including information for its ligands as well as the possible function of autoantibodies against the protein. Furthermore, the current evidence on its potential utility as a biomarker of

Case report: A case of proliferative glomerulonephritis with monoclonal kappa-light chain deposits treated with daratumumab combination therapy.

INTRODUCTION: Proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID) is a chronic glomerular disease caused by monoclonal gammopathy. IgG (mainly IgG3) is the most commonly involved isotype of PGNMID. Here we illustrated a novel medication regimen for the rare variant of PGNMID with deposition of monoclonal immunoglobulin light chain only (PGNMID-LC). Daratumumab has been proved effective in the treatment of plasma cell myeloma while its effect for PGNMID-LC has rarely been reported. METHODS: A daratumumab combination therapy (D-VCd regimen, specifically are daratum

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… However, the challenge with clone-directed therapy is that circulating paraproteins and pathogenic clones are often undetectable, making treatment selection difficult. …

The Synthetic Collagen-Binding Peptide NIPEP-OSS Delays Mouse Myeloma Progression.

Multiple myeloma (MM) is the second most common hematological malignancy. It is a clonal B-cell disorder characterized by the proliferation of malignant plasma cells in the bone marrow, the presence of monoclonal serum immunoglobulin, and osteolytic lesions. An increasing amount of evidence shows that the interactions of MM cells and the bone microenvironment play a significant role, suggesting that these interactions may be good targets for therapy. The osteopontin-derived collagen-binding motif-bearing peptide NIPEP-OSS stimulates biomineralization and enhances bone remodeling dynamics. Due

Cytotoxic Effect on Human Myeloma Cells and Leukemic Cells by the Agaricus blazei Murill Based Mushroom Extract, Andosan™.

Agaricus blazei Murill is an edible mushroom of the Basidiomycetes family, which has been found to contain a number of compounds with antitumor properties, such as proteoglycans and ergosterol. In the present investigation, we show that the commercial mushroom product Andosan, which contains 82.4% Agaricus blazei Murill, together with medicinal mushrooms Hericium erinaceus (14.7%) and Grifola frondosa (2.9%), has a cytotoxic effect on primary myeloma cells, other myeloma cell lines, and leukemia cell lines in vitro. Although the exact content and hence the mechanisms of action of the Andosan e

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… The main clinical features are lytic lesions caused by local growth of malignant plasma cells in bones and renal insufficiency due to deposition of paraproteins produced by myeloma cells [ 24 ]. Most patients respond well to initial therapy, but relapse occurs in virtually all cases [ 25 ]. …

Improvement in renal function and its impact on survival in patients with newly diagnosed multiple myeloma.

Renal impairment (RI) is seen in over a quarter of patients with newly diagnosed multiple myeloma (NDMM). It is not clear if reversal of RI improves the outcome to that expected for NDMM patients without RI. We evaluated 1135 consecutive patients with NDMM seen at the Mayo Clinic between January 2003 and December 2012. RI was defined as having a creatinine clearance (CrCl) <40ml/min. The median overall survival (OS) for patients with RI at diagnosis receiving and not receiving novel agent induction therapy was not reached vs 46 months (P<0.001). The median OS for patients with CrCl ⩾40 ml/min

Evaluating Therapeutic Plasma Exchange in Pediatric Acute Disseminated Encephalomyelitis: A Comprehensive Review.

Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune disorder characterized by brain and spinal cord inflammation. In pediatric patients, ADEM presents unique challenges due to its potential for rapid progression and long-term neurological sequelae. Therapeutic plasma exchange (TPE) has emerged as a potential treatment option by targeting the underlying autoimmune process and modulating the inflammatory response. This comprehensive review evaluates the role of TPE in pediatric ADEM, synthesizing evidence from clinical studies and providing insights into its efficacy, safety, and po

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… Firstly, TPE facilitates the removal of pathological substances, such as autoantibodies, immune complexes, and monoclonal paraproteins, from the plasma, all of which contribute to disease pathogenesis [ 15 ]. …

Multiple myeloma in Niger Delta, Nigeria: complications and the outcome of palliative interventions.

BACKGROUND: Multiple myeloma (MM) is one of the hematological malignancies that require palliative care. This is because of the life-threatening nature and the suffering associated with the illness. The aim of this study is to bring to the fore the complications experienced by people living with MM in the Niger-Delta region of Nigeria and the outcome of various palliative interventions. METHODS: This was a 10-year multi-center retrospective study of 26 patients diagnosed and managed in three major centers in the Niger-Delta region of Nigeria from January 2003 to December 2012. Information on

Minimal Residual Disease in Myeloma: Application for Clinical Care and New Drug Registration.

The development of novel agents has transformed the treatment paradigm for multiple myeloma, with minimal residual disease (MRD) negativity now achievable across the entire disease spectrum. Bone marrow-based technologies to assess MRD, including approaches using next-generation flow and next-generation sequencing, have provided real-time clinical tools for the sensitive detection and monitoring of MRD in patients with multiple myeloma. Complementary liquid biopsy-based assays are now quickly progressing with some, such as mass spectrometry methods, being very close to clinical use, while othe

CAR T therapies in multiple myeloma: unleashing the future.

In recent years, the field of cancer treatment has witnessed remarkable breakthroughs that have revolutionized the landscape of care for cancer patients. While traditional pillars such as surgery, chemotherapy, and radiation therapy have long been available, a cutting-edge therapeutic approach called CAR T-cell therapy has emerged as a game-changer in treating multiple myeloma (MM). This novel treatment method complements options like autologous stem cell transplants and immunomodulatory medications, such as proteasome inhibitors, by utilizing protein complexes or anti-CD38 antibodies with pot

Доказательства для мониторинга динамики минимальных остаточных заболеваний с целью руководства клинической практикой у пациентов с множественной миеломой.

Множественная миелома (ММ) — разнородный рак, который остается неизлечимым. После лечения наличие остаточных клоновых клеток миэломы или минимального остаточного заболевания (МВД) приводит к рецидиву (воскрещению) опухоли и неисправности лечения (рекреторное заболевание). Твердые клинические данные свидетельствуют о том, что MRD является суррогатным показателем выживания у пациентов с MM и имеет важное прогнозируемое значение для лечения заболеваний. Статус MRD представляет собой динамические изменения, а продольные изменения в статусе MRD имеют различные клинические последствия. Например, уст

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… 2017–2021; 75% of pts) or NGF + NGS (from 2021 onwards; 25% of pts) [ 36 ] d Pts had maintained ≥ VGPR for ≥ 6 months and achieved CR + MRD− prior to starting a TFI (owing to AEs or at physician discretion) following a deep treatment response; TFR was defined as the time from the start of treatment to paraprotein … Mass spectrometry (MS) is another form of peripheral blood-based MRD testing that can identify plasma cell clones through its high sensitivity to the mass–charge ratio of secreted paraproteins [ 66 – 68 ]. …

Brazilian Academy of Neurology recommendations for diagnosis, management, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired autoimmune disorder that leads to progressive motor and sensory impairment, resulting in significant morbidity. While the incidence rates vary, CIDP remains a challenging condition requiring a standardized and optimized approach to diagnosis and management. In Brazil, a middle-income country with substantial regional disparities in healthcare access, the availability of specialized neuromuscular centers is uneven, creating obstacles to timely and effective treatment. To address these challenges, the Brazilian Acade

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… Table 7 Differential diagnosis of CIDP according to clinical approach Abbreviations: CANDA, chronic ataxic neuropathy and disialosyl antibodies; CANOMAD, chronic ataxic neuropathy, ophthalmoplegia, immunoglobulin M paraprotein, cold agglutinins, and disialosyl antibodies; CASPR, contactin-associated … be related to underlying hematological malignancy, and are associated with a relative risk of 5.9% (95%CI: 1.2–28.4) of developing CIDP. 65 All patients with slowed CV and suspected acquired neuropathy need to be investigated for paraproteinemic neuropathy. 47 Both immunoglobulin M (IgM) and non-IgM paraproteins …

The Onco-Nephrology Field: The Role of Personalized Chemotherapy to Prevent Kidney Damage.

In recent years, the onco-nephrology field has acquired a relevant role in internal medicine due to the growing number of cases of renal dysfunction that have been observed in cancer patients. This clinical complication can be induced by the tumor itself (for example, due to obstructive phenomena affecting the excretory tract or by neoplastic dissemination) or by chemotherapy, as it is potentially nephrotoxic. Kidney damage can manifest as acute kidney injury or represent a worsening of pre-existing chronic kidney disease. In cancer patients, physicians should try to set preventive strategies

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… the (i) metabolic alterations associated with cancer (such as tumor lysis syndrome-TLS, uric acid nephropathy and hypercalcemia), (ii) the hematopoietic stem-cell transplantation (HSCT) that is often complicated by AKI, (iii) the use of nephrotoxic chemotherapeutic agents and (iv) tissue deposits of paraproteins … In dysproteinaemias, the mechanisms responsible for AKI are the precipitation, the aggregation or the misfolding of the paraprotein in different kidney sites and/or the hypercalcemia [ 2 ]. …

Доклад о деле: модификация пульсодоза пелфаланской терапии для клинической паллиэктальной плазматомы с предполагаемым распространением печени после хирургической эксцизии.

У старшего мальтийского пса был диагностирован одинокий ректальный экстрамеуллярный плазмоцитома, который был полностью акцизирован чистыми окраинами. 5 Месяцами позже были выявлены опухоли многофуакальной печени в плазменных клетках без признаков множественной миэломы. Системная химиотерапия была начата с использованием протокола пульсовой дозы в сочетании с преднизоном. В связи с периодическим ограничением доз миэлосоппрессией протокол лечения постепенно модифицировался путем сокращения продолжительности управления мелфаланом в рамках фиксированного цикла лечения. Модифицированный протокол б

Successful Treatment of Relapsed/Refractory Extramedullary Multiple Myeloma With Anti-BCMA CAR-T Cell Therapy Followed by Haploidentical Hematopoietic Stem Cell Transplantation: A Case Report and a Review of the Contemporary Literature.

Extramedullary multiple myeloma (EMM) is an aggressive sub-entity of multiple myeloma (MM). Despite an excellent improvement in survival for most patients with MM over recent decades, the overall survival (OS) of patients with EMM was usually not longer than 3 years. Standard treatment for patients with EMM has not been established, and their management is particularly challenging. We presented a heavily pretreated young patient with relapsed EMM and refractoriness to a proteasome inhibitor (PI; bortezomib), a next-generation PI (ixazomib), immunomodulatory drugs (IMiDs; lenalidomide), autolog

Characterization, outcome and identification of prognostic factors for patients with systemic immunoglobulin lightchain amyloidosis requiring dialysis prior to initial anticlonal therapy.

AL amyloidosis is a serious disease characterized by the deposition of immunoglobulin light chains in multiple organs. Renal involvement occurs in up to 70% of patients, but only a minority require dialysis before initiating anti-clonal treatment. Understanding the occurrence of end-stage organ damage is crucial to pave the way for reversing deposition. Currently, there is no detailed analysis available for this rare patient subgroup. We conducted a systematic search across three amyloidosis centers and characterized 68 biopsy-proven AL amyloidosis patients who required dialysis prior to initi

A Combined Cyto- and Histopathological Diagnostic Approach Reduces Time to Diagnosis and Time to Therapy in First Manifestation of Metastatic Spinal Disease: A Cohort Study.

Malignant spinal lesions (MSLs) are frequently the first manifestation of malignant disease. Spinal care, diagnostic evaluation, and the initiation of systemic therapy are crucial for outcomes in patients (pts) with advanced cancer. However, histopathology (HP) may be time consuming. The additional evaluation of spinal lesions using cytopathology (CP) has the potential to reduce the time to diagnosis (TTD) and time to therapy (TTT). CP and HP specimens from spinal lesions were evaluated in parallel in 61 pts (CP/HP group). Furthermore, 139 pts in whom only HP was performed were analyzed (HP gr