MCID: LYM095
MIFTS: 24

Lymphangiomatosis

Categories: Immune diseases, Rare diseases
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Aliases & Classifications for Lymphangiomatosis

MalaCards integrated aliases for Lymphangiomatosis:

Name: Lymphangiomatosis 19 71 75

Classifications:



External Ids:

UMLS 71 C0334102

Summaries for Lymphangiomatosis

MalaCards based summary: Lymphangiomatosis is related to diffuse lymphatic malformation and lymphangiectasia, pulmonary, congenital. An important gene associated with Lymphangiomatosis is TSC2 (TSC Complex Subunit 2). The drugs Sirolimus and Miconazole have been mentioned in the context of this disorder. Affiliated tissues include endothelial, spleen and bone.

Wikipedia: 75 Lymphangiomatosis is a condition where a lymphangioma is not present in a single localised mass, but in... more...

Related Diseases for Lymphangiomatosis

Diseases related to Lymphangiomatosis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 145)
# Related Disease Score Top Affiliating Genes
1 diffuse lymphatic malformation 11.4
2 lymphangiectasia, pulmonary, congenital 11.3
3 kaposiform lymphangiomatosis 11.2
4 lymphangioma 10.8
5 rare lymphatic malformation 10.6
6 gorham's disease 10.5
7 generalized lymphatic anomaly 10.3
8 lymphatic system disease 10.3
9 splenomegaly 10.3
10 factor viii deficiency 10.3
11 cystic angiomatosis of bone, diffuse 10.3
12 lymphangioleiomyomatosis 10.3
13 cystic lymphangioma 10.3
14 protein-losing enteropathy 10.2
15 hemangioendothelioma 10.2
16 kaposiform hemangioendothelioma 10.2
17 ascites, chylous 10.2
18 disseminated intravascular coagulation 10.2
19 hereditary lymphedema i 10.2
20 respiratory failure 10.2
21 pericardial effusion 10.2
22 lymphangiectasis 10.2
23 thrombocytopenia 10.1
24 coloboma of optic nerve 10.1
25 bone disease 10.1
26 interstitial lung disease 10.1
27 congenital hemidysplasia with ichthyosiform erythroderma and limb defects 10.0
28 colonic lymphangioma 10.0
29 hemangioma 10.0
30 liver disease 10.0
31 children's interstitial lung disease 10.0
32 hemangioma-thrombocytopenia syndrome 9.9
33 kaposi sarcoma 9.9
34 factor vii deficiency 9.9
35 hemihyperplasia, isolated 9.9
36 short stature, onychodysplasia, facial dysmorphism, and hypotrichosis 9.9
37 polycystic kidney disease 9.9
38 otorrhea 9.9
39 heart disease 9.9
40 angiomyolipoma 9.9
41 mediastinal cancer 9.9
42 peritonitis 9.9
43 kidney angiomyolipoma 9.9
44 meningitis 9.9
45 cerebrospinal fluid leak 9.9
46 skeletal-extraskeletal angiomatosis 9.9
47 rare vascular tumor 9.9
48 blue rubber bleb nevus 9.8
49 keratitis, hereditary 9.8
50 klippel-trenaunay-weber syndrome 9.8

Graphical network of the top 20 diseases related to Lymphangiomatosis:



Diseases related to Lymphangiomatosis

Symptoms & Phenotypes for Lymphangiomatosis

Drugs & Therapeutics for Lymphangiomatosis

Drugs for Lymphangiomatosis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sirolimus Approved, Investigational Phase 2 53123-88-9 5284616 6436030
2
Miconazole Approved, Investigational, Vet_approved Phase 2 22916-47-8 4189
3
Clotrimazole Approved, Vet_approved Phase 2 23593-75-1 2812
4 Anti-Bacterial Agents Phase 2
5 Anti-Infective Agents Phase 2
6 Antifungal Agents Phase 2
7 Antibiotics, Antitubercular Phase 2
8 Immunosuppressive Agents Phase 2
9 Immunologic Factors Phase 2
10 Anesthetics
11 Endothelial Growth Factors
12 Mitogens

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase 2 Study - Clinical Trial Assessing Efficacy and Safety of the mTOR Inhibitor Sirolimus in the Treatment of Complicated Vascular Anomalies Unknown status NCT00975819 Phase 2 sirolimus
2 Dynamic Contrast Enhanced MR Lymphangiogram Imaging of Lymphatic Anomalies (LA) Completed NCT02744027
3 Lymphatic Anomalies Registry for the Assessment of Outcome Data Recruiting NCT02399527
4 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
5 Characterization of the Pathogenesis of Primary and Secondary Lymphatic Disorders Recruiting NCT02156115

Search NIH Clinical Center for Lymphangiomatosis

Genetic Tests for Lymphangiomatosis

Anatomical Context for Lymphangiomatosis

Organs/tissues related to Lymphangiomatosis:

MalaCards : Endothelial, Spleen, Bone, Lung, Liver, Small Intestine, Colon

Publications for Lymphangiomatosis

Articles related to Lymphangiomatosis:

(show top 50) (show all 569)
# Title Authors PMID Year
1
[Kaposiform lymphangiomatosis diagnosed by circulating tumor DNA in a child]. 62
36319163 2022
2
Erratum to "Generalized lymphangiomatosis-A rare manifestation of lymphatic malformation" [Radiology Case Reports 16 (2021) 66-71]. 62
35909921 2022
3
Recurrent haemoptysis: a rare diagnosis of kaposiform lymphangiomatosis and review of literature. 62
36171013 2022
4
How we approach the diagnosis and management of complex lymphatic anomalies. 62
33844431 2022
5
Kaposiform lymphangiomatosis complicated with effusive constrictive pericarditis: a rare systemic malformation involving the heart. 62
35639989 2022
6
Idiopathic bilateral massive perirenal subcapsular effusion: The first case in the literature. 62
33730943 2022
7
Kaposiform Lymphangiomatosis: Pathologic Aspects in 43 Patients. 62
35385405 2022
8
Diffuse pulmonary lymphangiomatosis involving lungs and mediastinal soft tissue. 62
35405139 2022
9
Bilateral renal lymphangiomatosis causes swelling and lower back pain. 62
35717990 2022
10
Pathologic Fracture as Presentation of Generalized Lymphangiomatosis in an Eight-Year-Old: A Case Report and Literature Review. 62
35619846 2022
11
Cellular variant of kaposiform lymphangiomatosis: a report of three cases, expanding the morphologic and molecular genetic spectrum of this rare entity. 62
35202617 2022
12
Kaposiform lymphangiomatosis presenting with a Group A Streptococcus pericardial effusion. 62
35232734 2022
13
Treatment of severe Kaposiform lymphangiomatosis positive for NRAS mutation by MEK inhibition. 62
35246606 2022
14
Congenital interstitial lung diseases: What the anesthesiologist needs to know. 62
34738691 2022
15
Lymphangiomatosis of the Colon. 62
32827725 2022
16
Daratumumab, Lenalidomide, and Dexamethasone (DRD), an Active Regimen in the Treatment of Immunosuppression-Associated Plasmablastic Lymphoma (PBL) in the Setting of Gorham's Lymphangiomatosis: Review of the Literature. 62
35795542 2022
17
Laparoscopy for evaluating mesenteric lymphangiomatosis: A case report. 62
36338709 2022
18
Extensive abdominal lymphangiomatosis involving the small bowel mesentery: A case report. 62
34877341 2021
19
Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report. 62
34946253 2021
20
Cerebrospinal fluid-lymphatic fistula causing spontaneous intracranial hypotension in a child with kaposiform lymphangiomatosis. 62
34286352 2021
21
Macrodystrophia Lipomatosa: A Rare Cause of Bilateral Lower Limb Gigantism. 62
34820241 2021
22
Characterization of kaposiform lymphangiomatosis tissue-derived cells. 62
33913609 2021
23
KRAS mutation identified in a patient with melorheostosis and extended lymphangiomatosis treated with sirolimus and trametinib. 62
34190333 2021
24
Carboplatin, pemetrexed, and pembrolizumab was effective for primary salivary gland-type lung adenocarcinoma diagnosed due to esophageal stricture: A case report. 62
34369074 2021
25
Late recurrence of lung adenocarcinoma harboring EGFR exon 20 insertion (A763_Y764insFQEA) mutation successfully treated with osimertinib. 62
33901930 2021
26
Generalized lymphatic anomalies and review of the current management landscape: a case report and review of the literature. 62
34372919 2021
27
Transthoracic ultrasound: an essential diagnostic tool in a very rare case of thoracic lymphangiomatosis. 62
32905574 2021
28
The efficacy and safety of paclitaxel plus bevacizumab therapy in breast cancer patients with visceral crisis. 62
33901922 2021
29
Gorham-Stout Disease with Multiple Bone Involvement-Challenging Diagnosis of a Rare Disease and Literature Review. 62
34356962 2021
30
Sirolimus in the treatment of kaposiform lymphangiomatosis. 62
34103076 2021
31
[Diffuse pulmonary lymphangiomatosis with pleural and pericardial involvement. Pediatric case report]. 62
34033435 2021
32
Refractory chylothorax and chylous ascites as form of presentation of diffuse lymphangiomatosis. 62
32446681 2021
33
Squamous cell carcinoma of the vulva together with vulvar lymphangiomatosis: vulvectomy and reconstruction (DIEP). 62
33811544 2021
34
Lymphangiomatosis associated with protein losing enteropathy: A case report. 62
34046480 2021
35
Kaposiform Hemangioendothelioma: clinicopathological characteristics of 8 cases of a rare vascular tumor and review of literature. 62
33722245 2021
36
Isolated abdominal kaposiform lymphangiomatosis: a novel presentation of a rare entity. 62
33036770 2021
37
[Lymphangiomatosis: Rare Cause of Pleural Effusion]. 62
32707589 2021
38
Lymphangiomatosis - very rare disease of the lymphatic vessels. 62
34275313 2021
39
Case Report: Multiple Cavernous Pericardial Lymphangioma (Pericardial Lymphangiomatosis) in a Captive Peregrine Falcon (Falco peregrinus brookei). 62
33937381 2021
40
CT and MRI Findings of Focal Splenic Lesions and Ascites in Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease. 62
34513208 2021
41
Systemic generalised lymphangiomatosis: unknown aetiology and a challenge to treat. 62
33462010 2021
42
Renal Lymphangiomatosis Mimicking Polycystic Kidney Disease In An Adult, A Case Report and Literature Review. 62
33492306 2021
43
A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism. 62
33628792 2021
44
Cystic Hygroma with Multiple Benign Bone Lymphangiomas in an Adult Patient: A Rare Entity in the Differential Diagnosis of Multiple Osseous Lesions in Oncology Practice. 62
33327921 2021
45
Generalized lymphangiomatosis-A rare manifestation of lymphatic malformation. 62
33193931 2021
46
Rare cystic lymphangioma in the chest wall of an adult patient: A case report and comprehensive review of the literature. 62
32986288 2020
47
A Case of Lymphangiomatosis With Infected Lymphangiomas Effectively Treated by Thoracoscopic Debridement and Drainage. 62
33160540 2020
48
Chylovenous bypass for mesenteric lymphangiomatosis: A case report. 62
32668017 2020
49
Kaposiform lymphangiomatosis effectively treated with MEK inhibition. 62
32894644 2020
50
A case report of generalized lymphangiomatosis with chylopericardium: the crucial role of magnetic resonance lymphangiography. 62
33204977 2020

Variations for Lymphangiomatosis

Expression for Lymphangiomatosis

Search GEO for disease gene expression data for Lymphangiomatosis.

Pathways for Lymphangiomatosis

GO Terms for Lymphangiomatosis

Sources for Lymphangiomatosis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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