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Lymphatic malformation 2(LMPHM2)

MedGen UID:
1648459
Concept ID:
C4747568
Disease or Syndrome
Synonyms: LMPH1B; LMPHM2; Lymphedema, hereditary, 1B; Lymphedema, hereditary, IB
 
Monarch Initiative: MONDO:0012765
OMIM®: 611944

Definition

Hereditary primary lymphedema is caused by anatomic or functional defects in the lymphatic system, resulting in chronic swelling of body parts. There may be accompanying nail and skin changes, such as nail dysplasia or papillomatosis. Onset is usually at birth or in early childhood but can occur later, and the severity is variable (summary by Gordon et al., 2013 and Balboa-Beltran et al., 2014). For a discussion of genetic heterogeneity of lymphatic malformation, see 153100. [from OMIM]

Clinical features

From HPO
Lymphedema
MedGen UID:
6155
Concept ID:
C0024236
Disease or Syndrome
Localized fluid retention and tissue swelling caused by a compromised lymphatic system.

Professional guidelines

PubMed

Biggs SE, Gilchrist B, May KR
Curr Allergy Asthma Rep 2023 Apr;23(4):213-222. Epub 2023 Mar 10 doi: 10.1007/s11882-023-01071-4. PMID: 36897497Free PMC Article
Craven MD, Washabau RJ
J Vet Intern Med 2019 Mar;33(2):383-402. Epub 2019 Feb 14 doi: 10.1111/jvim.15406. PMID: 30762910Free PMC Article
Carqueja IM, Sousa J, Mansilha A
Int Angiol 2018 Apr;37(2):127-142. Epub 2018 Feb 8 doi: 10.23736/S0392-9590.18.03961-5. PMID: 29424187

Recent clinical studies

Etiology

Shin D, Kim CN, Ross J, Hennick KM, Wu SR, Paranjape N, Leonard R, Wang JC, Keefe MG, Pavlovic BJ, Donohue KC, Moreau C, Wigdor EM, Larson HH, Allen DE, Cadwell CR, Bhaduri A, Popova G, Bearden CE, Pollen AA, Jacquemont S, Sanders SJ, Haussler D, Wiita AP, Frost NA, Sohal VS, Nowakowski TJ
Cell Stem Cell 2024 Mar 7;31(3):421-432.e8. Epub 2024 Feb 20 doi: 10.1016/j.stem.2024.01.010. PMID: 38382530Free PMC Article
Chaudhry SG, Fung AT
Retin Cases Brief Rep 2023 Mar 1;17(2):137-139. doi: 10.1097/ICB.0000000000001120. PMID: 36821469
Blagowidow N, Nowakowska B, Schindewolf E, Grati FR, Putotto C, Breckpot J, Swillen A, Crowley TB, Loo JCY, Lairson LA, Óskarsdóttir S, Boot E, Garcia-Minaur S, Cristina Digilio M, Marino B, Coleman B, Moldenhauer JS, Bassett AS, McDonald-McGinn DM
Genes (Basel) 2023 Jan 6;14(1) doi: 10.3390/genes14010160. PMID: 36672900Free PMC Article
Mustillo PJ, Sullivan KE, Chinn IK, Notarangelo LD, Haddad E, Davies EG, de la Morena MT, Hartog N, Yu JE, Hernandez-Trujillo VP, Ip W, Franco J, Gambineri E, Hickey SE, Varga E, Markert ML
J Clin Immunol 2023 Feb;43(2):247-270. Epub 2023 Jan 17 doi: 10.1007/s10875-022-01418-y. PMID: 36648576Free PMC Article
Palmer LD, Heung T, Corral M, Boot E, Brooks SG, Bassett AS
J Appl Res Intellect Disabil 2022 Jul;35(4):966-975. Epub 2021 Jul 21 doi: 10.1111/jar.12927. PMID: 34291536

Diagnosis

Biggs SE, Gilchrist B, May KR
Curr Allergy Asthma Rep 2023 Apr;23(4):213-222. Epub 2023 Mar 10 doi: 10.1007/s11882-023-01071-4. PMID: 36897497Free PMC Article
Altshuler E, Saidi A, Budd J
BMJ Case Rep 2022 Feb 2;15(2) doi: 10.1136/bcr-2021-245164. PMID: 35110278Free PMC Article
Cirillo A, Lioncino M, Maratea A, Passariello A, Fusco A, Fratta F, Monda E, Caiazza M, Signore G, Esposito A, Baban A, Versacci P, Putotto C, Marino B, Pignata C, Cirillo E, Giardino G, Sarubbi B, Limongelli G, Russo MG
Heart Fail Clin 2022 Jan;18(1):155-164. Epub 2021 Oct 25 doi: 10.1016/j.hfc.2021.07.009. PMID: 34776076
McDonald-McGinn DM, Sullivan KE, Marino B, Philip N, Swillen A, Vorstman JA, Zackai EH, Emanuel BS, Vermeesch JR, Morrow BE, Scambler PJ, Bassett AS
Nat Rev Dis Primers 2015 Nov 19;1:15071. doi: 10.1038/nrdp.2015.71. PMID: 27189754Free PMC Article
McDonald-McGinn DM, Sullivan KE
Medicine (Baltimore) 2011 Jan;90(1):1-18. doi: 10.1097/MD.0b013e3182060469. PMID: 21200182

Therapy

Vong KI, Lee S, Au KS, Crowley TB, Capra V, Martino J, Haller M, Araújo C, Machado HR, George R, Gerding B, James KN, Stanley V, Jiang N, Alu K, Meave N, Nidhiry AS, Jiwani F, Tang I, Nisal A, Jhamb I, Patel A, Patel A, McEvoy-Venneri J, Barrows C, Shen C, Ha YJ, Howarth R, Strain M, Ashley-Koch AE, Azam M, Mumtaz S, Bot GM, Finnell RH, Kibar Z, Marwan AI, Melikishvili G, Meltzer HS, Mutchinick OM, Stevenson DA, Mroczkowski HJ, Ostrander B, Schindewolf E, Moldenhauer J, Zackai EH, Emanuel BS, Garcia-Minaur S, Nowakowska BA, Stevenson RE, Zaki MS, Northrup H, McNamara HK, Aldinger KA, Phelps IG, Deng M, Glass IA; Spina Bifida Sequencing Consortium‡, Morrow B, McDonald-McGinn DM, Sanna-Cherchi S, Lamb DJ, Gleeson JG
Science 2024 May 3;384(6695):584-590. Epub 2024 May 2 doi: 10.1126/science.adl1624. PMID: 38696583
Queisser A, Seront E, Boon LM, Vikkula M
Circ Res 2021 Jun 25;129(1):155-173. Epub 2021 Jun 24 doi: 10.1161/CIRCRESAHA.121.318145. PMID: 34166070
Carqueja IM, Sousa J, Mansilha A
Int Angiol 2018 Apr;37(2):127-142. Epub 2018 Feb 8 doi: 10.23736/S0392-9590.18.03961-5. PMID: 29424187
Markert ML, Devlin BH, McCarthy EA
Clin Immunol 2010 May;135(2):236-46. Epub 2010 Mar 16 doi: 10.1016/j.clim.2010.02.007. PMID: 20236866Free PMC Article
Merli GJ
Clin Podiatry 1984 Aug;1(2):363-72. PMID: 6536398

Prognosis

McInerney NJ, O'Keeffe N, Nae A, Morariu J, Timon C
Ir J Med Sci 2023 Oct;192(5):2373-2377. Epub 2023 Jan 16 doi: 10.1007/s11845-022-03271-9. PMID: 36642745Free PMC Article
Xia H, Huang Z, Liu S, Zhao X, He R, Wang Z, Shi W, Chen W, Li Z, Yu L, Huang P, Kang P, Su Z, Xu Y, Yam JWP, Cui Y
Biomed Pharmacother 2021 Sep;141:111889. Epub 2021 Jul 14 doi: 10.1016/j.biopha.2021.111889. PMID: 34323697
Clemens RK, Pfammatter T, Meier TO, Alomari AI, Amann-Vesti BR
Vasa 2015 Mar;44(2):92-105. doi: 10.1024/0301-1526/a000414. PMID: 25698387
Ioannidis I, Kahn AG
Arch Pathol Lab Med 2015 Feb;139(2):278-82. doi: 10.5858/arpa.2013-0656-RS. PMID: 25611113
Colice GL
Clin Geriatr Med 1986 May;2(2):411-32. PMID: 3516371

Clinical prediction guides

Santinha AJ, Klingler E, Kuhn M, Farouni R, Lagler S, Kalamakis G, Lischetti U, Jabaudon D, Platt RJ
Nature 2023 Oct;622(7982):367-375. Epub 2023 Sep 20 doi: 10.1038/s41586-023-06570-y. PMID: 37730998Free PMC Article
Maruani A, Tavernier E, Boccara O, Mazereeuw-Hautier J, Leducq S, Bessis D, Guibaud L, Vabres P, Carmignac V, Mallet S, Barbarot S, Chiaverini C, Droitcourt C, Bursztejn AC, Lengellé C, Woillard JB, Herbreteau D, Le Touze A, Joly A, Léauté-Labrèze C, Powell J, Bourgoin H, Gissot V, Giraudeau B, Morel B
JAMA Dermatol 2021 Nov 1;157(11):1289-1298. doi: 10.1001/jamadermatol.2021.3459. PMID: 34524406Free PMC Article
Lalani SR
Am J Med Genet C Semin Med Genet 2020 Mar;184(1):107-115. Epub 2020 Jan 7 doi: 10.1002/ajmg.c.31762. PMID: 31909881
Burke S, Maramaldi P
Fetal Pediatr Pathol 2017 Feb;36(1):33-41. Epub 2016 Oct 12 doi: 10.1080/15513815.2016.1231248. PMID: 27732116
Unolt M, DiCairano L, Schlechtweg K, Barry J, Howell L, Kasperski S, Nance M, Adzick NS, Zackai EH, McDonald-McGinn DM
Am J Med Genet A 2017 Jan;173(1):135-142. Epub 2016 Sep 28 doi: 10.1002/ajmg.a.37980. PMID: 27682988

Recent systematic reviews

Kamhieh Y, Mitra R, Burnett T, Jones H, Roblin G, Hall A
Laryngoscope 2024 May;134(5):2038-2047. Epub 2023 Oct 9 doi: 10.1002/lary.31091. PMID: 37812168
Saibene AM, Rosso C, Felisati G, Pignataro L, Schindler A, Ghilardi G, Colletti G, Gaffuri M, Mozzanica F
Eur Arch Otorhinolaryngol 2023 Aug;280(8):3529-3540. Epub 2023 Apr 28 doi: 10.1007/s00405-023-07991-1. PMID: 37115326Free PMC Article
Boot E, Óskarsdóttir S, Loo JCY, Crowley TB, Orchanian-Cheff A, Andrade DM, Arganbright JM, Castelein RM, Cserti-Gazdewich C, de Reuver S, Fiksinski AM, Klingberg G, Lang AE, Mascarenhas MR, Moss EM, Nowakowska BA, Oechslin E, Palmer L, Repetto GM, Reyes NGD, Schneider M, Silversides C, Sullivan KE, Swillen A, van Amelsvoort TAMJ, Van Batavia JP, Vingerhoets C, McDonald-McGinn DM, Bassett AS
Genet Med 2023 Mar;25(3):100344. Epub 2023 Feb 2 doi: 10.1016/j.gim.2022.11.012. PMID: 36729052
Wiegand S, Dietz A, Wichmann G
Eur Arch Otorhinolaryngol 2022 Aug;279(8):3801-3810. Epub 2022 May 8 doi: 10.1007/s00405-022-07378-8. PMID: 35526176Free PMC Article
De Maria L, De Sanctis P, Balakrishnan K, Tollefson M, Brinjikji W
J Vasc Surg Venous Lymphat Disord 2020 Jan;8(1):154-164. Epub 2019 Nov 14 doi: 10.1016/j.jvsv.2019.09.007. PMID: 31734224

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