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Mu-heavy chain disease

MedGen UID:
66875
•Concept ID:
C0242310
•
Neoplastic Process
Synonyms: mu Chain Disease; mu-Chain Disease; mu-Chain Diseases
SNOMED CT: Mu heavy chain disease (61493004); IgM heavy chain disease (61493004); Mu heavy chain disease (6381009)
 
Monarch Initiative: MONDO:0015044
Orphanet: ORPHA100024

Definition

A type of HCD characterized by the production of incomplete monoclonal mu-heavy chains without associated light chains. The clinical presentation resembles that of patients with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMu-heavy chain disease

Professional guidelines

PubMed

Chronic lymphocytic leukemia treatment algorithm 2022.
Hampel PJ, Parikh SA
Blood Cancer J 2022 Nov 29;12(11):161. doi: 10.1038/s41408-022-00756-9. PMID: 36446777Free PMC Article
Immunoglobulin light chain amyloidosis: 2022 update on diagnosis, prognosis, and treatment.
Gertz MA
Am J Hematol 2022 Jun 1;97(6):818-829. Epub 2022 Apr 25 doi: 10.1002/ajh.26569. PMID: 35429180
Mantle cell lymphoma: 2019 update on the diagnosis, pathogenesis, prognostication, and management.
Jain P, Wang M
Am J Hematol 2019 Jun;94(6):710-725. Epub 2019 Apr 19 doi: 10.1002/ajh.25487. PMID: 30963600

Recent clinical studies

Etiology

Cast nephropathy in mu heavy chain disease.
Preud'homme JL, Bauwens M, Dumont G, Goujon JM, Dreyfus B, Touchard G
Clin Nephrol 1997 Aug;48(2):118-21. PMID: 9285150

Diagnosis

Mu heavy chain disease with MYD88 L265P mutation: an unusual manifestation of lymphoplasmacytic lymphoma.
Baloda V, Wheeler SE, Murray DL, Kohlhagen MC, Vos JA, Yatsenko SA, Agha ME, Djokic M, Swerdlow SH, Bailey NG
Diagn Pathol 2022 Aug 5;17(1):63. doi: 10.1186/s13000-022-01244-1. PMID: 35932039Free PMC Article
Light chain proteinuria revealing mu-heavy chain disease: an atypical presentation of Waldenström macroglobulinemia in two cases.
Vergneault H, Bengoufa D, Frazier-Mironer A, Brocheriou I, Bitoun S, Villesuzanne C, Talbot A, Harel S, Arnulf B, Royer B
Haematologica 2021 Jul 1;106(7):2034-2036. doi: 10.3324/haematol.2020.277137. PMID: 33596645Free PMC Article
The heavy chain diseases: clinical and pathologic features.
Bianchi G, Anderson KC, Harris NL, Sohani AR
Oncology (Williston Park) 2014 Jan;28(1):45-53. PMID: 24683718
Capillary immunotyping electrophoresis and high resolution two-dimensional electrophoresis for the detection of mu-heavy chain disease.
Maisnar V, Tichy M, Stulik J, Urban P, Adam Z, Kadlckova E, Vavrova J, Palicka V, Jebavy L, Kodet R, Buchler T, Hajek R
Clin Chim Acta 2008 Mar;389(1-2):171-3. Epub 2007 Nov 6 doi: 10.1016/j.cca.2007.10.035. PMID: 18036562
Clinical aspects of multiple myeloma and related disorders including amyloidosis.
Kyle RA
Pathol Biol (Paris) 1999 Feb;47(2):148-57. PMID: 10192881

Therapy

Successful treatment of mu-heavy chain disease with fludarabine monophosphate: a case report.
Yanai M, Maeda A, Watanabe N, Sugimoto N, Matsushita A, Nagai K, Oida T, Takahashi T
Int J Hematol 2004 Feb;79(2):174-7. doi: 10.1532/ijh97.03053. PMID: 15005347
A case of mu heavy-chain disease associated with hyperglobulinemia, anemia, and a positive Coombs test.
Witzens M, Egerer G, Stahl D, Werle E, Goldschmidt H, Haas R
Ann Hematol 1998 Nov;77(5):231-4. doi: 10.1007/s002770050448. PMID: 9858149

Prognosis

The heavy chain diseases: clinical and pathologic features.
Bianchi G, Anderson KC, Harris NL, Sohani AR
Oncology (Williston Park) 2014 Jan;28(1):45-53. PMID: 24683718
Successful treatment of mu-heavy chain disease with fludarabine monophosphate: a case report.
Yanai M, Maeda A, Watanabe N, Sugimoto N, Matsushita A, Nagai K, Oida T, Takahashi T
Int J Hematol 2004 Feb;79(2):174-7. doi: 10.1532/ijh97.03053. PMID: 15005347
Cast nephropathy in mu heavy chain disease.
Preud'homme JL, Bauwens M, Dumont G, Goujon JM, Dreyfus B, Touchard G
Clin Nephrol 1997 Aug;48(2):118-21. PMID: 9285150
A DNA insertion/deletion necessitates an aberrant RNA splice accounting for a mu heavy chain disease protein.
Bakhshi A, Guglielmi P, Siebenlist U, Ravetch JV, Jensen JP, Korsmeyer SJ
Proc Natl Acad Sci U S A 1986 Apr;83(8):2689-93. doi: 10.1073/pnas.83.8.2689. PMID: 3085103Free PMC Article

Clinical prediction guides

A case of mu heavy-chain disease associated with hyperglobulinemia, anemia, and a positive Coombs test.
Witzens M, Egerer G, Stahl D, Werle E, Goldschmidt H, Haas R
Ann Hematol 1998 Nov;77(5):231-4. doi: 10.1007/s002770050448. PMID: 9858149
A DNA insertion/deletion necessitates an aberrant RNA splice accounting for a mu heavy chain disease protein.
Bakhshi A, Guglielmi P, Siebenlist U, Ravetch JV, Jensen JP, Korsmeyer SJ
Proc Natl Acad Sci U S A 1986 Apr;83(8):2689-93. doi: 10.1073/pnas.83.8.2689. PMID: 3085103Free PMC Article

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