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Heavy chain disease

MedGen UID:
5469
Concept ID:
C0018852
Neoplastic Process
Synonyms: Heavy Chain Disease; Heavy Chain Diseases
SNOMED CT: Heavy chain disease (68979007); Heavy chain disease (6381009)
 
Monarch Initiative: MONDO:0019464
Orphanet: ORPHA86864

Definition

Heavy-chain diseases (HCDs) are rare monoclonal lymphoplasma-cell proliferative disorders involving B cells and are characterized by the synthesis of truncated heavy chains without associated light chains. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHeavy chain disease
Follow this link to review classifications for Heavy chain disease in Orphanet.

Professional guidelines

PubMed

Fine KD, Stone MJ
Am J Gastroenterol 1999 May;94(5):1139-52. doi: 10.1111/j.1572-0241.1999.01057.x. PMID: 10235185
George ED, Sadovsky R
Am Fam Physician 1999 Apr 1;59(7):1885-94. PMID: 10208707

Recent clinical studies

Etiology

Zhang Y, Li X, Liang D, Xu F, Liang S, Zhu X, Zheng N, Huang X, Liu Z, Zeng C
Am J Kidney Dis 2020 May;75(5):736-743. Epub 2019 Nov 5 doi: 10.1053/j.ajkd.2019.08.013. PMID: 31699519
Bridoux F, Javaugue V, Bender S, Leroy F, Aucouturier P, Debiais-Delpech C, Goujon JM, Quellard N, Bonaud A, Clavel M, Trouillas P, Di Meo F, Gombert JM, Fermand JP, Jaccard A, Cogné M, Touchard G, Sirac C
Kidney Int 2017 Feb;91(2):423-434. Epub 2016 Oct 20 doi: 10.1016/j.kint.2016.09.004. PMID: 27773425
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070
Buxbaum J, Gallo G
Hematol Oncol Clin North Am 1999 Dec;13(6):1235-48. doi: 10.1016/s0889-8588(05)70123-4. PMID: 10626147
Martin IG, Aldoori MI
Br J Surg 1994 Jan;81(1):20-4. doi: 10.1002/bjs.1800810107. PMID: 8313111

Diagnosis

Kušnierová P, Zeman D, Jelínek T, Hájek R
Klin Onkol 2020 Summer;33(4):282-285. doi: 10.14735/amko2020280. PMID: 32894957
Wahner-Roedler DL, Kyle RA
Best Pract Res Clin Haematol 2005;18(4):729-46. doi: 10.1016/j.beha.2005.01.029. PMID: 16026747
O'Connell TX, Horita TJ, Kasravi B
Am Fam Physician 2005 Jan 1;71(1):105-12. PMID: 15663032
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070
Fermand JP, Brouet JC
Hematol Oncol Clin North Am 1999 Dec;13(6):1281-94. doi: 10.1016/s0889-8588(05)70127-1. PMID: 10626151

Therapy

Rao Q, Xu R, Wan Q
J Int Med Res 2022 Mar;50(3):3000605221086428. doi: 10.1177/03000605221086428. PMID: 35301906Free PMC Article
Bianchi G, Sohani AR
Curr Gastroenterol Rep 2018 Jan 25;20(1):3. doi: 10.1007/s11894-018-0608-y. PMID: 29372346
Bridoux F, Javaugue V, Bender S, Leroy F, Aucouturier P, Debiais-Delpech C, Goujon JM, Quellard N, Bonaud A, Clavel M, Trouillas P, Di Meo F, Gombert JM, Fermand JP, Jaccard A, Cogné M, Touchard G, Sirac C
Kidney Int 2017 Feb;91(2):423-434. Epub 2016 Oct 20 doi: 10.1016/j.kint.2016.09.004. PMID: 27773425
Hamers-Casterman C, Atarhouch T, Muyldermans S, Robinson G, Hamers C, Songa EB, Bendahman N, Hamers R
Nature 1993 Jun 3;363(6428):446-8. doi: 10.1038/363446a0. PMID: 8502296
Seligmann M
Ciba Found Symp 1977 Apr 26-28;(46):263-81. doi: 10.1002/9780470720288.ch13. PMID: 416936

Prognosis

Kušnierová P, Zeman D, Jelínek T, Hájek R
Klin Onkol 2020 Summer;33(4):282-285. doi: 10.14735/amko2020280. PMID: 32894957
Bianchi G, Sohani AR
Curr Gastroenterol Rep 2018 Jan 25;20(1):3. doi: 10.1007/s11894-018-0608-y. PMID: 29372346
Wahner-Roedler DL, Kyle RA
Best Pract Res Clin Haematol 2005;18(4):729-46. doi: 10.1016/j.beha.2005.01.029. PMID: 16026747
O'Connell TX, Horita TJ, Kasravi B
Am Fam Physician 2005 Jan 1;71(1):105-12. PMID: 15663032
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070

Clinical prediction guides

Katsuno T, Mizuno S, Mabuchi M, Tsuboi N, Komatsuda A, Maruyama S
BMC Nephrol 2017 Jul 17;18(1):239. doi: 10.1186/s12882-017-0645-z. PMID: 28716013Free PMC Article
Vignon M, Cohen C, Faguer S, Noel LH, Guilbeau C, Rabant M, Higgins S, Hummel A, Hertig A, Francois H, Lequintrec M, Vilaine E, Knebelmann B, Pourrat J, Chauveau D, Goujon JM, Javaugue V, Touchard G, El Karoui K, Bridoux F
Kidney Int 2017 Mar;91(3):720-728. Epub 2017 Jan 6 doi: 10.1016/j.kint.2016.10.026. PMID: 28069266
Oe Y, Nakaya I, Yahata M, Sakuma T, Sato H, Soma J
Intern Med 2010;49(14):1411-5. Epub 2010 Jul 15 doi: 10.2169/internalmedicine.49.3499. PMID: 20647658
O'Connell TX, Horita TJ, Kasravi B
Am Fam Physician 2005 Jan 1;71(1):105-12. PMID: 15663032
Terry WD, Ohms J
Proc Natl Acad Sci U S A 1970 Jun;66(2):558-63. doi: 10.1073/pnas.66.2.558. PMID: 4194868Free PMC Article

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