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1.
Neurology ; 103(7): e209879, 2024 Oct 08.
Artigo em Inglês | MEDLINE | ID: mdl-39236269

RESUMO

Approaching patients with paraproteinemic neuropathies can be challenging for the practicing neurologist, and a well-defined strategy considering specific etiologies is necessary to arrive at the correct diagnosis. In this case, a 49-year-old man presented with a 2-year history of progressive upper then lower extremity numbness, weakness, gait instability, and tremors. His examination was marked by proximal and distal symmetric upper and lower extremity weakness, large more than small-fiber sensory loss, prominent sensory ataxia, action and postural tremors, and globally absent deep tendon reflexes. His workup was notable for a chronic demyelinating sensorimotor polyradiculoneuropathy and a monoclonal immunoglobulin (Ig) M kappa gammopathy. This case highlights the approach to a patient with a rare subtype of IgM paraproteinemic neuropathy with a review of the differential diagnoses, red flag features of co-occurring hematologic disorders, and guided workup. We further discuss typical features of this rare diagnosis and therapeutic options.


Assuntos
Raciocínio Clínico , Transtornos Neurológicos da Marcha , Hipestesia , Paraproteinemias , Tremor , Humanos , Masculino , Pessoa de Meia-Idade , Tremor/diagnóstico , Tremor/etiologia , Hipestesia/etiologia , Hipestesia/diagnóstico , Transtornos Neurológicos da Marcha/etiologia , Transtornos Neurológicos da Marcha/diagnóstico , Paraproteinemias/complicações , Paraproteinemias/diagnóstico , Diagnóstico Diferencial
2.
Ugeskr Laeger ; 186(29)2024 Jul 15.
Artigo em Dinamarquês | MEDLINE | ID: mdl-39115215

RESUMO

Current guidelines recommend screening with serum M-protein and serum-free light chain analysis (S-FLC) when an M-protein-related disorder is suspected. Many patients with multiple myeloma will be overlooked if only serum M-protein is measured. Despite this, the general practitioners in some areas of Denmark cannot order S-FLC. This review aims to disseminate knowledge of the S-FLC analysis, its applicability, and limitations in the diagnostic workup for suspected monoclonal gammopathies.


Assuntos
Cadeias Leves de Imunoglobulina , Mieloma Múltiplo , Humanos , Cadeias Leves de Imunoglobulina/sangue , Mieloma Múltiplo/diagnóstico , Mieloma Múltiplo/sangue , Paraproteinemias/diagnóstico , Paraproteinemias/sangue , Proteínas do Mieloma/análise
4.
Clin Exp Med ; 24(1): 198, 2024 Aug 24.
Artigo em Inglês | MEDLINE | ID: mdl-39180586

RESUMO

The clinical significance of an abnormal free light chain (FLC) test, performed due to unspecific complains in the absence of a known plasma cell dyscrasia (PCD) or lymphoproliferative disease (LPD), is not fully elucidated. We investigated the importance of an abnormal FLC ratio (FLC-R) in this setting. Patients registered in the Maccabi Healthcare Services database, tested for FLC during 2007-2023 without previously documented PCD/LPD or increased total protein (TP) level, were reviewed. Demographics, co-morbidities, and laboratory tests were recorded. FLC-R was defined as normal (0.26-1.65) or slightly (slAb 0.1-0.26/1.65-4), moderately (mAbn 0.1-0.05/4-8) and significantly abnormal (sigAb- < 0.05 or > 8). Factors associated with PCD/LPD and overall survival were identified. In total, 8,661 patients, 2,215 (25.6%) with abnormal FLC-R [2,090 (24.1%)-slAb, 65 (0.75%)-mAbn and 60 (0.7%)-sigAb], were analyzed. Almost none had anemia nor acute renal failure. 14% had concomitant increased immunoglobulins. Within a median follow-up of 52 months, 943 were diagnosed with PCD (816-MGUS, 127-MM/Amyloidosis/plasmacytoma) and 48 with LPD. Median time to PCD and LPD were 19 and 28 months. Multivariate analysis found slAb (HR = 1.8, CI95%:1.53-2.12, p < 0.001), mAbn (HR = 6.3, CI95%:4.16-9.53, p < 0.001), and sigAb FLC (HR = 10.4, CI95%:7.0-15.35, p < 0.001), to be associated with PCD/LPD diagnosis. Decreased IgG, increased IgA, and concomitant comorbidities predicted PCD, whereas increased IgM predicted LPD. Older age, male gender, anemia, decreased albumin, increased IgG and concomitant comorbidities, predicted shorter survival. Our large study emphasizes the independent clinical significance of abnormal FLC-R as a predictor of PCD/LPD diagnosis even in patients with normal TP level, promoting early detection of PCD/LPD.


Assuntos
Cadeias Leves de Imunoglobulina , Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Cadeias Leves de Imunoglobulina/sangue , Adulto , Paraproteinemias/sangue , Paraproteinemias/diagnóstico , Estudos Retrospectivos , Análise de Sobrevida , Transtornos Linfoproliferativos/diagnóstico , Transtornos Linfoproliferativos/sangue , Transtornos Linfoproliferativos/mortalidade , Doenças Hematológicas/sangue , Relevância Clínica
5.
Lupus Sci Med ; 11(2)2024 Aug 30.
Artigo em Inglês | MEDLINE | ID: mdl-39216876

RESUMO

OBJECTIVES: Rheumatic diseases were previously associated with increased incidence of monoclonal gammopathy (MG) and its malignant transformation. The present study aimed to investigate the prevalence, malignant transformation risk, clinical correlates and prognostic impact of MG in SLE. METHODS: A retrospective cohort study based on the medical records of n=1039 patients with SLE fulfilling the 1997 American College of Rheumatology (ACR), the 2019 European Alliance of Associations for Rheumatology (EULAR)/ACR and/or the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria managed at two tertiary care departments of the University Hospital (Krakow, Poland) from January 2012 until November 2019. RESULTS: SLE+MG cases were older at SLE diagnosis compared with non-MG SLE controls (53±15 years vs 37±15 years, respectively, p<0.01), had higher rates of lymphopenia, anaemia, haemolysis, serous effusions and interstitial lung disease (all p<0.05), and were more frequently treated with cyclophosphamide (57% vs 28%, p<0.01) or rituximab (13% vs 3%, p<0.01). Most MG cases were detected within a year after SLE diagnosis (Q25, Q75: 0, 12 years). With the median follow-up of 11 years (Q25, Q75: 6, 19 years), 34.8% (8 cases) of the SLE+MG cohort were diagnosed with malignancy, compared with 8.1% (82 cases) among the SLE controls (p<0.001). MG was associated with the relative hazard of death of HR 2.99 (95% CI 1.26 to 7.06, p<0.05) and a median survival time from SLE diagnosis to death of 5 years (Q25, Q75: 1, 14; range 0-41) for SLE+MG cases, as compared with 12 years (Q25, Q75: 6, 19; range 0-62) for the controls. The effect was non-independent on antimalarial medication use. CONCLUSIONS: Our study emphasises heightened malignancy and mortality rates in SLE+MG cases. The association between immunosuppression, MG incidence and progression warrants further research.


Assuntos
Lúpus Eritematoso Sistêmico , Neoplasias , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/mortalidade , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Estudos Retrospectivos , Pessoa de Meia-Idade , Feminino , Masculino , Adulto , Idoso , Neoplasias/mortalidade , Neoplasias/complicações , Neoplasias/epidemiologia , Paraproteinemias/complicações , Paraproteinemias/mortalidade , Paraproteinemias/epidemiologia , Polônia/epidemiologia , Ciclofosfamida/uso terapêutico , Prognóstico , Prevalência
6.
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
10.
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
11.
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
12.
Adv Kidney Dis Health ; 31(4): 358-373, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39084761

RESUMO

Paraproteinemias are a group of complex diseases associated with an overproduction of a monoclonal immunoglobulin that can cause a diversity of kidney disorders and end-organ damage. In this review, we focus on paraprotein-mediated glomerular diseases. Kidney biopsy plays a crucial role in diagnosing these disorders, enabling the identification of specific histological patterns. These lesions are categorized into organized (such as amyloidosis, immunotactoid glomerulopathy, fibrillary glomerulonephritis, cryoglobulinemic glomerulonephritis, and monoclonal crystalline glomerulopathies) and nonorganized deposits (such as monoclonal Ig deposition disease and proliferative glomerulonephritis with monoclonal Ig deposits) based on the characteristics of immunofluorescence findings and the ultrastructural appearance of deposits on electron microscopy. This review aims to provide an update, highlight, and discuss clinicopathological aspects such as definition, epidemiology, clinical manifestations, mechanisms of kidney injury, histological features, and diagnostic procedures.


Assuntos
Glomerulonefrite , Glomérulos Renais , Paraproteinemias , Humanos , Paraproteinemias/patologia , Paraproteinemias/diagnóstico , Paraproteinemias/metabolismo , Glomerulonefrite/patologia , Glomerulonefrite/metabolismo , Glomerulonefrite/diagnóstico , Glomérulos Renais/patologia , Glomérulos Renais/metabolismo , Paraproteínas/metabolismo , Nefropatias/patologia , Nefropatias/metabolismo , Biópsia , Amiloidose/patologia , Amiloidose/metabolismo , Amiloidose/diagnóstico
13.
Ann Hematol ; 103(9): 3787-3793, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-39078435

RESUMO

TEMPI syndrome is a rare, acquired disorder with multisystemic manifestations. It is classified as a plasma cell disorder and is characterized by telangiectasias, erythrocytosis, monoclonal gammopathy, perinephric fluid collections and intrapulmonary shunt. Even though TEMPI's pathophysiology remains elusive, it responds to anti-myeloma therapy indicating that the monoclonal protein or clone plays a key role. We present a challenging case of a 73-year-old man with erythrocytosis and deteriorating renal function with nephrotic-range proteinuria in whom after extensive work up, the diagnosis of TEMPI syndrome was made. He was received treatment with daratumumab-bortezomib-cyclophosphamide and dexamethasone (Dara-VCD) and achieved a hematological and clinical response. We also report preliminary data on a multiplex assay for cytokines and growth factors for two patients with TEMPI syndrome and note lower levels for non-specific innate immunity related cytokines. A direct link between renal impairment and TEMPI syndrome is not currently established; cytokine deregulation could potentially be involved in the ischemic changes observed in the renal biopsy of our patient.


Assuntos
Policitemia , Humanos , Idoso , Masculino , Policitemia/diagnóstico , Policitemia/terapia , Paraproteinemias/diagnóstico , Paraproteinemias/complicações , Síndrome , Ciclofosfamida/uso terapêutico , Ciclofosfamida/administração & dosagem , Bortezomib/uso terapêutico , Bortezomib/administração & dosagem , Anticorpos Monoclonais/uso terapêutico , Anticorpos Monoclonais/administração & dosagem , Dexametasona/uso terapêutico , Dexametasona/administração & dosagem , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico
14.
Clin Rheumatol ; 43(9): 3019-3028, 2024 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-38990379

RESUMO

IgG4-related diseases (IgG-RDs) are a group of fibroinflammatory diseases that affect a variety of tissues, resulting in tumour-like effects and/or organ dysfunction. Monoclonal gammopathies (MGPs) are a group of disorders characterized by clonal proliferation of plasma cells or lymphoid cells resulting in the secretion of a monoclonal immunoglobulin. Cases of MGPs in IgG4-RDs coexisting with plasma cell dyscrasias and lymphoid neoplasms have been reported over the past few years. Therefore, the results of examinations of M protein in IgG4-RD patients should be interpreted with caution. Herein, we report the case of a 58-year-old male with a history of type 2 diabetes who presented with submandibular masses, anosmia, swollen lymph nodes, proteinuria, and renal impairment. Laboratory tests revealed hyperglobulinemia and elevated levels of IgG4 (124 g/L) and serum-free light chains (sFLCs). Serum protein electrophoresis (SPEP) revealed an M spike of 5.6 g/dL, and immunofixation electrophoresis (IPE) revealed biclonal IgG-κ and IgG-λ. The patient underwent bone marrow, lymph node, and kidney biopsy, which ruled out plasma cell disorders and lymphoma. He was finally diagnosed with an IgG4-RD comorbid with diabetic nephropathy. The findings in this case highlight that significant activation of B cells in IgG4-RD patients, especially those with multiorgan involvement can lead to significant hyperglobulinemia and high sFLC and IgG4 levels, which are more pronounced in the setting of renal impairment. Relatively high concentrations of polyclonal IgG4 can give rise to a focal band bridging the ß and γ fractions, which may mimic the appearance of a monoclonal band on SPEP and monoclonal gammaglobulinemia in IFE. The patient experienced considerable improvement in his symptoms after rituximab combined with glucocorticoid therapy, and a monoclonal immunoglobulin was not detected.


Assuntos
Doença Relacionada a Imunoglobulina G4 , Imunoglobulina G , Paraproteinemias , Humanos , Masculino , Pessoa de Meia-Idade , Imunoglobulina G/sangue , Doença Relacionada a Imunoglobulina G4/diagnóstico , Doença Relacionada a Imunoglobulina G4/complicações , Paraproteinemias/diagnóstico , Paraproteinemias/imunologia , Paraproteinemias/complicações , Diagnóstico Diferencial , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/imunologia , Diabetes Mellitus Tipo 2/diagnóstico
15.
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
16.
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
17.
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
18.
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
19.
Ann Hematol ; 103(8): 3005-3013, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38842565

RESUMO

The diagnosis of multiple myeloma requires detection of paraproteinemia and confirmation of monoclonal bone marrow infiltration, along with signs of end-organ damage. Despite the increasing prevalence, serum paraproteinemia is not routinely measured. We examined the relationship between alterations in routine hematological parameters and the development of paraproteinemia in a case-control study. Data was retrieved from a laboratory database in the capital region of Denmark between 01/01/2012 and 31/12/2022. Patients were included if they had a test for paraproteinemia (n = 134,740) and at least one prior hematological parameter (white blood cells, hemoglobin and platelet count) with a minimum follow-up of 1 year.Between 96,999 and 103,590 patients were included in each of the three hematological groups. We found white blood cell count and the presence of paraproteinemia followed an inverse J-shaped curve, with the highest presence below 3 × 109/L and above > 9 × 109/L. The adjusted OR below and above the nadir of 4 × 109/L was 1.61 (95% CI 1.25; 2.08, p < 0.0001) and 1.03 (95% CI 1.03; 1.04, p < 0.0001). Hemoglobin levels were inversely associated the presence of paraproteinemia, with the highest association below 6 mmol/L with an OR of 1.30 (95% CI 1.28; 1.32, p < 0.0001) adjusted for age and gender. Platelet count followed a U-shaped curve with the highest association at < 100 × 109/L. The adjusted OR below and above the nadir of 250 × 109/L was 1.13 (95% CI 1.10; 1.17, p < 0.0001) and 1.10 (95% CI 1.08; 1.12, p < 0.0001) respectively. In conclusion, all three parameters showed significant association with later paraproteinemia.


Assuntos
Paraproteinemias , Humanos , Masculino , Feminino , Estudos de Casos e Controles , Pessoa de Meia-Idade , Idoso , Paraproteinemias/sangue , Idoso de 80 Anos ou mais , Mieloma Múltiplo/sangue , Mieloma Múltiplo/epidemiologia , Mieloma Múltiplo/diagnóstico , Dinamarca/epidemiologia , Adulto , Contagem de Plaquetas , Contagem de Leucócitos , Hemoglobinas/análise
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