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Lymphomatoid granulomatosis

MedGen UID:
6161
Concept ID:
C0024307
Neoplastic Process
Synonyms: Granulomatoses, Lymphomatoid; Granulomatosis, Lymphomatoid; Lymphomatoid Granulomatoses; Lymphomatoid Granulomatosis
SNOMED CT: LG - Lymphomatoid granulomatosis (239940004); Lymphomatoid granulomatosis (239940004); Lymphomatoid granulomatosis (789177000); Angiocentric immunoproliferative lesion (41556003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019466
Orphanet: ORPHA86869

Definition

Lymphomatoid granulomatosis (LYG) is a very rare Epstein-Barr virus (EBV)-driven lymphoproliferative disease most commonly occurring in adults (in the fourth to sixth decade of life) and commonly affecting the lungs (with presentations varying from small bilateral pulmonary nodules to large necrotic and sometimes cavitating lesions), skin, central nervous system, and kidneys, but only very rarely affecting the lymph nodes and spleen. The symptoms associated with LYG depend on the site of disease involvement but mainly include cough, dyspnea or chest pain (in those with pulmonary involvement) and constitutional symptoms such as weight loss and fever. [from ORDO]

Professional guidelines

PubMed

Melani C, Jaffe ES, Wilson WH
Blood 2020 Apr 16;135(16):1344-1352. doi: 10.1182/blood.2019000933. PMID: 32107539Free PMC Article
Sirajuddin A, Raparia K, Lewis VA, Franks TJ, Dhand S, Galvin JR, White CS
Radiographics 2016 Jan-Feb;36(1):53-70. doi: 10.1148/rg.2016140339. PMID: 26761531
Cadranel J, Wislez M, Antoine M
Eur Respir J 2002 Sep;20(3):750-62. doi: 10.1183/09031936.02.00404102. PMID: 12358356

Recent clinical studies

Etiology

Quintanilla-Martinez L, Swerdlow SH, Tousseyn T, Barrionuevo C, Nakamura S, Jaffe ES
Virchows Arch 2023 Jan;482(1):227-244. Epub 2022 Oct 11 doi: 10.1007/s00428-022-03414-4. PMID: 36216980Free PMC Article
Zhang H, Zhao S, Cao Z
Front Immunol 2022;13:1001055. Epub 2022 Oct 28 doi: 10.3389/fimmu.2022.1001055. PMID: 36389673Free PMC Article
Sirajuddin A, Raparia K, Lewis VA, Franks TJ, Dhand S, Galvin JR, White CS
Radiographics 2016 Jan-Feb;36(1):53-70. doi: 10.1148/rg.2016140339. PMID: 26761531
Tomasini D, Berti E
G Ital Dermatol Venereol 2015 Feb;150(1):95-113. Epub 2014 Dec 22 PMID: 25531150
DePaso WJ, Winterbauer RH
Dis Mon 1991 Feb;37(2):61-133. PMID: 1991445

Diagnosis

Quintanilla-Martinez L, Swerdlow SH, Tousseyn T, Barrionuevo C, Nakamura S, Jaffe ES
Virchows Arch 2023 Jan;482(1):227-244. Epub 2022 Oct 11 doi: 10.1007/s00428-022-03414-4. PMID: 36216980Free PMC Article
Zhang H, Zhao S, Cao Z
Front Immunol 2022;13:1001055. Epub 2022 Oct 28 doi: 10.3389/fimmu.2022.1001055. PMID: 36389673Free PMC Article
Melani C, Jaffe ES, Wilson WH
Blood 2020 Apr 16;135(16):1344-1352. doi: 10.1182/blood.2019000933. PMID: 32107539Free PMC Article
Sukswai N, Lyapichev K, Khoury JD, Medeiros LJ
Pathology 2020 Jan;52(1):53-67. Epub 2019 Nov 15 doi: 10.1016/j.pathol.2019.08.013. PMID: 31735345
Morales AT, Cignarella AG, Jabeen IS, Barkin JS, Mirsaeidi M
Eur J Intern Med 2019 Aug;66:18-24. Epub 2019 Jun 19 doi: 10.1016/j.ejim.2019.06.010. PMID: 31227290

Therapy

Rezk SA, Weiss LM
Surg Pathol Clin 2019 Sep;12(3):745-770. doi: 10.1016/j.path.2019.03.002. PMID: 31352986
Morales AT, Cignarella AG, Jabeen IS, Barkin JS, Mirsaeidi M
Eur J Intern Med 2019 Aug;66:18-24. Epub 2019 Jun 19 doi: 10.1016/j.ejim.2019.06.010. PMID: 31227290
Frey JJ
WMJ 2012 Apr;111(2):53. PMID: 22616470
Yevich I
Int J Dermatol 1988 Oct;27(8):540-6. doi: 10.1111/j.1365-4362.1988.tb02400.x. PMID: 3061945
Leavitt RY, Fauci AS
Am Rev Respir Dis 1986 Jul;134(1):149-66. doi: 10.1164/arrd.1986.134.1.149. PMID: 2873770

Prognosis

Quintanilla-Martinez L, Swerdlow SH, Tousseyn T, Barrionuevo C, Nakamura S, Jaffe ES
Virchows Arch 2023 Jan;482(1):227-244. Epub 2022 Oct 11 doi: 10.1007/s00428-022-03414-4. PMID: 36216980Free PMC Article
Zhang H, Zhao S, Cao Z
Front Immunol 2022;13:1001055. Epub 2022 Oct 28 doi: 10.3389/fimmu.2022.1001055. PMID: 36389673Free PMC Article
Morales AT, Cignarella AG, Jabeen IS, Barkin JS, Mirsaeidi M
Eur J Intern Med 2019 Aug;66:18-24. Epub 2019 Jun 19 doi: 10.1016/j.ejim.2019.06.010. PMID: 31227290
Borie R, Wislez M, Antoine M, Cadranel J
Respiration 2017;94(2):157-175. Epub 2017 Jun 14 doi: 10.1159/000477740. PMID: 28609772
Leavitt RY, Fauci AS
Am Rev Respir Dis 1986 Jul;134(1):149-66. doi: 10.1164/arrd.1986.134.1.149. PMID: 2873770

Clinical prediction guides

Marano M, Brunelli C, De Marco C, Martini M, Quattrocchi CC, Lauriola L, Di Lazzaro V
Can J Neurol Sci 2021 Jan;48(1):114-115. Epub 2020 Jul 6 doi: 10.1017/cjn.2020.133. PMID: 32624028
Yang M, Rosenthal AC, Ashman JB, Craig FE
Curr Probl Diagn Radiol 2021 May-Jun;50(3):443-449. Epub 2019 Feb 10 doi: 10.1067/j.cpradiol.2019.02.002. PMID: 30826140
Castillo JJ, Beltran BE, Miranda RN, Young KH, Chavez JC, Sotomayor EM
Am J Hematol 2016 May;91(5):529-37. doi: 10.1002/ajh.24370. PMID: 27093913
Chung JH, Wu CC, Gilman MD, Palmer EL, Hasserjian RP, Shepard JA
Korean J Radiol 2011 Nov-Dec;12(6):671-8. Epub 2011 Sep 27 doi: 10.3348/kjr.2011.12.6.671. PMID: 22043148Free PMC Article
Mrusek S, Marx A, Kummerle-Deschner J, Tzaribachev N, Enders A, Riede UN, Warnatz K, Dannecker GE, Ehl S
Clin Exp Immunol 2004 Sep;137(3):578-83. doi: 10.1111/j.1365-2249.2004.02558.x. PMID: 15320909Free PMC Article

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