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1.
Klin Onkol ; 38(3): 209-219, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38960677

RESUMO

INTRODUCTION: Multiple myeloma is a common plasma cell neoplasia usually accompanied by the formation of osteolytic foci, whereas osteosclerotic myeloma is a very rare form of plasma cell dyscrasia. When osteosclerotic myeloma is detected, osteosclerotic foci are usually part of the POEMS syndrome. Osteosclerotic myeloma without other manifestations of the POEMS syndrome is an unusual finding. CASE DESCRIPTION: In a 46-year-old woman, osteosclerotic changes of the temporoparietal region caused soft tissue induration over this lesion, which initiated further investigation. Imaging studies subsequently showed multiple osteosclerotic foci in the skull. Examination of blood proteins revealed 8 g/L of IgG-lambda monoclonal immunoglobulin, subclass IgG1. In search of the cause of the osteosclerotic changes, FDG-PET/CT was performed, which revealed no FDG accumulation, i.e., no other tumor (breast or stomach cancer). Low-dose CT showed irregular bone structure, but not significant osteolytic or osteosclerotic foci. To map the extent of osteosclerotic changes, NaF-PET/CT imagination followed, which revealed multiple spots with high fluoride accumulation. A parietal bone biopsy showed osteosclerosis with minor clonal plasma cell infiltration. Trepanobioptic bone marrow sampling revealed an infiltration of bone marrow with atypical plasma cells in 8%. Flow-cytometric examination of bone marrow showed 0,37% of plasma cells, however predominantly (91%) clonal with lambda expression. MRI of the brain identified asymptomatic meningeal thickening. There was no evidence of POEMS syndrome in the patient; thus, we concluded the diagnosis as monoclonal gammopathy of clinical significance with osteosclerosis which was previously termed osteosclerotic multiple myeloma. CONCLUSION: Monoclonal gammopathy of clinical significance (MGCS) with osteosclerotic skeletal changes, documented on CT and multiple foci with intensive osteoneogenesis, documented on NaF-PET/CT without evidence of POEMS syndrome, is an extremely rare form of plasma cell dyscrasia. This publication documents the unique clinical manifestations of IgG-lambda type plasma cell proliferation without signs of POEMS syndrome and the role of NaF-PET/CT imaging. Classification of this disease as MGSC with osteosclerotic manifestations is more consistent with the indolent nature of the disease with a significantly better prognosis, compared with multiple myeloma.


Assuntos
Mieloma Múltiplo , Osteosclerose , Humanos , Pessoa de Meia-Idade , Feminino , Osteosclerose/diagnóstico por imagem , Osteosclerose/etiologia , Osteosclerose/patologia , Mieloma Múltiplo/complicações , Mieloma Múltiplo/patologia , Mieloma Múltiplo/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Paraproteinemias/complicações , Paraproteinemias/patologia
2.
Zhonghua Xue Ye Xue Za Zhi ; 45(4): 313-321, 2024 Apr 14.
Artigo em Chinês | MEDLINE | ID: mdl-38951057

RESUMO

Flow cytometry plays an important role in the diagnosis and treatment of plasma cell diseases, particularly in the detection of circulating plasma cells (CPCs) in the peripheral blood. A consensus about the normalized use of flow cytometry in detection of CPCs in peripheral blood in clinical practice has been achieved. This consensus is founded on evidence-based principles, which elucidates the timing and value of flow cytometry for the detection of CPCs in the monoclonal gammopathy of undetermined significance, smoldering myeloma, multiple myeloma, and plasma cell leukemia and standardizes flow cytometry in the detection of CPCs in plasma cell diseases.


Assuntos
Citometria de Fluxo , Mieloma Múltiplo , Plasmócitos , Citometria de Fluxo/métodos , Humanos , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/sangue , China , Paraproteinemias/diagnóstico , Paraproteinemias/sangue , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/sangue , Consenso , População do Leste Asiático
3.
Cas Lek Cesk ; 163(3): 98-105, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38981730

RESUMO

The incidence of monoclonal gammopathy (MG) increases with age. In individuals over 80 years of age, we can diagnose the presence of monoclonal immunoglobulin (MIg) in up to 10 % of cases. Not only malignant diseases such as multiple myeloma (MM), but also benign forms such as MGUS (monoclonal gammopathy of undetermined significance) can lead to renal involvement. The light chains of immunoglobulins (LC) are the most damaging to the kidneys, as they are freely filtered into the urine due to their molecular weight. Detection of MIg relies mainly on a combination of immunofixation electrophoresis of serum (IELFO) and urine and determination of free light chains (FLC) of kappa and lambda and their ratio (κ/λ) in serum. The combination of these tests will detect the presence of MIg with 99 % sensitivity. Renal damage in MG may be caused by direct deposition of MIg in the glomeruli (e.g. AL amyloidosis, LC deposition disease) or tubules (in the distal tubule as a myeloma kidney or in the proximal tubule as Fanconi syndrome or proximal tubulopathy). Typical urinary findings in these diseases are moderate or severe proteinuria or nephrotic syndrome. Acute kidney injury (AKI) can be expected especially when serum FLC is >500 mg/l. Renal biopsy is crucial to establish an accurate diagnosis and thus initiate the correct treatment. Treatment of these types of renal damage involves the same treatment regimens used in the treatment of MM, including proteasome inhibitors or daratumumab.


Assuntos
Mieloma Múltiplo , Paraproteinemias , Humanos , Mieloma Múltiplo/complicações , Mieloma Múltiplo/diagnóstico , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Nefropatias/etiologia , Nefropatias/diagnóstico
7.
BMJ Case Rep ; 17(6)2024 Jun 06.
Artigo em Inglês | MEDLINE | ID: mdl-38844356

RESUMO

A man in his 40s presented to the emergency department after 2 weeks of abdominal pain and bloating. Radiological investigations revealed multiple unusual sites of thrombosis, including large thrombi in his portal and mesenteric veins, and a left ventricular thrombus with resultant embolic infarcts to his spleen, kidneys, coronary arteries and brain. Standard causes of underlying thrombophilia were excluded. A serum protein electrophoresis and serum-free light chains, with subsequent bone marrow biopsy, lead to the diagnosis of smouldering multiple myeloma (sMM), albeit an unusual presentation with severe clinical sequelae. Although sMM is known to be associated with an increased risk of venous thromboembolism, it is not recognised to cause thrombosis in both venous and arterial vascular beds simultaneously. Physicians encountering patients with multiple thrombi in unusual vascular beds without clear aetiology should consider an underlying monoclonal gammopathy in their list of differentials.


Assuntos
Mieloma Múltiplo , Humanos , Masculino , Mieloma Múltiplo/complicações , Mieloma Múltiplo/diagnóstico , Trombose Venosa/etiologia , Trombose Venosa/diagnóstico por imagem , Adulto , Trombose/etiologia , Trombose/diagnóstico por imagem , Trombose/diagnóstico , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Diagnóstico Diferencial
8.
BMC Ophthalmol ; 24(1): 263, 2024 Jun 19.
Artigo em Inglês | MEDLINE | ID: mdl-38898421

RESUMO

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.


Assuntos
Doenças da Córnea , Paraproteinemias , Tomografia de Coerência Óptica , Humanos , Idoso , Feminino , Diagnóstico Diferencial , Doenças da Córnea/diagnóstico , Paraproteinemias/diagnóstico , Paraproteinemias/complicações , Tomografia de Coerência Óptica/métodos
9.
Zhonghua Jie He He Hu Xi Za Zhi ; 47(6): 501-503, 2024 Jun 12.
Artigo em Chinês | MEDLINE | ID: mdl-38858199

RESUMO

Pulmonary light chain deposition disease (PLCDD) is a rare monoclonal immunoglobulin deposition disease characterized by the deposition of specific immunoglobulin light chains in lung tissue. In its early stages, PLCDD presents with mild clinical symptoms, while pulmonary imaging shows multiple nodules and thin-walled cysts. Pathologically, there is a deposition of eosinophilic amorphous protein-like material in the tissues, which stains negative for Congo red. The exact pathological mechanism of PLCDD remains unclear, and its clinical presentation lacks specificity. Challenges associated with this condition include difficulties in diagnosis, selection and evaluation of treatment options, lack of clear monitoring criteria, and standards for prognosis assessment. Further research is needed to elucidate the pathogenesis of PLCDD, to establish standardized diagnostic and therapeutic protocols, and to evaluate treatment efficacy and prognostic factors.


Assuntos
Cadeias Leves de Imunoglobulina , Humanos , Cadeias Leves de Imunoglobulina/metabolismo , Paraproteinemias/diagnóstico , Pulmão/diagnóstico por imagem , Pulmão/patologia , Prognóstico , Pneumopatias/diagnóstico
10.
J Peripher Nerv Syst ; 29(2): 161-172, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38873841

RESUMO

Monoclonal gammopathy-related peripheral neuropathies encompass a spectrum of clinical presentations in which the monoclonal protein directly damages the tissues, including the peripheral nervous system. Given the prevalence of both peripheral neuropathy and monoclonal gammopathy in the general population, these conditions may overlap in clinical practice, posing a challenge for clinicians in determining causality. Therefore, a comprehensive understanding of primary clinical syndromes and their neurophysiological patterns is of great importance for accurate differential diagnoses and effective treatment strategies. In this article, we examine the main forms of monoclonal gammopathies that affect the peripheral nerve. We explore the clinical and electrophysiological aspects and their correlation with each syndrome's corresponding monoclonal protein type. This knowledge is essential for healthcare professionals to diagnose better and manage patients presenting with monoclonal gammopathy-related peripheral nervous system involvement.


Assuntos
Paraproteinemias , Doenças do Sistema Nervoso Periférico , Humanos , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/etiologia , Doenças do Sistema Nervoso Periférico/fisiopatologia
11.
Med Sci (Basel) ; 12(2)2024 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-38804381

RESUMO

Phospholipidosis is a rare disorder which consists of an excessive intracellular accumulation of phospholipids and the appearance of zebra bodies or lamellar bodies when looking at them using electron microscopy. This disease is associated with certain genetic diseases or is secondary to drugs or toxins. Drug-induced phospholipidosis encompasses many types of pharmaceuticals, most notably chloroquine, amiodarone or ciprofloxacin. Clinically and histologically, renal involvement can be highly variable, with the diagnosis not being made until the zebra bodies are seen under an electron microscope. These findings may require genetic testing to discount Fabry disease, as its histological findings are indistinguishable. Most of the chemicals responsible are cationic amphiphilic drugs, and several mechanisms have been hypothesized for the formation of zebra bodies and their pathogenic significance. However, the relationship between drug toxicity and phospholipid accumulation, zebra bodies and organ dysfunction remains enigmatic, as do the renal consequences of drug withdrawal. We present, to our knowledge, the first case report of acute renal injury with a monoclonal gammopathy of renal significance, lesions, and sclerodermiform syndrome, with zebra bodies that were associated with the initiation of a hydroxychloroquine and amiodarone treatment, as an example of drug-induced-phospholipidosis.


Assuntos
Amiodarona , Hidroxicloroquina , Fosfolipídeos , Humanos , Injúria Renal Aguda/induzido quimicamente , Amiodarona/efeitos adversos , Hidroxicloroquina/efeitos adversos , Hidroxicloroquina/uso terapêutico , Lipidoses/induzido quimicamente , Paraproteinemias/induzido quimicamente , Feminino , Idoso
13.
Arch Dermatol Res ; 316(6): 268, 2024 May 25.
Artigo em Inglês | MEDLINE | ID: mdl-38795175

RESUMO

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by ulcerative painful lesions with violaceous undermined borders. Up to 75% of PG cases develop in association with an underlying systemic disease. Monoclonal gammopathy is reportedly a concomitant condition with PG, with studies indicating immunoglobulin (Ig) A gammopathy as the most common. Whether gammopathy is associated with PG or is an incidental finding has been debated. We sought to investigate the association and characteristics of gammopathy in patients with PG. We retrospectively identified PG patients at our institution from 2010 to 2022 who were screened for plasma cell dyscrasia. Of 106 patients identified, 29 (27%) had a gammopathy; subtypes included IgA (41%), IgG (28%), and biclonal (IgA and IgG) (14%). Mean age was similar between those with and without gammopathy (60.7 vs. 55.9 years; P = .26). In addition, hematologic or solid organ cancer developed in significantly more patients with vs. without gammopathy (8/29 [28%] vs. 5/77 [6%]; P = .003). Among the subtypes of gammopathy, IgG monoclonal gammopathy had the highest proportion of patients with subsequent cancer development (4 of 8 patients, 50%). Study limitations include a retrospective, single-institution design with a limited number of patients. Overall, our data show a high prevalence of gammopathy in patients with PG; those patients additionally had an increased incidence of cancer, especially hematologic cancer.


Assuntos
Paraproteinemias , Pioderma Gangrenoso , Humanos , Pioderma Gangrenoso/diagnóstico , Pioderma Gangrenoso/epidemiologia , Estudos Retrospectivos , Pessoa de Meia-Idade , Feminino , Masculino , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Paraproteinemias/epidemiologia , Paraproteinemias/imunologia , Idoso , Imunoglobulina A/sangue , Imunoglobulina A/imunologia , Adulto , Imunoglobulina G/sangue , Imunoglobulina G/imunologia
14.
Muscle Nerve ; 70(2): 173-179, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38816958

RESUMO

The diagnostic evaluation of a peripheral neuropathy includes testing for the presence of monoclonal gammopathy, which can be found in about 10% of patients with peripheral neuropathy. Our role, as physicians, is to determine whether the neuropathy is directly related to the gammopathy or whether the co-occurrence of these two disorders is purely coincidental. The evaluating physician needs to be familiar with the different types of neuropathies associated with monoclonal gammopathies, their clinical and electrodiagnostic characteristics, and their appropriate diagnostic evaluation and management. Testing for monoclonal protein disorders includes serum protein electrophoresis (SPEP) and immunofixation of blood, and in some cases of urine, as well as measurement of free light chains and quantitative immunoglobulins. Specific antibody testing is directed by paraprotein type and neuropathy phenotype. Patients with abnormal free light chains in association with sensory and autonomic neuropathy should be evaluated for AL amyloidosis. When a lambda monoclonal protein is identified together with a clinical phenotype of chronic inflammatory demyelinating neuropathy (CIDP), a diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome should be considered. Patients with IgM paraprotein associated neuropathy should be assessed for distal acquired demyelinating sensorimotor (DADS) neuropathy, with or without anti myelin associated glycoprotein (MAG) antibody or CANOMAD syndrome. In many cases, a monoclonal gammopathy of uncertain significance (MGUS) is incidental and unrelated to the neuropathy. Collaboration with oncology is critical in evaluating patients with monoclonal proteins to assess for underlying plasma cell neoplasms or B cell lymphomas.


Assuntos
Paraproteinemias , Doenças do Sistema Nervoso Periférico , Humanos , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Doenças do Sistema Nervoso Periférico/diagnóstico
15.
Kidney Int ; 106(2): 201-213, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38723749

RESUMO

Monoclonal Ig crystalline nephropathies are rare lesions resulting from precipitation of monoclonal Igs in the kidney as crystalline inclusions. They can be categorized into lesions with predominant intracellular crystals (light chain [LC] proximal tubulopathy, LC crystal-storing histiocytosis, and LC crystalline podocytopathy) and lesions with predominant extracellular crystals (crystalglobulin-induced nephropathy and crystalline variant of LC cast nephropathy). The majority of these lesions are associated with low tumor burden lymphoproliferative disorders, with the exception of crystalline variant of LC cast nephropathy. Extrarenal involvement (e.g., skin and cornea) is frequent. Kidney biopsy is the cornerstone for diagnosis, which often requires electron microscopy and antigen retrieval. A thorough hematologic workup and evaluation of extrarenal involvement is mandatory for management. Treatment of these lesions is with clone-directed therapy, with the goal of achieving hematologic very good partial response or complete response, which preserves or improves kidney function. In vitro and in vivo studies, animal models, and novel sequencing techniques have been invaluable tools to understand the pathogenesis of LC proximal tubulopathy and can be used to increase our limited knowledge of the pathogenesis of the other monoclonal Ig crystalline nephropathies. This review provides an update on the pathology, renal and hematologic characteristics, extrarenal manifestations, prognosis, treatment, and pathogenesis of monoclonal Ig crystalline nephropathies.


Assuntos
Cristalização , Nefropatias , Humanos , Animais , Nefropatias/patologia , Nefropatias/diagnóstico , Rim/patologia , Rim/efeitos dos fármacos , Anticorpos Monoclonais/uso terapêutico , Biópsia , Cadeias Leves de Imunoglobulina/metabolismo , Paraproteinemias/diagnóstico , Paraproteinemias/patologia , Paraproteinemias/tratamento farmacológico , Paraproteinemias/complicações
16.
Sci Rep ; 14(1): 7667, 2024 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-38561447

RESUMO

Renal involvement is common in monoclonal gammopathy (MG); however, the same patient may have both MG and non-paraprotein-associated renal damage. Accordingly, distinguishing the cause of renal damage is necessary because of the different clinical characteristics and associated treatments. In this multicenter retrospective cohort study, we described the clinicopathological characteristics and prognosis of 703 patients with MG and renal damage in central China. Patients were classified as having MG of renal significance (MGRS), MG of undetermined significance (MGUS), or hematological malignancy. 260 (36.98%), 259 (36.84%), and 184 (26.17%) had MGRS, MGUS, and hematological malignancies, respectively. Amyloidosis was the leading pattern of MGRS (74.23%), followed by thrombotic microangiopathy (8.85%) and monoclonal immunoglobulin deposition disease (8.46%). Membranous nephropathy was the leading diagnosis of MGUS (39.38%). Renal pathological findings of patients with hematological malignancies included paraprotein-associated lesions (84.78%) and non-paraprotein-associated lesions (15.22%). The presence of nephrotic syndrome and an abnormal free light chain (FLC) ratio were independently associated with MGRS. The overall survival was better in patients with MGUS than in those with MGRS or hematological malignancies.


Assuntos
Neoplasias Hematológicas , Nefropatias , Gamopatia Monoclonal de Significância Indeterminada , Paraproteinemias , Humanos , Estudos Retrospectivos , Nefropatias/diagnóstico , Nefropatias/etiologia , Nefropatias/patologia , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/complicações , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Prognóstico , Neoplasias Hematológicas/complicações
17.
Int J Mol Sci ; 25(7)2024 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-38612612

RESUMO

Multiple myeloma (MM) is a plasma cell disorder representing the second most common blood cancer [...].


Assuntos
Neoplasias Hematológicas , Mieloma Múltiplo , Segunda Neoplasia Primária , Paraproteinemias , Humanos , Mieloma Múltiplo/tratamento farmacológico
18.
Clin Exp Med ; 24(1): 69, 2024 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-38578383

RESUMO

Liquid biopsy is a minimally invasive diagnostic tool for identification of tumor-related mutations in circulating cell-free DNA (cfDNA). The aim of this study was to investigate feasibility, sensitivity, and specificity of non-invasive prenatal test (NIPT) for identification of chromosomal abnormalities in cfDNA from a total of 77 consecutive patients with non-Hodgkin B-cell lymphomas, Hodgkin lymphoma (HL), or plasma cell dyscrasia. In this case series, half of patients had at least one alteration, more frequently in chromosome 6 (23.1%), chromosome 9 (20.5%), and chromosomes 3 and 18 (16.7%), with losses of chromosome 6 and gains of chromosome 7 negatively impacting on overall survival (OS), with a 5-year OS of 26.9% and a median OS of 14.6 months, respectively (P = 0.0009 and P = 0.0004). Moreover, B-cell lymphomas had the highest NIPT positivity, especially those with aggressive lymphomas, while patients with plasma cell dyscrasia with extramedullary disease had a higher NIPT positivity compared to conventional cytogenetics analysis and a worse outcome. Therefore, we proposed a NIPT-based liquid biopsy a complementary minimally invasive tool for chromosomal abnormality detection in hematological malignancies. However, prospective studies on larger cohorts are needed to validate clinical utility of NIPT-based liquid biopsy in routinely clinical practice.


Assuntos
Ácidos Nucleicos Livres , Neoplasias Hematológicas , Linfoma de Células B , Paraproteinemias , Gravidez , Feminino , Humanos , Estudos Prospectivos , Hematopoiese Clonal , Aberrações Cromossômicas , Ácidos Nucleicos Livres/genética , Neoplasias Hematológicas/diagnóstico , Neoplasias Hematológicas/genética
19.
Ann Intern Med ; 177(4): 449-457, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38560901

RESUMO

BACKGROUND: Monoclonal gammopathy of undetermined significance (MGUS) and smoldering multiple myeloma (SMM) are asymptomatic precursor conditions to multiple myeloma and related disorders. Smoldering multiple myeloma is distinguished from MGUS by 10% or greater bone marrow plasma cells (BMPC) on sampling, has a higher risk for progression, and requires specialist management. OBJECTIVE: To develop a multivariable prediction model that predicts the probability that a person with presumed MGUS has 10% or greater BMPC (SMM or worse by bone marrow criteria) to inform the decision to obtain a bone marrow sample and compare its performance to the Mayo Clinic risk stratification model. DESIGN: iStopMM (Iceland Screens, Treats or Prevents Multiple Myeloma), a prospective population-based screening study of MGUS. (ClinicalTrials.gov: NCT03327597). SETTING: Icelandic population of adults aged 40 years or older. PATIENTS: 1043 persons with IgG, IgA, light-chain, and biclonal MGUS detected by screening and an interpretable bone marrow sample. MEASUREMENTS: Monoclonal gammopathy of undetermined significance isotype; monoclonal protein concentration; free light-chain ratio; and total IgG, IgM, and IgA concentrations were used as predictors. Bone marrow plasma cells were categorized as 0% to 4%, 5% to 9%, 10% to 14%, or 15% or greater. RESULTS: The c-statistic for SMM or worse was 0.85 (95% CI, 0.82 to 0.88), and calibration was excellent (intercept, -0.07; slope, 0.95). At a threshold of 10% predicted risk for SMM or worse, sensitivity was 86%, specificity was 67%, positive predictive value was 32%, and negative predictive value was 96%. Compared with the Mayo Clinic model, the net benefit for the decision to refer for sampling was between 0.13 and 0.30 higher over a range of plausible low-risk thresholds. LIMITATION: The prediction model will require external validation. CONCLUSION: This accurate prediction model for SMM or worse was developed in a population-based cohort of persons with presumed MGUS and may be used to defer bone marrow sampling and referral to hematology. PRIMARY FUNDING SOURCE: International Myeloma Foundation and the European Research Council.


Assuntos
Gamopatia Monoclonal de Significância Indeterminada , Mieloma Múltiplo , Paraproteinemias , Mieloma Múltiplo Latente , Adulto , Humanos , Gamopatia Monoclonal de Significância Indeterminada/diagnóstico , Gamopatia Monoclonal de Significância Indeterminada/epidemiologia , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/epidemiologia , Mieloma Múltiplo/terapia , Medula Óssea , Estudos de Coortes , Estudos Prospectivos , Imunoglobulina A , Imunoglobulina G , Progressão da Doença
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