-
Journal of Radiology Case Reports 2024Multiple myeloma is a plasma cell neoplasm, which may present as a solitary plasmacytoma and, uncommonly, as an extramedullary plasmacytoma. Intracranial plasmacytomas...
Multiple myeloma is a plasma cell neoplasm, which may present as a solitary plasmacytoma and, uncommonly, as an extramedullary plasmacytoma. Intracranial plasmacytomas may manifest in central nervous system involvement as cranial nerve palsies. Cranial nerve six palsy is the most common in cases of malignancy. However, isolated abducens palsy presenting as multiple myeloma recurrence is very uncommon. Here, we detail two cases in which intracranial plasmacytoma lesions were present within the region of the Dorello canal, resulting in acute isolated unilateral diplopia from disease recurrence in the absence of systemic marrow involvement.
Topics: Humans; Abducens Nerve Diseases; Multiple Myeloma; Neoplasm Recurrence, Local; Male; Magnetic Resonance Imaging; Middle Aged; Aged; Diagnosis, Differential; Plasmacytoma; Female; Diplopia; Brain Neoplasms
PubMed: 38910589
DOI: 10.3941/jrcr.v18i1.5240 -
Journal of the American Heart... Jul 2024Cardiac amyloidosis (CA) is frequently found in older patients with aortic stenosis (AS). However, the prevalence of AS among patients with CA is unknown. The objective...
BACKGROUND
Cardiac amyloidosis (CA) is frequently found in older patients with aortic stenosis (AS). However, the prevalence of AS among patients with CA is unknown. The objective was to study the prevalence and prognostic impact of AS among patients with CA.
METHODS AND RESULTS
We conducted a retrospective analysis of a prospective registry comprising 976 patients with native aortic valves who were confirmed with wild type transthyretin amyloid (ATTRwt), hereditary variant transthyretin amyloid (ATTRv), or immunoglobulin light-chain (AL) CA. CA patients' echocardiograms were re-analyzed focusing on the aortic valve. Multivariable Cox regression analysis was performed to assess the mortality risk associated with moderate or greater AS in ATTRwt CA. The crude prevalence of AS among patients with CA was 26% in ATTRwt, 8% in ATTRv, and 5% in AL. Compared with population-based controls, all types of CA had higher age- and sex-standardized rate ratios (SRRs) of having any degree of AS (AL: SRR, 2.62; 95% Confidence Interval (CI) [1.09-3.64]; ATTRv: SRR, 3.41; 95%CI [1.64-4.60]; ATTRwt: SRR, 10.8; 95%CI [5.25-14.53]). Compared with hospital controls, only ATTRwt had a higher SRR of having any degree of AS (AL: SRR, 0.97, 95%CI [0.56-1.14]; ATTRv: SRR, 1.27; 95%CI [0.85-1.44]; ATTRwt: SRR, 4.01; 95%CI [2.71-4.54]). Among patients with ATTRwt, moderate or greater AS was not associated with increased all-cause death after multivariable adjustment (hazard ratio, 0.71; 95%CI [0.42-1.19]; =0.19).
CONCLUSIONS
Among patients with CA, ATTRwt but not ATTRv or AL is associated with a higher prevalence of patients with AS compared with hospital controls without CA, even after adjusting for age and sex. In our population, having moderate or greater AS was not associated with a worse outcome in patients with ATTRwt.
Topics: Humans; Male; Female; Prevalence; Aged; Retrospective Studies; Aortic Valve Stenosis; Prognosis; Cardiomyopathies; Aged, 80 and over; Registries; Amyloid Neuropathies, Familial; Risk Factors; Echocardiography; Middle Aged; Amyloidosis; Immunoglobulin Light-chain Amyloidosis; Prealbumin; Aortic Valve
PubMed: 38904242
DOI: 10.1161/JAHA.124.034723 -
Blood Jun 2024
Azab AK, Runnels JM, Pitsillides C, et al. CXCR4 inhibitor AMD3100 disrupts the interaction of multiple myeloma cells with the bone marrow microenvironment and enhances their sensitivity to therapy. Blood. 2009;113(18):4341-4351.
Topics: Cyclams; Humans; Multiple Myeloma; Receptors, CXCR4; Benzylamines; Heterocyclic Compounds; Bone Marrow; Tumor Microenvironment
PubMed: 38900482
DOI: 10.1182/blood.2024025275 -
Blood Jun 2024
Azab AK, Azab F, Blotta S, et al. RhoA and Rac1 GTPases play major and differential roles in stromal cell-derived factor-1-induced cell adhesion and chemotaxis in multiple myeloma. Blood. 2009;114(3):619-629.
Topics: Humans; rhoA GTP-Binding Protein; Multiple Myeloma; rac1 GTP-Binding Protein; Chemokine CXCL12; Cell Adhesion; Chemotaxis
PubMed: 38900475
DOI: 10.1182/blood.2024025276 -
Blood Jun 2024
Topics: Multiple Myeloma; Humans; Immunotherapy
PubMed: 38900472
DOI: 10.1182/blood.2024024709 -
Blood Jun 2024
Azab AK, Quang P, Azab F, et al. P-selectin glycoprotein ligand regulates the interaction of multiple myeloma cells with the bone marrow microenvironment. Blood. 2012;119(6):1468-1478.
Topics: Multiple Myeloma; Humans; Bone Marrow; Tumor Microenvironment; Membrane Glycoproteins; P-Selectin
PubMed: 38900471
DOI: 10.1182/blood.2024024988 -
Blood Jun 2024
Houde CA, Khan A, Jacobus SJ, et al. Treatment outcomes and prognostic factors with lenalidomide, bortezomib, and dexamethasone (RVd) alone versus Rvd plus autologous stem cell transplantation (ASCT) in African American (AA) patients (Pts) with newly diagnosed multiple myeloma (NDMM) in the...
Topics: Humans; Multiple Myeloma; Dexamethasone; Lenalidomide; Antineoplastic Combined Chemotherapy Protocols; Bortezomib; Transplantation, Autologous; Black or African American; Prognosis; Hematopoietic Stem Cell Transplantation; Treatment Outcome; Male; Female; Middle Aged
PubMed: 38900470
DOI: 10.1182/blood.2024025081 -
Oncology (Williston Park, N.Y.) Jun 2024The June Hot Topics focuses on the challenges venetoclax regimens have faced in multiple myeloma trials.
The June Hot Topics focuses on the challenges venetoclax regimens have faced in multiple myeloma trials.
Topics: Multiple Myeloma; Humans; Bridged Bicyclo Compounds, Heterocyclic; Sulfonamides; Chromosomes, Human, Pair 14; Antineoplastic Agents; Translocation, Genetic; Chromosomes, Human, Pair 11; Clinical Trials as Topic; Antineoplastic Combined Chemotherapy Protocols
PubMed: 38899981
DOI: 10.46883/2024.25921023 -
BMC Medical Genomics Jun 2024Immunoregulatory drugs regulate the ubiquitin-proteasome system, which is the main treatment for multiple myeloma (MM) at present. In this study, bioinformatics analysis...
BACKGROUND
Immunoregulatory drugs regulate the ubiquitin-proteasome system, which is the main treatment for multiple myeloma (MM) at present. In this study, bioinformatics analysis was used to construct the risk model and evaluate the prognostic value of ubiquitination-related genes in MM.
METHODS AND RESULTS
The data on ubiquitination-related genes and MM samples were downloaded from The Cancer Genome Atlas (TCGA) and Gene Expression Omnibus (GEO) databases. The consistent cluster analysis and ESTIMATE algorithm were used to create distinct clusters. The MM prognostic risk model was constructed through single-factor and multiple-factor analysis. The ROC curve was plotted to compare the survival difference between high- and low-risk groups. The nomogram was used to validate the predictive capability of the risk model. A total of 87 ubiquitination-related genes were obtained, with 47 genes showing high expression in the MM group. According to the consistent cluster analysis, 4 clusters were determined. The immune infiltration, survival, and prognosis differed significantly among the 4 clusters. The tumor purity was higher in clusters 1 and 3 than in clusters 2 and 4, while the immune score and stromal score were lower in clusters 1 and 3. The proportion of B cells memory, plasma cells, and T cells CD4 naïve was the lowest in cluster 4. The model genes KLHL24, HERC6, USP3, TNIP1, and CISH were highly expressed in the high-risk group. AICAr and BMS.754,807 exhibited higher drug sensitivity in the low-risk group, whereas Bleomycin showed higher drug sensitivity in the high-risk group. The nomogram of the risk model demonstrated good efficacy in predicting the survival of MM patients using TCGA and GEO datasets.
CONCLUSIONS
The risk model constructed by ubiquitination-related genes can be effectively used to predict the prognosis of MM patients. KLHL24, HERC6, USP3, TNIP1, and CISH genes in MM warrant further investigation as therapeutic targets and to combat drug resistance.
Topics: Humans; Multiple Myeloma; Computational Biology; Prognosis; Ubiquitination; Gene Expression Regulation, Neoplastic; Biomarkers, Tumor; Nomograms; Cluster Analysis
PubMed: 38898455
DOI: 10.1186/s12920-024-01937-0 -
BMC Ophthalmology Jun 2024Paraproteinemic keratopathy is a rare disorder characterized by the bilateral accumulation of polychromatic deposits diffusely in all corneal layers together or not with...
BACKGROUND
Paraproteinemic keratopathy is a rare disorder characterized by the bilateral accumulation of polychromatic deposits diffusely in all corneal layers together or not with diffuse or patchy pseudo lipid deposits. We present an atypical case of paraproteinemic keratopathy which lead to an initial misdiagnosis of infectious crystalline keratopathy.
CASE PRESENTATION
a 69-year-old woman with an asymptomatic keratopathy detected during a cataract intervention. Slit-lamp examination revealed several hyper refringent subepithelial foci with fern-shaped branches, resembling crystalline keratopathy, in her left eye. Anterior segment optical coherence tomography revealed exclusively subepithelial hyperreflective lesions limited to the anterior stroma. The progressive bilateralization and progression of the condition prompted us to include other entities with crystalline corneal deposits in our differential diagnosis. Hematological analysis showed a high number of free Kappa light chains. Despite the typical clinical appearance of crystalline keratopathy, the atypical evolution and test results led us to consider that monoclonal gammopathy could be the cause of this entity.
CONCLUSIONS
Paraproteinemic keratopathy may present in its early stages as a unilateral subepithelial crystalline keratopathy. Thus, it must always be taken into account in the differential diagnosis of any crystalline keratopathy, particularly when there are no predisposing factors for an infectious crystalline keratopathy. Early recognition of this rare entity is important to address the associated potentially serious systemic disease.
Topics: Humans; Aged; Female; Diagnosis, Differential; Corneal Diseases; Paraproteinemias; Tomography, Optical Coherence
PubMed: 38898421
DOI: 10.1186/s12886-024-03487-6