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Diffuse Pulmonary Lymphangiomatosis

MedGen UID:
310695
•Concept ID:
C1707744
•
Neoplastic Process
Synonym: Diffuse pulmonary lymphangiomatosis
SNOMED CT: Diffuse pulmonary lymphangiomatosis (1179719005)

Definition

Lymphangiomatosis diffusely involving the lung parenchyma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiffuse Pulmonary Lymphangiomatosis

Recent clinical studies

Etiology

Congenital interstitial lung diseases: What the anesthesiologist needs to know.
Bertolizio G, Engelhardt T, Veyckemans F
Paediatr Anaesth 2022 Feb;32(2):138-147. Epub 2021 Nov 11 doi: 10.1111/pan.14325. PMID: 34738691
Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography.
Sun X, Shen W, Xia S, Wen T, Wang R
AJR Am J Roentgenol 2017 Feb;208(2):300-305. Epub 2016 Nov 15 doi: 10.2214/AJR.16.16589. PMID: 27845836
Tumorlike conditions of the pleura.
Walker CM, Takasugi JE, Chung JH, Reddy GP, Done SL, Pipavath SN, Schmidt RA, Godwin JD 2nd
Radiographics 2012 Jul-Aug;32(4):971-85. doi: 10.1148/rg.324115184. PMID: 22786988
Diffuse pulmonary lymphangiomatosis: CT findings.
Swensen SJ, Hartman TE, Mayo JR, Colby TV, Tazelaar HD, Müller NL
J Comput Assist Tomogr 1995 May-Jun;19(3):348-52. doi: 10.1097/00004728-199505000-00002. PMID: 7790540

Diagnosis

Diffuse pulmonary lymphangiomatosis involving lungs and mediastinal soft tissue.
Zhang S, Zhong D, Zhao L, Liu M, Gao Q, Wan J, Zhai Z
Am J Med Sci 2022 Jul;364(1):118-123. Epub 2022 Apr 9 doi: 10.1016/j.amjms.2022.03.015. PMID: 35405139
Congenital interstitial lung diseases: What the anesthesiologist needs to know.
Bertolizio G, Engelhardt T, Veyckemans F
Paediatr Anaesth 2022 Feb;32(2):138-147. Epub 2021 Nov 11 doi: 10.1111/pan.14325. PMID: 34738691
Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report.
Dimiene I, Bieksiene K, Zaveckiene J, Andrulis M, Optazaite DE, Vaguliene N, Zemaitis M, Miliauskas S
Medicina (Kaunas) 2021 Nov 29;57(12) doi: 10.3390/medicina57121308. PMID: 34946253Free PMC Article
Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography.
Sun X, Shen W, Xia S, Wen T, Wang R
AJR Am J Roentgenol 2017 Feb;208(2):300-305. Epub 2016 Nov 15 doi: 10.2214/AJR.16.16589. PMID: 27845836
Diffuse pulmonary lymphangiomatosis: a case report with literature review.
DU MH, Ye RJ, Sun KK, Li JF, Shen DH, Wang J, Gao ZC
Chin Med J (Engl) 2011 Mar;124(5):797-800. PMID: 21518582

Therapy

Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report.
Dimiene I, Bieksiene K, Zaveckiene J, Andrulis M, Optazaite DE, Vaguliene N, Zemaitis M, Miliauskas S
Medicina (Kaunas) 2021 Nov 29;57(12) doi: 10.3390/medicina57121308. PMID: 34946253Free PMC Article
Diffuse thoracic lymphangiomatosis with disseminated intravascular coagulation in a child.
Tamay Z, Saribeyoglu E, Ones U, Anak S, Güler N, Bilgic B, Yilmazbayhan D, Gun F
J Pediatr Hematol Oncol 2005 Dec;27(12):685-7. doi: 10.1097/01.mph.0000193476.14493.06. PMID: 16344679

Prognosis

Congenital interstitial lung diseases: What the anesthesiologist needs to know.
Bertolizio G, Engelhardt T, Veyckemans F
Paediatr Anaesth 2022 Feb;32(2):138-147. Epub 2021 Nov 11 doi: 10.1111/pan.14325. PMID: 34738691
Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report.
Dimiene I, Bieksiene K, Zaveckiene J, Andrulis M, Optazaite DE, Vaguliene N, Zemaitis M, Miliauskas S
Medicina (Kaunas) 2021 Nov 29;57(12) doi: 10.3390/medicina57121308. PMID: 34946253Free PMC Article
Diffuse Pulmonary Lymphangiomatosis: MDCT Findings After Direct Lymphangiography.
Sun X, Shen W, Xia S, Wen T, Wang R
AJR Am J Roentgenol 2017 Feb;208(2):300-305. Epub 2016 Nov 15 doi: 10.2214/AJR.16.16589. PMID: 27845836
Diffuse pulmonary lymphangiomatosis: a case report with literature review.
DU MH, Ye RJ, Sun KK, Li JF, Shen DH, Wang J, Gao ZC
Chin Med J (Engl) 2011 Mar;124(5):797-800. PMID: 21518582
Diffuse pulmonary lymphangiomatosis: imaging findings.
Yekeler E, Dursun M, Yildirim A, Tunaci M
Diagn Interv Radiol 2005 Mar;11(1):31-4. PMID: 15795841

Clinical prediction guides

A gene missense mutation in diffuse pulmonary lymphangiomatosis with thrombocytopenia: A case report.
Zheng G, Tang H, Su R, Liang Y, He Z, Zhang J, Deng J, Bai J, Zhong X
Medicine (Baltimore) 2020 Sep 25;99(39):e21941. doi: 10.1097/MD.0000000000021941. PMID: 32991402Free PMC Article
Lymphangiomatosis involving the pulmonary and extrapulmonary lymph nodes and surrounding soft tissue: A rare case report.
Fang X, Huang Z, Zeng Y, Zhu X, Wang S, Yu X, Li X, Wu C, Yi X
Medicine (Baltimore) 2017 Dec;96(49):e9032. doi: 10.1097/MD.0000000000009032. PMID: 29245295Free PMC Article
Diagnostic value of bronchoscopy, CT and transbronchial biopsies in diffuse pulmonary lymphangiomatosis: case report and review of the literature.
El Hajj L, Mazières J, Rouquette I, Mittaine M, Bolduc JP, Didier A, Dahan M, Joffre F, Chabbert VC
Clin Radiol 2005 Aug;60(8):921-5. doi: 10.1016/j.crad.2005.03.006. PMID: 16039928

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