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IgA heavy chain paraproteinemia

MedGen UID:
1705867
•Concept ID:
C5209276
•
Finding
Synonym: Alpha heavy chain disease
 
HPO: HP:0020194

Definition

An abnormal IgA heavy chain in the circulation and typically produced by a clonal population of B-cell derived plasma cells. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIgA heavy chain paraproteinemia

Professional guidelines

PubMed

Heavy/light chain assay as a biomarker for diagnosis and follow-up of multiple myeloma.
Chae H, Han E, Yoo J, Lee J, Lee JJ, Cha K, Kim M, Kim Y, Lee SE, Min CK
Clin Chim Acta 2018 Apr;479:7-13. Epub 2018 Jan 8 doi: 10.1016/j.cca.2018.01.010. PMID: 29324245
Benefits of new immunoglobulin-derived biomarkers for the diagnosis and follow-up of patients with dysglobulinemia.
Ermak N, Nguyen-Khoa T, Alyanakian MA
Ann Biol Clin (Paris) 2016 Oct 1;74(5):597-605. doi: 10.1684/abc.2016.1180. PMID: 27707674
The clinical relevance and management of monoclonal gammopathy of undetermined significance and related disorders: recommendations from the European Myeloma Network.
van de Donk NW, Palumbo A, Johnsen HE, Engelhardt M, Gay F, Gregersen H, Hajek R, Kleber M, Ludwig H, Morgan G, Musto P, Plesner T, Sezer O, Terpos E, Waage A, Zweegman S, Einsele H, Sonneveld P, Lokhorst HM; European Myeloma Network
Haematologica 2014 Jun;99(6):984-96. Epub 2014 Mar 21 doi: 10.3324/haematol.2013.100552. PMID: 24658815Free PMC Article

Recent clinical studies

Etiology

Clinicopathological Characteristics of Light and Heavy Chain Deposition Disease: A Case Series.
Wang Y, Chen D, Hu R, Zhang Y, Liang D, Xu F, Liu F, Zhu X, Lin Y, Yang X, Liu X, Xing G, Liang S, Zeng C
Am J Kidney Dis 2024 Oct;84(4):447-456.e1. Epub 2024 May 14 doi: 10.1053/j.ajkd.2024.03.021. PMID: 38750878
The relationship between hypogammaglobulinemia, monoclonal gammopathy of undetermined significance and humoral immunodeficiency: a case series.
Zemble RM, Takach PA, Levinson AI
J Clin Immunol 2011 Oct;31(5):737-43. Epub 2011 Jun 5 doi: 10.1007/s10875-011-9548-0. PMID: 21643891
Heavy chain disease.
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070
Ultrastructural characterisation of the M protein in nerve biopsy of patients with POEMS syndrome.
Adams D, Said G
J Neurol Neurosurg Psychiatry 1998 Jun;64(6):809-12. doi: 10.1136/jnnp.64.6.809. PMID: 9647319Free PMC Article
Monoclonal gammopathies in children.
Gerritsen E, Vossen J, van Tol M, Jol-van der Zijde C, Van der Weijden-Ragas R, Radl J
J Clin Immunol 1989 Jul;9(4):296-305. doi: 10.1007/BF00918661. PMID: 2504763

Diagnosis

Analysis of Multiple Bands on Serum Protein Immunofixation Electrophoresis: Challenge in Interpretation of Clonality in a Patient with Light Chain-Predominant Multiple Myeloma.
Nwogbo OV, Jin Y, Sliker T, Wilhite D, Singh G
Lab Med 2021 Sep 1;52(5):503-508. doi: 10.1093/labmed/lmab001. PMID: 33693845
Severe enteropathy caused by α-heavy chain disease lacking detectable M-proteins.
Kurimoto M, Sonoki T, Nakamura Y, Yamamoto Y, Maeda Y, Kuriyama K, Mushino T, Hosoi H, Murata S, Shimanuki M, Hatanaka K, Hanaoka N, Kato J, Nakakuma H
Intern Med 2014;53(6):581-5. doi: 10.2169/internalmedicine.53.1625. PMID: 24633028
Challenges with serum protein electrophoresis in assessing progression and clinical response in patients with Waldenström macroglobulinemia.
Uljon SN, Treon SP, Tripsas CK, Lindeman NI
Clin Lymphoma Myeloma Leuk 2013 Apr;13(2):247-9. doi: 10.1016/j.clml.2013.03.001. PMID: 23664026
Heavy chain disease.
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070
Ultrastructural characterisation of the M protein in nerve biopsy of patients with POEMS syndrome.
Adams D, Said G
J Neurol Neurosurg Psychiatry 1998 Jun;64(6):809-12. doi: 10.1136/jnnp.64.6.809. PMID: 9647319Free PMC Article

Therapy

Recovery of uninvolved heavy/light chain pair immunoparesis in newly diagnosed transplant-eligible myeloma patients complements the prognostic value of minimal residual disease detection.
Lakhwani S, Rosiñol L, Puig N, Pico-Picos MA, Medina-González L, Martínez-López J, Paiva B, Cedena MT, Oriol A, Ríos-Tamayo R, Blanchard MJ, Jarque I, Bargay J, Moraleda JM, Carrillo-Cruz E, Sureda A, Krsnik I, González E, Casado LF, Martí JM, Encinas C, De Arriba F, Palomera L, Sampol A, González-Montes Y, Motlló C, De La Cruz J, Alonso R, Mateos MV, Bladé J, Lahuerta JJ, San-Miguel J, Hernández MT
Haematologica 2024 Jun 1;109(6):1909-1917. doi: 10.3324/haematol.2023.284154. PMID: 38031761Free PMC Article
Systemic Amyloidosis due to Low-Grade Lymphoma.
Wechalekar AD, Chakraborty R, Lentzsch S
Hematol Oncol Clin North Am 2020 Dec;34(6):1027-1039. Epub 2020 Oct 1 doi: 10.1016/j.hoc.2020.08.016. PMID: 33099421
Severe enteropathy caused by α-heavy chain disease lacking detectable M-proteins.
Kurimoto M, Sonoki T, Nakamura Y, Yamamoto Y, Maeda Y, Kuriyama K, Mushino T, Hosoi H, Murata S, Shimanuki M, Hatanaka K, Hanaoka N, Kato J, Nakakuma H
Intern Med 2014;53(6):581-5. doi: 10.2169/internalmedicine.53.1625. PMID: 24633028
Familial predisposition to monoclonal gammopathies: deviations in B-cell biology.
Ögmundsdóttir HM, Valgeirsdóttir S, Schiffhauer HR, Óskarsdóttir LB, Steingrímsdóttir H, Haraldsdóttir V
Clin Lymphoma Myeloma Leuk 2013 Apr;13(2):191-3. Epub 2013 Mar 13 doi: 10.1016/j.clml.2013.02.017. PMID: 23490993
Where disease pathogenesis meets protein formulation: renal deposition of immunoglobulin aggregates.
Demeule B, Gurny R, Arvinte T
Eur J Pharm Biopharm 2006 Feb;62(2):121-30. Epub 2005 Oct 10 doi: 10.1016/j.ejpb.2005.08.008. PMID: 16221544

Prognosis

Challenges with serum protein electrophoresis in assessing progression and clinical response in patients with Waldenström macroglobulinemia.
Uljon SN, Treon SP, Tripsas CK, Lindeman NI
Clin Lymphoma Myeloma Leuk 2013 Apr;13(2):247-9. doi: 10.1016/j.clml.2013.03.001. PMID: 23664026
Immunoglobulin crystal-storing histiocytosis in a pleural effusion from a woman with IgA kapa multiple myeloma: a case report.
Galed-Placed I
Acta Cytol 2006 Sep-Oct;50(5):539-41. doi: 10.1159/000326010. PMID: 17017441
Heavy chain disease.
Witzig TE, Wahner-Roedler DL
Curr Treat Options Oncol 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3. PMID: 12057070
Monoclonal gammopathies in children.
Gerritsen E, Vossen J, van Tol M, Jol-van der Zijde C, Van der Weijden-Ragas R, Radl J
J Clin Immunol 1989 Jul;9(4):296-305. doi: 10.1007/BF00918661. PMID: 2504763
Detection of monoclonal B lymphocytes in multiple myeloma by immunofluorescence tests of surface immunoglobulins.
Sato I, Abo T, Onodera S, Kumagai K
Scand J Haematol 1978 Nov;21(5):433-44. doi: 10.1111/j.1600-0609.1978.tb00391.x. PMID: 366730

Clinical prediction guides

Immunoglobulin crystal-storing histiocytosis in a pleural effusion from a woman with IgA kapa multiple myeloma: a case report.
Galed-Placed I
Acta Cytol 2006 Sep-Oct;50(5):539-41. doi: 10.1159/000326010. PMID: 17017441
Plasma cells composing plasmacytoma have phenotypes different from those of myeloma cells.
Sakai A, Fujii T, Noda M, Hyodo H, Oda K, Kimura A
Am J Hematol 1996 Dec;53(4):251-3. doi: 10.1002/(SICI)1096-8652(199612)53:4<251::AID-AJH9>3.0.CO;2-X. PMID: 8948665
A simplified technique of high resolution two-dimensional electrophoresis: serum immunoglobulins.
Latner AL, Marshall T, Gambie M
Clin Chim Acta 1980 Apr 11;103(1):51-9. doi: 10.1016/0009-8981(80)90230-2. PMID: 6771060
Detection of monoclonal B lymphocytes in multiple myeloma by immunofluorescence tests of surface immunoglobulins.
Sato I, Abo T, Onodera S, Kumagai K
Scand J Haematol 1978 Nov;21(5):433-44. doi: 10.1111/j.1600-0609.1978.tb00391.x. PMID: 366730
Structural characterization of a human monoclonal IgA protein.
Parkhouse RM, Virella G, Dourmashkin RR
Clin Exp Immunol 1971 Apr;8(4):581-91. PMID: 4995936Free PMC Article

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