Heavy chain diseases

Dietlind L. Wahner-Roedler, Robert A. Kyle

Research output: Contribution to journalReview article

49 Scopus citations

Abstract

Heavy chain diseases (HCDs) are rare B-cell lymphoplasma-cell proliferative disorders characterized by production of truncated monoclonal immunoglobulin heavy chains without associated light chains. HCDs involving the three main immunoglobulin classes have been described; α-HCD is the most common and has the most uniform presentation, γ- and μ-HCDs have variable clinical presentations and histopathologic features. HCDs can be thought of as variant types of non-Hodgkin lymphoma: α-HCD presents as an extranodal marginal-zone lymphoma of mucosa-associated lymph-node tissue, γ-HCD as lymphoplasmacytoid non-Hodgkin lymphoma, and μ-HCD as small lymphocytic non-Hodgkin lymphoma or chronic lymphocytic leukemia. Diagnosis of HCD requires documentation of a deleted immunoglobulin heavy chain without a bound light chain in the serum or urine. Prognosis is variable, and no standardized effective treatment programs are available except for α-HCD, which in its early stage may respond to antibiotics.

Original languageEnglish (US)
Pages (from-to)729-746
Number of pages18
JournalBest Practice and Research: Clinical Haematology
Volume18
Issue number4 SPEC. ISS.
DOIs
StatePublished - Dec 2005

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Keywords

  • Heavy chain
  • Heavy chain diseases
  • Monoclonal gammopathy

ASJC Scopus subject areas

  • Oncology
  • Clinical Biochemistry

Cite this

Wahner-Roedler, D. L., & Kyle, R. A. (2005). Heavy chain diseases. Best Practice and Research: Clinical Haematology, 18(4 SPEC. ISS.), 729-746. https://doi.org/10.1016/j.beha.2005.01.029