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Neoplastic Diseases of the Blood pp 701–728Cite as

Heavy-Chain Disease

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Abstract

The heavy-chain diseases (HCDs) are monoclonal lymphoplasma cell proliferative disorders involving B cells and are characterized by the synthesis of truncated heavy chains without associated light chains. The complex abnormalities of HCD proteins and the usual lack of normal light chains are due to several distinct gene alterations, including somatic mutations, deletions, and insertions. HCDs have been described for the three main immunoglobulin classes. The most frequent is α-HCD; μ-HCD is rare and the incidence of γ-HCD is intermediate. In contrast to γ-HCD and α-HCD, which usually have no detectable monoclonal light chains, the light chains that do not assemble with the secreted deleted heavy chains are produced in about two-thirds of cases of μ-HCD. The monoclonal proteins are always present in the serum and are often in the urine of patients with γ-HCD, but monoclonal heavy chains are infrequent in the urine in μ-HCD and occur in small amounts in α-HCD.

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Abbreviations

HCDD:

Heavy-chain deposition disease

HCD:

Heavy-chain disease

IgA:

Immunoglobulin A

IgD:

Immunoglobulin D

IgG:

Immunoglobulin G

IgM:

Immunoglobulin M

IPSID:

Immunoproliferative small intestinal disease

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Acknowledgments

This paper was supported in part by research grant CA62242 from the National Institutes of Health.

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  1. Division of General Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Dietlind L. Wahner-Roedler M.D.

  2. Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Robert A. Kyle M.D.

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  1. Beth Israel Hospital, Cancer Center, St. Lukes-Roosevelt Hospital Center, 10th Avenue 1000, New York, 10019, New York, USA

    Peter H. Wiernik

  2. , Department of Hematology, Imperial College of London, Du Cane Road 150, London, W12 0NN, United Kingdom

    John M. Goldman

  3. Continuum Cancer Centers, Department of Medicine, St. Luke's-Roosevelt Hospital Center, 10th Avenue 1000, New York, 10019, New York, USA

    Janice P. Dutcher

  4. , Division of Hematology, Mayo Clinic, First Street SW. 200, Rochester, 55905, Minnesota, USA

    Robert A. Kyle

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Wahner-Roedler, D.L., Kyle, R.A. (2013). Heavy-Chain Disease. In: Wiernik, P., Goldman, J., Dutcher, J., Kyle, R. (eds) Neoplastic Diseases of the Blood. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-3764-2_36

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