Uma multiplicação descontrolada de plasmócitos idênticos, que são células que produzem imunoglobulinas (também chamadas de anticorpos). Essa categoria inclui o mieloma múltiplo, a leucemia de plasmócitos, o plasmocitoma, a doença de deposição de imunoglobulina monoclonal e a gamopatia monoclonal de significado incerto.
Introdução
O que você precisa saber de cara
Uma multiplicação descontrolada de plasmócitos idênticos, que são células que produzem imunoglobulinas (também chamadas de anticorpos). Essa categoria inclui o mieloma múltiplo, a leucemia de plasmócitos, o plasmocitoma, a doença de deposição de imunoglobulina monoclonal e a gamopatia monoclonal de significado incerto.
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Entender a doença
Do básico ao detalhe, leia no seu ritmo
Preparando trilha educativa...
Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 24 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 62 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Triagem neonatal (Teste do Pezinho)
A triagem neonatal permite diagnóstico precoce e início imediato do tratamento.
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
2 genes identificados com associação a esta condição.
Regulatory component of the cyclin D1-CDK4 (DC) complex that phosphorylates and inhibits members of the retinoblastoma (RB) protein family including RB1 and regulates the cell-cycle during G(1)/S transition (PubMed:1827756, PubMed:1833066, PubMed:19412162, PubMed:33854235, PubMed:8114739, PubMed:8302605). Phosphorylation of RB1 allows dissociation of the transcription factor E2F from the RB/E2F complex and the subsequent transcription of E2F target genes which are responsible for the progression
NucleusCytoplasmNucleus membrane
DNA ligase involved in DNA non-homologous end joining (NHEJ); required for double-strand break (DSB) repair and V(D)J recombination (PubMed:12517771, PubMed:17290226, PubMed:23523427, PubMed:29980672, PubMed:33586762, PubMed:8798671, PubMed:9242410, PubMed:9809069). Catalyzes the NHEJ ligation step of the broken DNA during DSB repair by resealing the DNA breaks after the gap filling is completed (PubMed:12517771, PubMed:17290226, PubMed:9242410, PubMed:9809069). Joins single-strand breaks in a d
Nucleus
LIG4 syndrome
Characterized by immunodeficiency and developmental and growth delay. Patients display unusual facial features, microcephaly, growth and/or developmental delay, pancytopenia, and various skin abnormalities.
Variantes genéticas (ClinVar)
274 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
20 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Tumor de plasmócitos
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
🟢 Recrutando agora
13 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
420 ensaios clínicos encontrados, 22 ativos.
Publicações mais relevantes
Pneumocystis jirovecii infection in autologous hematopoietic stem cell transplantation.
Pneumocystis jirovecii pneumonia (PJP) after autologous stem cell transplantation (ASCT) remains insufficiently characterized. We retrospectively analyzed 304 ASCT recipients (2005-2024) at a single center (B-cell lymphoma n = 138; plasma cell tumor n = 126). Fourteen patients (4.6%) developed PJP at a median of 143 days (86-286) post-ASCT; none were on prophylaxis at diagnosis. The 1-year cumulative incidence was 5.2% overall, and varied by disease: 24% in follicular lymphoma (FL), 5.9% in other B-cell lymphomas, 1.9% in plasma cell tumor, and 0% in T-cell lymphomas. Prior rituximab exposure (HR 6.75; 95% CI 1.50-30.2) and FL diagnosis (HR 7.26; 95% CI 2.51-20.9) were associated with higher risk; age, purine analogs, lymphocyte count, globulin/IgG, and conditioning were not. No death was directly attributable to PJP. These data suggest, diagnosis-dependent risk after ASCT; prophylaxis may be considered for rituximab-treated patients, especially FL, while the optimal duration warrants further study.
Conjunctival plasmacytoma: A case study.
Plasmacytomas are localized collections of monoclonal plasma cells. This is a 48-year-old female patient of mixed race, with no significant medical history, with a soft, painless tumor adhered to the sclera, in the bulbar conjunctiva and inferior temporal fornix. The method used was the presentation of a clinical case. The tumor was excised with pathological analysis and immunohistochemical study confirming the diagnosis of conjunctival plasmacytoma. Postoperative follow-up was uneventful. Extraosseous plasma cell tumors located in the eye are rare and difficult to diagnose because they present a wide range of clinical differentiation.
Intramuscular enhancing masses: Late relapse of multiple myeloma mimicking hematoma in trauma setting.
Extramedullary disease (EMD) is an uncommon and aggressive form of multiple myeloma, especially when localized to intramuscular locations. We present a case of a 45-year-old man with a history of IgG multiple myeloma (MM), who underwent allogeneic bone marrow transplantation and had been in clinical remission for more than a decade. He presented acutely after trauma with computed tomography (CT) revealed several enhancing masses in bilateral psoas, right obturator internus, right adductors and left sartorius muscles, which were initially misinterpreted as intramuscular hematomas. These masses remained stable on follow-up imaging, raising concern for underlying undiagnosed soft tissue tumors. Biopsy of one of the masses demonstrated a plasma cell tumor, and further evaluation confirmed relapse of multiple myeloma. Additionally, the largest mass in the left psoas exhibited peripheral calcification-an unusual imaging characteristic of extra ossous myeloma. Despite imaging following trauma, the presence of intramuscular masses in patients with history of MM should raise a high degree of clinical suspicion for EMD. Recognition of this diagnostic pitfall can help avoid delay diagnosis and inappropriate management.
Ethmoid tumors in free-ranging white-tailed deer (Odocoileus virginianus).
This report describes a series of ethmoid tumors in 6, free-ranging white-tailed deer (Odocoileus virginianus; WTD) in southwest to central Texas, which included 4 plasma cell tumors, 1 adenosquamous carcinoma, and 1 adenocarcinoma. The plasma cell tumors had a distinctive presentation with unilateral to bilateral facial masses located rostromedial to the eyes that were smooth and fluctuant. Upon dissection, these preorbital facial masses consisted of multiloculated, mucus-filled, cystic pockets with 2 having partial extension of the ethmoid tumors into the external facial masses. The 2 carcinomas were larger, unilateral, solid facial masses. All tumors caused extensive destruction of the ethmoid region with the carcinomas being paler, firmer, and more invasive into surrounding areas compared with the plasma cell tumors. Ancillary testing did not reveal an underlying infectious cause, although these reports of multiple WTD across a localized region suggest a possible infectious, environmental, or other shared stimulus.
Case Report: Ultrasonographic and computed tomographic features of presumed gastric plasmacytoma (plasma cell tumor) with ulceration in two dogs.
Gastric plasmacytoma is rare in dogs, with only four previous reports providing limited descriptions of abdominal ultrasound (AUS) and computed tomography (CT) findings. This study presents the AUS and CT imaging features of two histopathologically suspected gastric plasmacytomas. Combining the four previous and two new cases, these tumors predominantly occurred in the greater curvature and pyloric antrum as solitary round masses connected to the submucosal or transmural layers. Pre- and post-contrast tissue attenuation (Hounsfield unit; HU) differences exceeded 40, with variable AUS echogenicity and echotexture. Imaging features may resemble those of spindle cell tumors, requiring histological and immunohistochemical examinations for definitive diagnosis.
Publicações recentes
EXPRESS: Feline tarsal tumors: histologic spectrum, risk factors, and prognostic insights.
Craniofacial extramedullary plasmacytoma with hard palate and bilateral cervical lymph node involvement: a diagnostic challenge and literature review.
Primary extramedullary plasmacytoma of the kidney: a case report and literature review.
Pneumocystis jirovecii infection in autologous hematopoietic stem cell transplantation.
Conjunctival plasmacytoma: A case study.
📚 EuropePMC100 artigos no totalmostrando 109
Pneumocystis jirovecii infection in autologous hematopoietic stem cell transplantation.
Leukemia & lymphomaConjunctival plasmacytoma: A case study.
Archivos de la Sociedad Espanola de OftalmologiaIntramuscular enhancing masses: Late relapse of multiple myeloma mimicking hematoma in trauma setting.
Radiology case reportsPrognostic value assessment and in vitro validation of mitochondria-ferroptosis-related genes in multiple myeloma.
Scientific reportsCyanidin-3-O-glucoside-mediated reduction of regulatory T cells: a therapeutic strategy for multiple myeloma.
BMC immunology[Multiple Myeloma Without Monoclonal Component: Nonsecretory and Non-Producing. Case Report and Review].
Revista medica de ChileCase Report: Ultrasonographic and computed tomographic features of presumed gastric plasmacytoma (plasma cell tumor) with ulceration in two dogs.
Frontiers in veterinary scienceUnexplained cardiac hypertrophy as a clue to plasma cell tumour: a case study.
American journal of translational researchEthmoid tumors in free-ranging white-tailed deer (Odocoileus virginianus).
Veterinary pathologySolitary Plasmacytomas: Current Status in 2025.
Hematology reportsCase report and comprehensive literature review: A rare instance of solitary extramedullary plasmacytoma in the trachea.
Respiratory medicine case reportsSPAG6 Promotes Multiple Myeloma Through Activation of the MAPK/ERK Signaling Pathway.
Frontiers in pharmacologyFirst-line anti-BCMA CAR-T cell therapy in a fragile patient with biclonal gammopathy and giant plasma cell tumor multiple myeloma with multiple comorbidities: a case report.
Frontiers in immunologyReview of tracheal neoplasia in dogs and cats (1961-2024).
Veterinary pathologyCanine laryngotracheal plasma cell tumors: Ten cases and literature review.
Veterinary pathology[Risk stratified thromboprophylaxis and management of thrombotic events in plasma cell neoplasms].
Zhonghua yi xue za zhiPlasma Cell-Rich Lymphoid Neoplasm in an Endocervical Polyp of the Uterine Cervix in a Perimenopausal 52-Year-Old Woman.
HCA healthcare journal of medicineFIRST REPORT OF TUMORS IN GARDEN DORMICE (ELIOMYS QUERCINUS).
Journal of zoo and wildlife medicine : official publication of the American Association of Zoo Veterinarians[Analysis and reflections on the current status of diagnosis and treatment of marginal zone lymphoma].
Zhonghua yi xue za zhiA Rare Case of Solitary Endobronchial Plasmacytoma Presented With Complete Airway Obstruction: A Case Report.
Journal of investigative medicine high impact case reports2-Dimensional Operative Video for Staged Treatment of a Giant Plasma Cell Tumor: Principles for Neurosurgical Oncology During a Wartime Environment in Ukraine.
Operative neurosurgery (Hagerstown, Md.)Transendoscopic laser ablation of an esophageal plasma cell tumor in a cat.
JFMS open reportsAcquired Bortezomib Resistance in Multiple Myeloma: From Mechanisms to Strategy.
Current treatment options in oncologyMandibular salivary gland plasma cell tumor in a dog.
Topics in companion animal medicineChanges in the immune microenvironment during plasma cell tumor development in the IL6Myc mouse model of human multiple myeloma.
Experimental cell researchA "gullwing sign" on magnetic resonance imaging of extradural spinal tumors in dogs and cats allows prioritization of round cell neoplasia.
Veterinary radiology & ultrasound : the official journal of the American College of Veterinary Radiology and the International Veterinary Radiology AssociationPrognostic factors in Chinese patients with immunoglobulin light chain amyloidosis: a scoping review and meta-analysis.
Annals of medicineInterference with PLA2G16 promotes cell cycle arrest and apoptosis and inhibits the reprogramming of glucose metabolism in multiple myeloma cells by modulating the Hippo/YAP signaling pathway.
Anti-cancer drugsRefining the relationship between gut microbiota and common hematologic malignancies: insights from a bidirectional Mendelian randomization study.
Frontiers in cellular and infection microbiologyCutaneous plasmacytoma with Mott cell differentiation in a dog.
Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, IncSuccessful Treatment of Refractory Orbital Plasmacytoma with Chimeric Antigen Receptor T Cell Therapy: A Case Report and Review of the Literature.
Ophthalmic plastic and reconstructive surgerySolitary Extramedullary Plasmacytoma of the Left Lower Eyelid: A Case Report and Review of Literature.
CureusSolitary ovarian plasmacytoma: About a rare entity of extramedullary plasma cell tumor.
International journal of surgery case reportsOutcomes in dogs undergoing surgical stabilization and non-stereotactic radiation therapy for axial and appendicular bone tumors.
Frontiers in veterinary scienceRenal Riddles Unveiled: Decoding Homogeneous Kidney Enlargement in Multiple Myeloma.
CureusCase report: Corpectomy and iliac crest bone autograft as treatment for a vertebral plasma cell tumor in a dachshund dog.
Frontiers in veterinary scienceSolitary plasmacytoma of mandible: A case report.
Radiology case reportsMyocardial plasma cell tumor with intratumoral amyloidosis in a dog.
Journal of veterinary cardiology : the official journal of the European Society of Veterinary CardiologyRespiratory Distress Caused by Neoplastic Upper Respiratory Tract Obstructions in 2 Pet Rats (Rattus norvegicus domestica).
Topics in companion animal medicineDrug Expenditure, Price, and Utilization in US Medicaid: A Trend Analysis for New Multiple Myeloma Medications from 2016 to 2022.
Healthcare (Basel, Switzerland)Soft Tissue Mass Extramedullary Plasmacytoma Following Radiation Therapy for Solitary Bone Plasmacytoma.
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Biological trace element researchA case report of POEMS syndrome with lower limb numbness and weakness.
Asian journal of surgerySolitary Extramedullary Plasmacytoma of the Head and Neck: A Report of Three Cases Treated With Curative Radiotherapy and a Review of the Dose-Control Relationship.
CureusSolitary Plasmacytoma in the Calcaneus.
CureusA Patient with Synchronous Gallbladder and Bone Plasmacytoma.
Diagnostics (Basel, Switzerland)[Recent Research Progress of Extramedullary Plasmacytoma --Review].
Zhongguo shi yan xue ye xue za zhiNot Your Everyday Duodenal Ulcer: Massive Gastrointestinal Bleed Secondary to Extramedullary Plasma Cell Myeloma.
ACG case reports journalPolyneuropathy organomegaly endocrinopathy M-protein and skin changes syndrome with ascites as an early-stage manifestation: A case report.
World journal of clinical casesDiagnosis and management of extramedullary plasmacytoma in nasal cavity: Clinical experience and literature review.
MedicineMultiple Myeloma With Retroperitoneal Extramedullary Plasmacytoma Causing Renal Failure and Obstructive Shock From Inferior Vena Cava Compression: A Case Report.
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BiomoleculesSpheno-Orbital Plasmacytoma as an Initial Presentation of Multiple Myeloma.
Brain tumor research and treatmentCholangioscopic diagnosis of hemobilia: an unusual case of left hepatic portal hypertension by plasma cell tumor.
VideoGIE : an official video journal of the American Society for Gastrointestinal EndoscopySpatial Proteomics Reveals Differences in the Cellular Architecture of Antibody-Producing CHO and Plasma Cell-Derived Cells.
Molecular & cellular proteomics : MCPSuprasellar Plasmacytoma Leading to the Diagnosis of Multiple Myeloma.
Cureus[Extramedullary plasmacytoma of the larynx:a case report].
Lin chuang er bi yan hou tou jing wai ke za zhi = Journal of clinical otorhinolaryngology head and neck surgeryA Rare Case of Hormone-Induced Plasma Cell Granuloma of the Gingiva.
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Veterinary pathology[Primary extramedullary plasma cell tumor of the right kidney: report of a case].
Zhonghua bing li xue za zhi = Chinese journal of pathologyIgG4-Related Kidney Disease Associated With End-Stage Kidney Disease, Renal Pseudotumor, and Renal Vein Thrombosis.
CureusTim-3 Blockade Elicits Potent Anti-Multiple Myeloma Immunity of Natural Killer Cells.
Frontiers in oncologyAre the Derived Indexes of Peripheral Whole Blood Cell Counts (NLR, PLR, LMR/MLR) Clinically Significant Prognostic Biomarkers in Multiple Myeloma? A Systematic Review And Meta-Analysis.
Frontiers in oncologySolitary bone plasmacytoma of spine with involvement of adjacent disc space: A case report.
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World journal of clinical casesImpact of hypoxia on the pathogenesis and therapy resistance in multiple myeloma.
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AIDS research and therapySpinal cord compression due to a solitary, bulky plasmacytoma of posterior mediastinum.
Radiology case reportsLymphoma in Psittacine Birds: A Histological and Immunohistochemical Assessment.
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Thoracic cancerMagnetic Resonance Imaging Features of Extradural Spinal Neoplasia in 60 Dogs and Seven Cats.
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Journal of ophthalmologySuper-Enhancer Associated Five-Gene Risk Score Model Predicts Overall Survival in Multiple Myeloma Patients.
Frontiers in cell and developmental biologyMouse tumor susceptibility genes identify drug combinations for multiple myeloma.
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Annals of medicine and surgery (2012)Quantification by Ultrafiltration and Immunofixation Electrophoresis Testing for Monoclonal Serum Free Light Chains.
Laboratory medicineLong non-coding RNA PVT1 regulates the migration of hepatocellular carcinoma HepG2 cells via miR-3619-5p/MKL1 axis.
Bosnian journal of basic medical sciencesClinical Features of Head and Neck Solitary Extramedullary Plasmacytoma in Taiwan.
In vivo (Athens, Greece)Plasma cell leukemia with plasmablastic morphology in a dog.
Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, IncPrimary plasmablastic plasmacytoma in the stomach of an immunocompetent adult: A case report.
MedicinePrediction and prognostic significance of BCAR3 expression in patients with multiple myeloma.
Journal of translational medicineTotal En Bloc Spondylectomy for Lumbar Spinal Tumors by Paraspinal Approach.
World neurosurgeryTonsillar plasmacytoma in a dog.
The Canadian veterinary journal = La revue veterinaire canadienneSolitary plasmacytoma of the jaws: therapeutical considerations and prognosis based on a case reports systematic survey.
Brazilian journal of otorhinolaryngologyA Case of Concurrent Gastric and Pancreatic Plasmacytomas in a Patient With Multiple Myeloma: An Extremely Rare Entity.
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International journal of molecular sciencesAutologous tumor cells/bacillus Calmette-Guérin/formalin-based novel breast cancer vaccine induces an immune antitumor response.
OncotargetTargeting MYC in multiple myeloma.
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Veterinary pathologyAnaplastic plasmacytoma: a rare tumor presenting as a pathological fracture in a younger adult.
Skeletal radiologyIntravascular neoplastic cells in canine cutaneous plasmacytomas.
Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, IncWaldenström's macroglobulinemia: Two malignant clones in a monoclonal disease? Molecular background and clinical reflection.
European journal of haematologyCurrent treatment options of T cell-associated immunotherapy in multiple myeloma.
Clinical and experimental medicineEZH2 inhibition in multiple myeloma downregulates myeloma associated oncogenes and upregulates microRNAs with potential tumor suppressor functions.
OncotargetExtramedullary Plasmacytoma Mimicking Pancreatic Cancer: An Unusual Presentation.
Case reports in oncological medicineProceedings of the 2016 National Toxicology Program Satellite Symposium.
Toxicologic pathologyCytology of plasma cell rich effusion in cases of plasma cell neoplasm.
Journal of cytologyRevisiting IL-6 antagonism in multiple myeloma.
Critical reviews in oncology/hematologyPlasmacytoma to the Axis Mimicking Metastatic Paraganglioma: Circumferential Reconstruction via Posterior Approach.
Korean Journal of SpineResident Rounds: Part III--Case Report: Metastatic Multiple Myeloma.
Journal of drugs in dermatology : JDDNon-secreting multiple myeloma switches to IgD of lamda type: a case report and review of literature.
International journal of clinical and experimental medicineDisentangling the microRNA regulatory milieu in multiple myeloma: integrative genomics analysis outlines mixed miRNA-TF circuits and pathway-derived networks modulated in t(4;14) patients.
OncotargetBone formation following lenalidomide-dexamethasone combination therapy in cases of multiple myeloma refractory to high-dose chemotherapy with bortezomib and autologous peripheral blood stem cell transplantation: report of a case and review of the literature.
International journal of clinical and experimental pathologySerous macular detachment, yellow macular deposits, and prominent middle limiting membrane in multiple myeloma.
Therapeutics and clinical risk managementOsseous plasma cell neoplasm of the mandible for initial diagnosis of multiple myeloma: case report and literature review.
Journal of maxillofacial and oral surgeryThe tumor microenvironment shapes hallmarks of mature B-cell malignancies.
OncogeneAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Comunidades
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Pneumocystis jirovecii infection in autologous hematopoietic stem cell transplantation.
- Conjunctival plasmacytoma: A case study.
- Intramuscular enhancing masses: Late relapse of multiple myeloma mimicking hematoma in trauma setting.
- Ethmoid tumors in free-ranging white-tailed deer (Odocoileus virginianus).
- Case Report: Ultrasonographic and computed tomographic features of presumed gastric plasmacytoma (plasma cell tumor) with ulceration in two dogs.
- EXPRESS: Feline tarsal tumors: histologic spectrum, risk factors, and prognostic insights.
- Craniofacial extramedullary plasmacytoma with hard palate and bilateral cervical lymph node involvement: a diagnostic challenge and literature review.
- Primary extramedullary plasmacytoma of the kidney: a case report and literature review.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98282(Orphanet)
- MONDO:0004959(MONDO)
- GARD:19440(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q18557283(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
