A Hiperplasia Linfoide Nodular Pulmonar (PNHL) é um aumento de células de defesa (linfócitos) que ocorre nos pulmões, como uma reação do organismo (não é câncer). Ela se manifesta como nódulos (pequenas massas), que podem ser um único ou vários, dentro do pulmão.
Introdução
O que você precisa saber de cara
A hiperplasia linfoide nodular pulmonar é um aumento benigno e reacional de células de defesa (linfócitos) no tecido pulmonar, formando um ou vários nódulos. A pessoa pode sentir tosse, dor no peito ou falta de ar, embora muitas vezes as massas sejam encontradas por acaso em tomografias do tórax. Por se assemelhar a lesões malignas nos exames de imagem, o diagnóstico exige cuidados para comprovar que não se trata de câncer. Esta condição rara não possui Protocolo Clínico (PCDT) próprio nem remédios dispensados de forma específica pelo SUS, sendo o suporte conduzido na rede de saúde conforme os sintomas apresentados.
A Hiperplasia Linfoide Nodular Pulmonar (PNHL) é um aumento de células de defesa (linfócitos) que ocorre nos pulmões, como uma reação do organismo (não é câncer). Ela se manifesta como nódulos (pequenas massas), que podem ser um único ou vários, dentro do pulmão.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
Encontrou um erro ou informação desatualizada? Sugira uma correção →
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
+ 3 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 10 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
Encontrou um erro ou informação desatualizada? Sugira uma correção →
Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
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 — Hiperplasia linfoide nodular pulmonar
Selecione um estado ou use sua localização para ver resultados.
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
Pesquisa e ensaios clínicos
Nenhum ensaio clínico registrado para esta condição.
Publicações mais relevantes
Pulmonary Nodular Lymphoid Hyperplasia Presenting With a Reversed Halo Sign.
The reversed halo sign is observed in a broad spectrum of lung diseases, most commonly organising pneumonia, but it has not been reported in pulmonary nodular lymphoid hyperplasia. Herein, we describe the first case of pulmonary nodular lymphoid hyperplasia presenting with a reversed halo sign.
Pulmonary Nodular Lymphoid Hyperplasia: Reviewing a Lung Cancer Mimicker.
Pulmonary nodular lymphoid hyperplasia is a rare benign lymphoproliferative disorder. Patients are commonly asymptomatic. Its radiological presentation is characterized by peripheral single nodular lesion, multiple nodules or mass-like consolidation. Therefore, it can mimic other more frequent pulmonary diseases, such as lung cancer and pulmonary lymphoma. The diagnosis is often late, relying on histopathological findings. We report a case of a 66-year-old woman who presented with cough and upper respiratory tract symptoms. Chest CT revealed a mass in the right lower lobe, mediastinal and hilar enlarged lymph nodes and multifocal ground-glass and part solid nodules. PET-CT findings were concerning for primary lung malignancy. She underwent transbronchial needle aspiration biopsy of the enlarged lymph nodes and pulmonary mass. The results were negative for malignancy, but immunophenotyping by flow cytometry was concerning for a lymphoproliferative disorder. For this reason, a percutaneous CT-guided transthoracic core needle biopsy of the mass was performed. Immunophenotypic features of lymphoma were not identified. Overall findings were suggestive of nodular lymphoid hyperplasia. The follow-up chest CT showed near complete resolution of the mass with a residual ground-glass lesion and stability of the enlarged lymph nodes, ground-glass and part solid nodules. Pulmonary nodular lymphoid hyperplasia is an uncommon lung disorder, usually detected incidentally on chest imaging. Its radiological features can mimic lung cancer and other more prevalent pulmonary diseases. Therefore, a multidisciplinary approach, including histopathological confirmation is essential for an accurate diagnosis. Although rare, it should be considered in the differential diagnosis of pulmonary malignancies.
IRTA1 expression in extranodal marginal zone (MALT) lymphoma is variable across anatomic sites.
Immune receptor translocation-associated 1 (IRTA1) expression, which has recently been shown to be highly specific for marginal zone lymphoma, is found in only a subset of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). In this study, we examined whether IRTA1 expression in MALT lymphoma was dependent on anatomic location. IRTA1 immunohistochemistry was performed in 60 cases of extranodal MALT lymphoma, arising at breast, conjunctiva, lung, salivary gland, skin, and stomach (10 cases each). Expression was also scored with respect to detailed morphologic features. IRTA1 staining was identified in 25 (42%) cases, ranging from 9 (90%) of 10 in lung to 2 (20%) of 10 in gastric MALT lymphoma. IRTA1 staining was not characteristic of specific morphologic features, as it was absent in most lymphoepithelial lesions, perifollicular cells, cells with overt monocytoid cytology, and plasmacytic cells. IRTA1 expression was also identified in 5 (50%) of 10 cases of pulmonary nodular lymphoid hyperplasia. IRTA1 expression is heterogeneous in extranodal MALT lymphoma. Staining for IRTA1 is not characteristic of specific morphologic features, but varies with the anatomic site of disease. Knowledge of site-specific differences in MALT lymphomas will facilitate diagnosis in routine clinical practice.
Clinical and Imaging Features of Pulmonary Nodular Lymphoid Hyperplasia.
To investigate the clinical and radiographic features of PNLH and the relationship with pathologic features. A total of 11 patients in whom PNLH was confirmed in our department were retrospectively studied. The clinical and radiographic features were extracted and analyzed, and we also discussed the relationship between radiologic and pathologic features. Of the 11 patients with PNLH, 5 were discovered incidentally, while 4 presented with chest symptoms. Laboratory tests showed no specificity and the lesions were located under the pleura with an adjacent pleural indentation. Most lesions were solid, with some showing signs of spiculation or spiculate protuberance. In some cases, hypodense areas and vocules were visible. The enhanced scan showed marked enhancement, but most had no lymph node enlargement, and there was no pleural effusion. The clinical manifestations of PNLH are nonspecific and the imaging features overlap with those of malignant lung tumors, and the diagnosis depends on pathologic examination.
[Pulmonary nodular lymphoid hyperplasia: a case report].
Pulmonary nodular lymphoid hyperplasia (PNLH) is a rare pulmonary lymphoproliferative disease characterized by the absence of specific clinical and imaging features. Consequently, diagnosis relies heavily on postoperative pathological examination, which often leads to preoperative misdiagnosis. Here, we report an atypical case of PNLH in a 57-year-old female patient who was admitted to hospital after a pulmonary nodule was discovered during a routine physical examination two days earlier. Chest CT scan revealed a mixed ground-glass nodule in the apicoposterior segment of the left upper lobe, with lobulation and spiculation. These imaging findings initially suggested a diagnosis of pulmonary malignancy. However, pathological examination and IgH gene rearrangement analysis of the resected tissue ultimately confirmed the diagnosis of PNLH. 肺结节性淋巴组织增生(PNLH)是一种肺内少见的淋巴组织增生性疾病,其临床和影像学上没有特定征象,诊断很大程度上取决于术后病理检查,因此术前易误诊。本文报道1例非典型的PNLH病例:患者女,57岁,因体检发现肺结节2 d入院。胸部CT显示左肺上叶尖后段混合磨玻璃结节,可见分叶、毛刺,基于此影像特征初步诊断考虑为肺部恶性肿瘤。术后切除组织行病理学及IgH基因克隆性重排检测分析,最终诊断为PNLH。.
Publicações recentes
Pulmonary Nodular Lymphoid Hyperplasia Presenting With a Reversed Halo Sign.
Pulmonary Nodular Lymphoid Hyperplasia: Reviewing a Lung Cancer Mimicker.
IRTA1 expression in extranodal marginal zone (MALT) lymphoma is variable across anatomic sites.
[Pulmonary nodular lymphoid hyperplasia: a case report].
Clinical and Imaging Features of Pulmonary Nodular Lymphoid Hyperplasia.
📚 EuropePMC26 artigos no totalmostrando 23
Pulmonary Nodular Lymphoid Hyperplasia Presenting With a Reversed Halo Sign.
Respirology case reportsPulmonary Nodular Lymphoid Hyperplasia: Reviewing a Lung Cancer Mimicker.
Lung Cancer (Auckland, N.Z.)IRTA1 expression in extranodal marginal zone (MALT) lymphoma is variable across anatomic sites.
American journal of clinical pathology[Pulmonary nodular lymphoid hyperplasia: a case report].
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseasesClinical and Imaging Features of Pulmonary Nodular Lymphoid Hyperplasia.
Journal of thoracic imagingSuspected Aspergillus nodule histologically consistent with pulmonary nodular lymphoid hyperplasia:a case report.
The journal of medical investigation : JMIPulmonary nodular lymphoid hyperplasia presenting as cavitating lung mass.
BMJ case reportsA Case of Pulmonary Nodular Lymphoid Hyperplasia Responding to Corticosteroid Treatment.
CureusPulmonary Nodular Lymphoid Hyperplasia Evaluated with Bronchoalveolar Lavage Fluid Findings: A Case Report and Review of the Literature on Japanese Patients.
Internal medicine (Tokyo, Japan)Pulmonary nodular lymphoid hyperplasia presenting as multifocal subsolid nodules: A case report and literature review.
Respiratory medicine case reportsThe diagnostic challenge of adenocarcinoma in pulmonary nodular lymphoid hyperplasia.
SAGE open medical case reportsPulmonary nodular lymphoid hyperplasia in a 53-year-old man with malignant sign: a case report.
Journal of cardiothoracic surgeryPulmonary nodular lymphoid hyperplasia and Sjögren's syndrome: a case report and literature review.
Rheumatology internationalPulmonary nodular lymphoid hyperplasia in pediatric patients with Hodgkin lymphoma.
Pediatric hematology and oncologyAntibiotic-induced reduction of abnormal lung shadow in pulmonary nodular lymphoid hyperplasia.
Respirology case reportsPulmonary nodular lymphoid hyperplasia: a rare benign disease with malignant mask.
Annals of translational medicinePulmonary Nodular Lymphoid Hyperplasia.
Archives of pathology & laboratory medicineRare case of nodular lymphoid hyperplasia of left lung in the patient with previous pulmonary tuberculosis.
Experimental oncologyBenign pulmonary nodular lymphoid hyperplasia (NLH) in an HIV infected patient; A diagnostic dilemma.
Respiratory medicine case reportsPulmonary Nodular Lymphoid Hyperplasia with Mass-Formation: Clinicopathologic Characteristics of Nine Cases and Review of the Literature.
Journal of pathology and translational medicinePulmonary Nodular Lymphoid Hyperplasia in a 33-Year-Old Woman.
The Korean journal of thoracic and cardiovascular surgeryNo definite clinical features of immunoglobulin G4-related disease in patients with pulmonary nodular lymphoid hyperplasia.
Human pathologyPulmonary Nodular Lymphoid Hyperplasia presenting cavitating pulmonary nodules.
Pathology, research and practiceAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Ainda não temos associações cadastradas para Hiperplasia linfoide nodular pulmonar.
É de uma associação que acompanha esta doença? Fale com a gente →
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Não, trata-se de um aumento benigno e reacional de células de defesa (linfócitos) nos pulmões. Apesar de formar nódulos que podem simular tumores malignos em exames de imagem, a doença não é um câncer.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
5 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (s.d.). Hiperplasia linfoide nodular pulmonar. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/hiperplasia-linfoide-nodular-pulmonar
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Hiperplasia linfoide nodular pulmonar
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Genética mendeliana
- fonte: OMIM
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Dado público estruturado
- fonte: Wikidata