MCID: KPS005
MIFTS: 18

Kaposiform Lymphangiomatosis

Categories: Blood diseases, Cardiovascular diseases, Fetal diseases, Immune diseases, Rare diseases, Respiratory diseases

Aliases & Classifications for Kaposiform Lymphangiomatosis

MalaCards integrated aliases for Kaposiform Lymphangiomatosis:

Name: Kaposiform Lymphangiomatosis 54 60

Classifications:



External Ids:

ICD10 via Orphanet 35 D18.1
Orphanet 60 ORPHA464329

Summaries for Kaposiform Lymphangiomatosis

NIH Rare Diseases : 54 Kaposiform lymphangiomatosis (KLA) is a rare type of tumor and vascular malformation that results from the abnormal development of the lymphatic system. The lymphatic system isĀ  part of the immune system made up of vessels that help to protect the body from infection and foreign substances. KLA involves multiple parts of the body, especially the lungs and chest. Symptoms of KLA usually start during childhood, and include shortness of breath (dyspnea) and cough due to the accumulation of fluid around the lungs (pleural effusion) and heart (pericardial effusion). Other common symptoms include chest and body pain, abnormal bleeding and bruising, and soft masses under the skin. Blood collections may form under the skull (epidural hematoma). The cause for KLA is unknown and it is not thought to be inherited in families. KLA is diagnosed based on the symptoms, laboratory testing, and a biopsy of tumor tissue. There is no specific treatment for KLA. Treatment is based on the symptoms and treatment options may include surgical procedures to drain excess fluid and reduce the size of masses, chemotherapy medications and steroids. KLA tends to be a progressive condition that gets worse with time. The most serious complications include the build-up of fluid around the lungs and heart, and the risk for abnormal bleeding.

MalaCards based summary : Kaposiform Lymphangiomatosis is related to lymphangiomatosis and cystic angiomatosis of bone, diffuse. The drugs Anesthetics and Endothelial Growth Factors have been mentioned in the context of this disorder. Affiliated tissues include lung, skin and heart.

Related Diseases for Kaposiform Lymphangiomatosis

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

# Related Disease Score Top Affiliating Genes
1 lymphangiomatosis 10.8
2 cystic angiomatosis of bone, diffuse 10.3
3 intracranial hypertension, idiopathic 10.2

Symptoms & Phenotypes for Kaposiform Lymphangiomatosis

Drugs & Therapeutics for Kaposiform Lymphangiomatosis

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Anesthetics Not Applicable
2 Endothelial Growth Factors
3 Mitogens

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Imaging of Lymphatic Anomalies Completed NCT02744027 Not Applicable
2 Identification of Biomarkers for Patients With Vascular Anomalies Recruiting NCT03001180
3 Lymphatic Anomalies Registry Recruiting NCT02399527

Search NIH Clinical Center for Kaposiform Lymphangiomatosis

Genetic Tests for Kaposiform Lymphangiomatosis

Anatomical Context for Kaposiform Lymphangiomatosis

MalaCards organs/tissues related to Kaposiform Lymphangiomatosis:

42
Lung, Skin, Heart, Testes, Endothelial, Bone

Publications for Kaposiform Lymphangiomatosis

Articles related to Kaposiform Lymphangiomatosis:

(show all 14)
# Title Authors Year
1
Signaling pathways and inhibitors of cells from patients with kaposiform lymphangiomatosis. ( 31045327 )
2019
2
Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis. ( 29654359 )
2018
3
Chest imaging in generalized lymphatic anomaly and kaposiform lymphangiomatosis. ( 29923669 )
2018
4
Proliferative Cells From Kaposiform Lymphangiomatosis Lesions Resemble Mesenchyme Stem Cell-like Pericytes Defective in Vessel Formation. ( 30256265 )
2018
5
A somatic activating NRAS variant associated with kaposiform lymphangiomatosis. ( 30542204 )
2018
6
Kaposiform Lymphangiomatosis with Human Papillomavirus Infection. ( 28398067 )
2017
7
MR imaging findings of vertebral involvement in Gorham-Stout disease, generalized lymphatic anomaly, and kaposiform lymphangiomatosis. ( 28795277 )
2017
8
Clinical Features and Prognosis of Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease. ( 26806875 )
2016
9
Imaging features of kaposiform lymphangiomatosis. ( 27053281 )
2016
10
Point-of-Care Thoracic Ultrasonography in the Diagnosis and Management of Kaposiform Lymphangiomatosis. ( 27898632 )
2016
11
Kaposiform lymphangiomatosis: unifying features of a heterogeneous disorder. ( 25307772 )
2015
12
Successful treatment of kaposiform lymphangiomatosis with sirolimus. ( 25598153 )
2015
13
Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly. ( 24252784 )
2014
14
Kaposiform lymphangiomatosis, a newly characterized vascular anomaly presenting with hemoptysis in an adult woman. ( 24460439 )
2014

Variations for Kaposiform Lymphangiomatosis

Expression for Kaposiform Lymphangiomatosis

Search GEO for disease gene expression data for Kaposiform Lymphangiomatosis.

Pathways for Kaposiform Lymphangiomatosis

GO Terms for Kaposiform Lymphangiomatosis

Sources for Kaposiform Lymphangiomatosis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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