Enfermedad de Von Willebrand adquirida en un Mieloma múltiple: reporte de caso

La Enfermedad de Von Willebrand adquirida (EVW adquirida) es un trastorno hemorrágico adquirido poco frecuente, con características clínicas y de laboratorio similares a la Enfermedad de Von Willebrand congénita. Asociándose con enfermedades hemato-oncológicas, autoinmunes, cardiovasculares y tumores sólidos. Las gammapatías monoclonales constituyen un grupo heterogéneo de trastornos caracterizados por la proliferación de linfocitos B en los últimos estadios madurativos o células plasmáticas que preservan la capacidad de producir una inmunoglobulina (Ig) monoclonal o alguno de sus componentes. Como consecuencia, se produce la aparición de una paraproteína o componente M (CM) en suero y/o orina que estará formado por la misma cadena pesada o ligera, y por regiones variables idénticas. Se presenta el caso de una mujer de 57 años, que se presenta con un síndrome hemorragíparo, alteración de la crasis en su vía intrínseca, donde se diagnostica EVW adq secundaria a Mieloma Múltiple (MM) con CM IgM 5,9 g/dl. El tratamiento tuvo como objetivos detener el sangrado, prevenir complicaciones y abordar precozmente la patología hemato-oncológica causante. Para su abordaje requirió la realización de recambios plasmáticos terapéuticos (RPT) que tuvieron un rol de acción terapéutica temprana y eficaz con excelente tolerancia. Ante el diagnóstico se inició rápidamente poliquimioterapia siendo ésta una paciente candidata a trasplante de progenitores hematopoyéticos. El objetivo de la presentación de este caso clínico es destacar la importancia de un correcto y oportuno diagnóstico ante la sospecha clínica de una coagulopatía secundaria a una enfermedad hemato-oncológica subyacente. Por lo que hacemos énfasis en el abordaje multidisciplinario.

Acquired von Willebrand disease (AVWS) is a rare acquired bleeding disorder with clinical and laboratory features similar to congenital von Willebrand disease. Associated with hemato-oncological, autoimmune, cardiovascular diseases and solid tumors. Monoclonal gammopathies constitute a heterogeneous group of disorders characterized by the proliferation of B lymphocytes in the last stages of maturation or plasma cells that preserve the capacity to produce a monoclonal immunoglobulin (Ig) or one of its components. As a consequence, the appearance of a paraprotein or M component (CM) occurs in serum and/or urine, which will be formed by the same heavy or light chain, and by identical variable regions. We present the case of a 57-year-old woman, who presented with a hemorrhagic parous syndrome, alteration of the crasis in its intrinsic way, where Acquired Von Willebrand Disease secondary to Multiple Myeloma is diagnosed. Treatment was aimed at stopping the bleeding, preventing complications, and promptly addressing the underlying hemato-oncological pathology. For its approach, it required the performance of therapeutic plasma exchanges that had a role of early and effective therapeutic action with excellent tolerance. Given the diagnosis, polychemotherapy was quickly started, this patient being a candidate for hematopoietic stem cell transplantation. The objective of presenting this clinical case is to highlight the importance of a correct and timely diagnosis in the face of clinical suspicion of a coagulopathy secondary to an underlying hemato-oncological disease. Therefore, we emphasize the multidisciplinary approach.

A doença de von Willebrand adquirida (EVW acq, AVWS) é um distúrbio hemorrágico adquirido raro com características clínicas e laboratoriais semelhantes à doença de von Willebrand congênita. Associado a doenças hemato-oncológicas, autoimunes, cardiovasculares e tumores sólidos. As gamopatias monoclonais constituem um grupo heterogêneo de distúrbios caracterizados pela proliferação de linfócitos B nos últimos estágios de maturação ou plasmócitos que preservam a capacidade de produzir uma imunoglobulina monoclonal (Ig) ou um de seus componentes. Como consequência, ocorre o aparecimento de uma paraproteína ou componente M (CM) no soro e/ou na urina, que serão formados pela mesma cadeia pesada ou leve, e por regiões variáveis idênticas. Apresentamos o caso de uma mulher de 57 anos, que apresentava um síndroma hemorrágico, alteração da crase na sua via intrínseca, onde é diagnosticada Doença de Von Willebrand Adquirida secundária a Mieloma Múltiplo. O tratamento visava estancar o sangramento, prevenir complicações e abordar prontamente a patologia hemato-oncológica subjacente. Para sua abordagem, exigiu a realização de plasmaférese terapêutica que teve papel de ação terapêutica precoce e efetiva com excelente tolerância. Diante do diagnóstico, rapidamente foi iniciada poliquimioterapia, sendo este paciente candidato a transplante de células-tronco hematopoiéticas. O objetivo da apresentação deste caso clínico é realçar a importância de um diagnóstico correto e atempado face à suspeita clínica de uma coagulopatia secundária a uma doença hemato-oncológica subjacente. Portanto, enfatizamos a abordagem multidisciplinar.

Multiple Myeloma: A Comprehensive Review Of Clinical Feature And Management

Multiple myeloma (MM) is a hematologic malignancy that accounts for 1-2% of all cancers and 17% of hematologic malignancies worldwide. It predominantly affects older adults, with a higher incidence in males and African American populations. Clinical manifestations often include bone pain, anemia, renal insufciency, and hypercalcemia, due to the proliferation of malignant plasma cells. Diagnosis is based on clinical, laboratory, and imaging ndings, including the detection of monoclonal proteins in serum or urine, and the presence of clonal plasma cells in bone marrow biopsy. Imaging techniques such as MRI and PET/CT play a key role in identifying osteolytic lesions and assessing disease progression. The management of MM has advanced with the use of autologous hematopoietic cell transplantation, triple drug regimens, and maintenance therapies.

Tratamiento secuencial de mieloma de alto riesgo en un paciente adulto mayor. A propósito de un caso / Sequential treatment of high-risk myeloma in an elderly patient. Case report

Resumen El mieloma múltiple es una neoplasia del paciente mayor con una edad media de diagnóstico de 70 años. Los pacientes de edad avanzada con mieloma de alto riesgo genético están poco representados en los estudios clínicos y son un grupo difícil de tratar, con una sobrevida menor a dos años. La edad trae consigo comorbilidades y fragilidad que determinan tolerancia pobre a terapias y mayor mortalidad, obligando a usar tratamientos no intensivos, con el objetivo de controlar la enfermedad y mantener la calidad de vida. En pacientes con mielomas de alto riesgo genético, en cambio, se busca lograr respuestas profundas con el uso de tripletes para superar el efecto adverso de la citogenética. Es así como el tratamiento del mieloma de alto riesgo a edades avanzadas implica requerir tratamientos efectivos en pacientes frágiles y con mala tolerancia a éstos. Se presenta el caso de una paciente de 84 años con mieloma de alto riesgo genético, determinado por la amplificación de 1q21, que recibe tratamientos sucesivos basados en lenalidomida, bortezomib, daratumumab y carfilzomib, lográndose mantener la enfermedad controlada por más de seis años. Este caso documenta que, en pacientes añosos con enfermedad de alto riesgo genético, un tratamiento continuado con drogas efectivas, un ajuste adecuado de dosis y tratamiento de soporte pueden mantener la enfermedad controlada con mínima toxicidad y prolongar la sobrevida del paciente adulto mayor.

Abstract Multiple myeloma is a neoplasm that primarily affects elderly patients, with an average age of diagnosis of 70 years. Elderly patients with high-risk genetic myeloma are underrepresented in clinical studies and pose a challenging group to treat, with a median survival of less than two years. Advanced age brings comorbidities and frailty, which result in poor tolerance to therapies and increased mortality, necessitating the use of less intensive treatments aimed at disease control and maintaining quality of life. In contrast, for high-risk myeloma, deep responses are sought through the use of triplets to overcome the adverse effects of cytogenetics. Thus, treating high-risk myeloma in elderly patients presents the challenge of requiring effective treatments in frail individuals with poor tolerance. We present the case of an 84-year-old patient with high-risk genetic myeloma determined by the amplification of 1q21, who received successive treatments based on lenalidomide, bortezomib, daratumumab, and carfilzomib, successfully maintaining disease control for over six years. This case documents that in elderly patients with high-risk disease, continuous treatment with effective drugs, appropriate dosage adjustments and supportive care can keep the disease under control with minimal toxicity and prolong the survival of the elderly patient.

Comparative assessment of beta-2 microglobulin as predictor of progression of multiple myeloma in asymptomatic and symptomatic multiple myeloma patients in a tertiary care hospital in Eastern India

Background: Multiple myeloma (MM) is a clonal late B-cell disorder in which malignant plasma cells expand and accumulate in the bone marrow and is associated with paraprotein production, bone lesions, anemia, hypercalcemia, susceptibility to infections, and renal impaired. Beta-2 microglobulin (B2M) is a serum marker of tumor burden in lymphoid malignancies, including MM. The purpose of this study was to find whether increased B2M levels can predict the progression of MM from asymptomatic to progressed symptomatic phase, as it is a less invasive and cost-effective method than the more invasive and costly ones to predict the progression of the disease. Aims and Objectives: This study was taken in a medical college, Kolkata, 50 asymptomatic Stage 1 MM patients were taken along with age and gender-matched 50 symptomatic progressed MM patients, blood samples of both groups were evaluated to estimate the difference in serum B2M (SB2M) level in both groups. Moreover, to study the difference in serum albumin, C-reactive protein, creatinine, immunoglobulin (IG), total protein, calcium, hemoglobin, and percentage of plasma cells in both groups and also their correlation with SB2M level in the progressed disease group. As well as to assess if there is increased B2M in the progressed disease group and if the B2M level correlates with the disease severity (i.e. progression of the disease). Materials and Methods: An observational descriptive non-interventional, hospital-based study conducted at central laboratory, medical college, Kolkata, for 6 months. Patients were chosen by systematic random sampling. Fifty asymptomatic MMs were chosen according to the International Myeloma Working Group criteria and out of 50 patients in the progressed disease group, 75% of patients were in Stage III and 25% of patients were in Stage II according to the Salmon and Durie system. Results: More than 80% in the progressed and symptomatic group had ?-2M >3.5 mg/L. Whereas in the newly diagnosed group, all the 50 patients had their ?-2M level <3.5 mg/L. Moreover, B2M value in the blood of progressed MM patients was found to be positively correlated with percentage of plasma cells from bone marrow, serum creatinine level, serum protein level, serum calcium level, and IgG level and negatively correlated with Hb level and serum albumin level. Hence, in a nutshell, increased B2M level was found in the progressed MM group, and its level positively correlated with the other predictors of the progression of MM. Conclusion: As per this study, SB2M level (which is a less invasive test than bone marrow testing and less costly) can be used as the predictor of progression of MM from asymptomatic to symptomatic. However, small sample size and cross-sectional pattern of thestudy were the limitations of this study. Bigger sample size and prospective cohort-type study designing method could be a better method to find a stronger correlation between MM staging and SB2M levels.

Solitary Plasmacytoma of Mandible Transforming to Multiple Myeloma? - A Rare Case Report

Background: Plasma cell dyscrasias is proliferation of the plasma cells that typically produces monoclonal immunoglobulin. Solitary plasmacytoma, multiple myeloma, and extramedullary plasmacytoma are their three distinct clinical entities. The most common among the plasmacytoma is the solitary bone type which accounts for less than 450 cases annually and constitutes only 2% to 5% of all plasma cell malignancies In case of solitary plasmacytoma, it is significant to make dissimilitude of whether minimal bone marrow clonal plasmacytosis are there or not, solitary bone lesion or soft tissue, negative whole-body imaging for further lesions, anemia and renal dysfunction. Almost 50% Solitary plasmacytoma cases shows transformation into multiple myeloma. Case Presentation: A 58 years old female patient with single, diffuse tumour mass in the left mandibular region of oral cavity was subjected for clinical, radiological, histopathological, immunohistochemical and biochemical investigation. The diagnosis of solitary plasmacytoma was done. But with correlation of all the investigation a suspect of transformation into multiple myeloma arise. Discussion: Despite of the excellent local control rates, majority of solitary plasmacytoma will eventually progress to multiple myeloma. Previous studies on solitary plasmacytoma had shown 5-year overall survival rate as 70% and 5-year of disease-free survival rate as 46%, with median time for development of multiple myeloma as 21 months, with a 5-year probability of 51%. Conclusion: Solitary plasmacytoma being a rare plasma cell malignancy with high potency to transform into multiple myeloma, a thorough investigation is needed before diagnosis. Early diagnosis and appropriate treatment is the key to increase disease free survivial rate of the patient.

Unveiling complexity: Chest wall swelling manifestation of extramedullary myeloma

There are two forms of myeloma: Solitary plasmacytoma and multiple myeloma. Both are malignant tumors and develop from bone marrow plasma cells, which secrete immunoglobulins. Pleural effusion secondary to myeloma is extremely rare, with an incidence of 1–2%. A 58-year-old man presented to the emergency department with a chief complaint of swelling and pain over the left side of his chest, breathlessness, generalized weakness, abdominal pain, and blood in his stool for 15 days. The chest X-ray posteroanterior view showed well-defined homogenous opacity in the left hemithorax. The patient underwent ultrasound-guided left-sided thoracocentesis. Pleural fluid was exudative, and cytology showed the presence of malignant cells. Contrast-enhanced computed tomography of the thorax and abdomen showed a well- defined soft-tissue density lesion involving the left posteriolateral chest wall with underlying rib erosion. For confirmation of malignant etiology, a USG-guided transthoracic needle biopsy was performed. A biopsy revealed a malignant round cell tumor in a histopathological review. For confirmation, a tissue sample was sent for immunohistochemistry markers, which had positivity for CD 138 and MUM 1, consistent with myeloma. Rarely, MM can present as chest wall swelling. Ultrasound is an easily available diagnostic modality that can be used for chest wall biopsy with a minimal incidence of complications.

Non Secretory Myeloma with Acute Renal Failure- A Case Report

Multiple myeloma, a cancer of plasma cell. They are usually two types; most common one is secretory type which is accounting to 95-97% & second one is non secretory type making up to 1-5%. Peripheral smear and Immunoglobulin levels will aid in diagnosis of multiple myeloma. Non secretory myeloma is a rare type, and all the definitive diagnostic features will be negative and poses diagnostic challenge. Non secretory myeloma has plasma cells that does not secrete immunoglobulins which is evaluated with immunofluorescence or immunoperoxide studies hence the symptoms like anemia, neutropenia, thrombocytopenia, infection recurrence, end organ damage fails to exhibit, which makes the diagnosis difficult.

A Case Report on Plasma Cell Leukemia Presenting as a Chest Wall Mass

Plasma cell leukemia (PCL) is an uncommon neoplasm of plasma cells with an aggressive clinical course and a poor outcome, even with the current standard of care. It can occur either de novo (primary PCL) or as a progression of multiple myeloma (MM). This disease has unique diagnostic criteria, but certain genetic markers and clinical features may overlap with multiple myeloma (MM). Due to the low prevalence of PCL, guidelines on its management are extrapolated from the management of MM and are based on small retrospective studies and case reports/series. We report the case of a sixty-nine-year-old man referred to the hematology department for the diagnosis of pPCL, revealed by thoracic plasmacytomas mimicking a thoracic neoplasm. The diagnostic approach, management, and outcomes of PCL are discussed.

Use of CAR-T cells for the treatment of relapsed and refractory multiple myeloma: a systematic review / Uso de células CAR-T no tratamento de mieloma múltiplo recidivado e refratário: uma revisão sistemática

A systematic review of published articles based on randomized clinical trials was conducted to ascertain the efficacy or perspective of using CAR-T cell therapy for refractory multiple myeloma. The PubMed database was searched with the combination of terms "multiple myeloma", "refractory multiple myeloma", "CAR T-cell", and the PRISMA criteria were followed. Of the 78 articles found, only 5 were selected. The studies used different treatment protocols and four different types of CAR-T cells. All studies obtained interesting results in terms of increased progression-free survival and negative minimal residual disease responses. Some authors detected an expansion of CAR-T cells and noted dose-dependent relationship between treatment effectiveness and serum BCMA levels. Although the results were promising, a small number of patients still relapsed a few months after CAR-T cell infusion. Therefore, this new line of therapy should be further investigated, as it significantly increases progression-free survival and improves quality of life.

Uma revisão sistemática de artigos publicados com base em ensaios clínicos randomizados foi realizada para verificar a eficácia ou perspectiva do uso da terapia com células CAR-T para mieloma múltiplo refratário. Foi pesquisada a base de dados PubMed com a combinação dos termos "multiple myeloma", "refratory multiple myeloma", "CAR T-cell" e foram seguidos os critérios PRISMA. Dos 78 artigos encontrados, apenas 5 foram selecionados. Os estudos utilizaram diferentes protocolos de tratamento e quatro tipos diferentes de células CAR-T. Todos os estudos obtiveram resultados interessantes em termos de aumento da sobrevida livre de progressão e respostas negativas à doença residual mínima. Alguns autores detectaram uma expansão das células CAR-T e observaram uma relação dose-dependente entre a eficácia do tratamento e os níveis séricos de BCMA. Embora os resultados tenham sido promissores, um pequeno número de pacientes ainda apresentou recaída alguns meses após a infusão de células CAR-T. Portanto, esta nova linha de terapia deve ser mais investigada, pois aumenta significativamente a sobrevida livre de progressão e melhora a qualidade de vida.

Teclistamab, cambiando el paradigma de pacientes con Mieloma Múltiple / Teclistamab, changing the paradigm of patients with Multiple Myeloma

Resumen El mieloma múltiple (MM) sigue siendo una patología incurable a pesar de las mejoras en las opciones de tratamiento que se desarrollaron en los últimos años. El antígeno de maduración de células B (BCMA) se expresa predominantemente en células de linaje B y representa un nuevo objetivo terapéutico prometedor para el MM recaído refractario (MMRR). Teclistamab (TECVAYLI) es el primer anticuerpo biespecífico de redirección de células T (CD3) contra BCMA (Figura 1) aprobado por la Administración de Drogas y Alimentos de Estados Unidos (FDA) en 2022 para pacientes con MMRR a 3 líneas de tratamiento previos, incluyendo Inhibi-dores de Proteosoma (IP), Inmunomoduladores (IMIDS), Anticuerpos Monoclonales (AcMo). La neurotoxicidad asociada a células efectoras inmunitarias (ICANS), el síndrome de liberación de citoquinas (CRS) e infecciones por hipogamaglobulinemia son los efectos adversos más comunes.

Abstract Multiple myeloma (MM) remains an incurable disease despite improvements in treatment options that have been developed recent years. B cell maturation antigen (BCMA) is predominantly expressed on B lineage cells and represents a promising new therapeutic target for relapsed re-fractory MM (RRMM). Teclistamab (TECVAYLI) is the first bispecific T cell (CD3) redirecting antibody against BCMA (Figure 1) approved by the US Food and Drug Administration (FDA) in 2022 for patients with RRMM on 3 prior lines of treatment, including Proteasome Inhibitors (PI), Immunomodulators (IMIDS), Monoclonal Antibodies (mAb). ICANS (immune effector cell-associated neurotoxicity), CRS (cytokine release syndrome), and hypogammaglobulinemia infections are the most common adverse effects.

Risk stratification in multiple myeloma – A review and update

Multiple myeloma (MM) is a hematological malignancy of plasma cell origin with a prevalence rate of 1% and 10% of all cancers and hematopoietic malignancies, respectively. Though the median survival time has improved dramatically in the patients diagnosed with MM with the administration of novel therapeutic agents, the disease, by and large, remains incurable with frequent progression and relapses. In the recent past, an increased understanding of MM pathogenesis has opened facets for improved diagnosis, prognosis, and response assessment in patients diagnosed with MM. This review focuses on the various laboratory and clinical features used to stratify the MM patients into high vs. low-risk groups. Furthermore, it also highlights the role of artificial intelligence-based innovative research tools for risk stratification and prognostication in MM patients.

Plasma cell myeloma with double M bands on Serum protein electrophoresis: A diagnostic conundrum?

Plasma cell myeloma (PCM) is a monoclonal gammopathy (MGM) characterized by proliferation of abnormal clone of plasma cells infiltrating the bone marrow with consequent end organ damage. The clonal plasma cells secrete a single clone of immunoglobulins (Ig) leading to presence of M?protein in the serum and/or urine. The M?protein is appreciated as a discrete band on serum protein electrophoresis (SPE) in the gamma globulin region, also called the M?band. Biclonal gammopathy (BGM) occurs due to neoplastic transformation of a plasma cell clone undergoing Ig class switching or due to an independent neoplastic transformation event yielding proliferation of unrelated plasma cell clones, therefore resulting in two distinct M?bands on SPE. It is, however, vital to distinguish a true BGM from an apparent one (MGM presenting with two distinct bands on SPE) so as to make an accurate diagnosis. Hereby, we report a case of a 61?year?old man, diagnosed with PCM and presenting with two discrete bands on SPE (simulating a BGM) which turned out to be monoclonal in nature.

Use of potentially inappropriate medications and medications of concern in older adults with multiple myeloma / Utilização de medicamentos potencialmente inapropriados e medicamentos que requerem cautela em idosos com mieloma múltiplo

Objetivo: Analisar o uso de medicamentos potencialmente inapropriados (MPIs) e o uso de medicamentos usados em terapia de suporte que requerem cautela em idosos com câncer (MTSRCICs), determinando os fatores associados. Visou-se também determinar a concordância entre os critérios explícitos empregados na identificação de MPI. Metodologia: Estudo transversal com indivíduos com mieloma múltiplo (MM), idade ≥ 60 anos em tratamento ambulatorial. Os MPI foram identificados de acordo com os critérios AGS Beers 2019, PRISCUS 2.0 e o Consenso Brasileiro de Medicamentos Potencialmente Inapropriados (CBMPI). Os MTSRCIC foram definidos de acordo com a National Comprehensive Cancer Network. Os fatores associados ao uso de MPI e MTSRCIC foram identificados por regressão logística múltipla. O grau de concordância entre os três critérios explícitos empregados no estudo foi mensurado pelo coeficiente kappa Cohen. Resultados: As frequências de MPI foram 52,29% (AGS Beers 2019), 62,74% (CBMPI), 65,36% (PRISCUS 2.0) e 52,29% (MTSRCICs). As concordâncias entre AGS Beers 2019 com PRISCUS 2,0 e com CBMPI foram altas, enquanto a concordância entre CBMPI e PRISCUS 2.0 foi excelente. No modelo final de regressão logística polifarmácia foi associada positivamente ao uso de MPI por idosos para os três critérios explícitos utilizados, além de associado à utilização de MTSRCICs. Conclusões: A frequência do uso de MPI e de MTSRCIC foi elevada. A concordância em relação ao uso de MPI entre os critérios AGS Beers 2019, CBMPI e PRISCUS 2.0 foi alta ou excelente. A polifarmácia apresentou associação independente e positiva com uso de MPIs e de MTSRCICs por pacientes idosos com MM. (AU)

Objectives: To analyze the use of potentially inappropriate medications (PIMs) and medications used in supportive therapy that require caution in older adults with cancer, in addition to determining associated factors the agreement between criteria sets used to identify PIMs. Methods: This cross-sectional study included individuals with multiple myeloma aged ≥ 60 years who were undergoing outpatient treatment. PIMs were identified according to American Geriatric Society Beers 2019, PRISCUS 2.0, and Brazilian Consensus on Potentially Inappropriate Medicines criteria. Medications of concern were defined according to National Comprehensive Cancer Network criteria. Factors associated with the use of PIMs and medications of concern were identified using multiple logistic regression. The degree of agreement between the 3 criteria sets was measured using Cohen's kappa coefficient. Results: The frequency of PIM use was 52.29% according to American Geriatric Society Beers criteria, 62.74% according to Brazilian Consensus criteria, and 65.36% according to PRISCUS criteria, while 52.29% of the patients were using medications of concern. Agreement between American Geriatric Society Beers, PRISCUS, and Brazilian Consensus criteria was high, while it was excellent between Brazilian Consensus and PRISCUS criteria. In the final logistic regression model, polypharmacy was associated with PIM use according to each criteria set, as well as the use of medications of concern. Conclusions: The frequency of PIMs and medications of concern was high. Agreement about PIM use between the American Geriatric Society Beers, Brazilian Consensus, and PRISCUS criteria was high or excellent. There was an independent association between polypharmacy and the use of PIMs and medications of concern by older patients with multiple myeloma. (AU)

Utility of serum protein electrophoresis, free light chain analysis, immunofixation electrophoresis and immunosubtraction for the diagnosis of plasma cell dyscrasias

Background: Monoclonal gammopathies (MG) are a spectrum of diseases, which if diagnosed early can improve the disease prognosis. Serum Protein Electrophoresis (SPE) was the preferred screening test for MG, but the low sensitivity of the same emphasizes the necessity of going for more sensitive and accurate tests. SPE is complemented with tests like serum Free Light Chain assay (sFLC), Serum Immunofixation Electrophoresis (sIFE) and Serum Immunosubtraction Electrophoresis (sISE). Aim: Our aim was to find out the utility of each of these tests in diagnosing MG and propose a panel of tests for the diagnosis of MGs Material and methods: 30 patients with features of MG were recruited and SPE, sFLC, sIFE and sISE were performed. Results: Accuracy of sIFE with respect to sISE was 100% and accuracy of sFLC with respect to SPE came to be 90%. SPE, sFLC and sIFE panel and SPE, sFLC and sISE panel were able to detect all cases. Combination of SPE + sFLC + sIFE and SPE + sFLC + sISE tests were able to detect all 24 cases of MG and found to be efficient than any of these tests performed alone. Discussion: The diagnostic accuracy of sIFE and sISEin MM patients was compared and was found to be statistically insignificant thus proving that both tests contribute equally for the diagnosis of MM.

Clear Cell Myeloma - A Rare Histopathological Variant of a Common Disease

Multiple myeloma (MM) is characterized by neoplastic proliferation of plasma cells and monoclonal immunoglobulin production. Clear-cell myeloma is an exceptionally rare histopathological variant of MM, with only ten cases reported worldwide. We present one such case in a 67-year-old female, presenting as a soft tissue osteolytic lesion in the thoracic vertebrae. The final diagnosis was made with the help of histopathological examination, immunohistochemical and hematological features, along with protein electrophoresis and serum free light chain assay. We aim to emphasize its importance as a differential diagnosis for clear cell neoplasms, as timely chemotherapeutic intervention improves the survival rate.

Myelomatous pleural effusion in multiple myeloma- A rare presentation

Multiple myeloma is a malignant plasma cell condition that mostly affects the skeletal system and bone marrow. Pleural effusions are uncommon and typically result from other conditions coexisting with multiple myeloma. Malignant myelomatous pleural effusions are rare complications of multiple myeloma, occurring in less than 1% of patients and are associated with poor prognosis having mean survival of less than 4 months. The present case report is a 41?year?old multiple myeloma patient who developed bilateral pleural effusion at a disease relapse. Chemotherapeutic regimen of cyclophosphamide, bortezomib, and dexamethasone given. Despite a positive response to treatment, the patient’s condition worsened over the course of following month and he eventually passed away. Myelomatous pleural effusion indicates poor prognosis and early consideration helps in quick diagnosis and initiation of treatment which may help in improving prognosis.

An ayurvedic evaluation & treatment of multiple myeloma: A case report

This case report discusses the management of multiple myeloma in a 59-year-old male patient through an integrative approach of Ayurvedic and conventional medical care. The patient presented with symptoms of pain in ribs, fatigue, nausea, weight loss, and mental stress. After undergoing chemotherapy and steroid therapy, the patient opted for Ayurvedic treatment instead of a recommended bone marrow transplant. Ayurvedic exami- nation revealed imbalances in Vata and Pitta doshas, affecting various body tissues and mental state. The Ay- urvedic regimen led to the recovery of the patient without adverse effects. This case highlights the role of Ayurvedic medication in managing multiple myeloma, warranting further research and clinical trials for broader validation.

Removing the interference of daratumumab on transfusion compatibility testing and transfusion efficacy comparison / 中国输血杂志

【Objective】 To explore the feasibility of blood transfusion compatibility testing for multiple myeloma(MM) patients treated with anti-CD38 monoclonal antibody daratumumab (DARA) after DARA-Fab fragment blocking, and to evaluate the transfusion efficacy by comparing with dithiothreitol(DTT) method. 【Methods】 After DARA was prepared into DARA-Fab fragments using PierceFab preparation kit, the neutralization effects of different volumes (5, 10, 15, 30 μL) on screening cells and panel cells were confirmed. DARA-Fab fragments and screening cells with specific antigens and corresponding monoclonal antibody reagents were used as the experimental group and the control group with the same volume of saline for incubating and centrifugin.Twenty MM patients treated with DARA were selected for cross-matching with DARA-Fab and DTT respectively, and the laboratory indexes before and after transfusion were statistically analyzed, and the two blood matching methods were compared. 【Results】 After incubating and centrifuging, the results of DARA-Fab fragments(15, 30 μL) with screening cells and serum mixed with DARA were negative, while those of DARA-Fab(5, 10 μL) were positive. 15μL DARA-Fab treated antibody identification cells (2, 3, 4, 5, 7, 9, 11) were negative, antibody identification cells (1, 6, 8, 10, 12) were negative after 30 μL DARA-Fab fragments treatment; the results of MNS, Duffy, Kidd, Kell, Lewis, Rh blood group system of the experimental group were consistent with those of the control group; the hemoglobin (Hb) (g/L) of 20 patients after infusion of RBC (73.90±1.90) was significantly higher than that before transfusion (63.60±1.58), P0.05).And no statistical difference was noticed in Hb (10.75±1.04 vs 10.30±0.98), TBil (3.31±1.47 vs 3.31±0.55), DBIL(2.76±1.24 vs 2.60±0.83), and I-Bil(1.97±0.40 vs 2.82±0.53) between the DTT treatment method and the DARA Fab fragment treatment before and after transfusion(P>0.05). 【Conclusion】 DARA-Fab can remove the interference of RBC on cross matching by blocking CD38 antigen. This method has no effect on the antigens of common RBC blood group systems, and shows significant blood transfusion efficacy as that of DTT method.

Research progress of selinexor in the treatment of recurrent and refractory multiple myeloma / 中国医师杂志

Multiple myeloma is an incurable hematological malignancy. Although the continuous development of therapeutic drugs such as proteasome inhibitors and immune modulators, as well as chimeric antigen receptor T-cell (CAR-T) therapy, has improved the prognosis in recent years, some patients are still drug-resistant, presenting as refractory and recurrent disease with limited treatment options. Selinexor, a first-in-class oral selective nuclear export protein inhibitor, binds to and inhibits nuclear export protein XPO-1 to function, leading to the accumulation of tumor suppressor proteins in the nucleus and selective apoptosis of cancer cells. It has shown controllable toxicity and good efficacy in the treatment of recurrent and refractory multiple myeloma. This article discusses the anti-tumor mechanism of selinexor, its clinical research progress, and adverse reactions.

Cinobufagin Combined with Thalidomide/Dexamethasone Regimen in the Treatment of Patients with Newly Diagnosed Multiple Myeloma of Phlegm and Stasis Obstruction： A Retrospective Study / 中医杂志

ObjectiveTo investigate the efficacy and safety of cinobufagin tablets combined with thalidomide/dexamethasone （TD） regimen in the treatment of newly diagnosed multiple myeloma （NDMM） with phlegm and stasis obstruction. MethodsThe clinical data of 50 patients with NDMM of phlegm and stasis obstruction who were hospitalized at the Jiangsu Province Hospital of Chinese Medicine from June 1st， 2015 to July 31th， 2019 were retrospectively analyzed， and they were divided into a control group （bortezomib/dexamethasone-containing regimen， 27 cases） and an observation group （cinobufagin tablets combined with TD regimen， 23 cases）. The clinical efficacy and safety were compared between the two groups after two or three courses of treatment. The primary outcomes were clinical remission rate including overall response rate and deep remission rate， one-year and two-year overall survival rate， and adverse effects. The secondary outcomes were the proportion of plasma cells in bone marrow， hemoglobin， β2-microglobulin， lactate dehydrogenase， serum creatinine， blood urea nitrogen， bone pain score， and KPS functional status score （KPS score） before and after treatment. ResultsIn terms of clinical efficacy， there was no statistically significant difference （P＞0.05） in the overall response rate ［the observation group 69.57%（16/23） vs the control group 70.37% （19/27）］ and deep remission rate ［the observation group 56.52% （13/23） vs the control group 55.56% （15/27）］ between groups after the treatment. The one-year overall survival rates of the observation group and the control group were 90.9% and 92.4%， and the two-year overall survival rates were 81.8% and 80.9% respectively， with no statistically significant differences between groups （P＞0.05）. During the treatment， no renal function injury occurred in both groups. The incidence of peripheral nerve injury in the observation group was 8.70%， which was lower than 48.15% in the control group （P＜0.01）. After the treatment， the proportion of myeloma plasma cells， β2-microglobulin， serum creatinine level， and bone pain score decreased， while the hemoglobin level and KPS score increased in both groups （P＜0.05 or P＜0.01）. Compared between groups after treatment， the bone pain score of the observation group was lower than that of the control group， while the KPS score was higher than that of the control group （P＜0.05）. ConclusionThe clinical efficacy of cinobufagin tablets combined with TD in the treatment of NDMM is equivalent to bortezomib/dexamethasone-containing regimen， but the former is more helpful in relieving the pain and improving the quality of life， and has better safety.