Heavy chain diseases.
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; alpha-HCD is the most common and has the most uniform presentation, gamma- and mu-HCDs have variable clinical presentations and histopathologic features. HCDs can be thought of as variant types of non-Hodgkin lymphoma: alpha-HCD presents as an extranodal marginal-zone lymphoma of mucosa-associated lymph-node tissue, gamma-HCD as lymphoplasmacytoid non-Hodgkin lymphoma, and mu-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 alpha-HCD, which in its early stage may respond to antibiotics.
Wahner-Roedler
Dietlind L
DL
Division of General Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. wahnerroedler.dietlind@mayo.edu
Kyle
Robert A
RA
D019411:Clinical Laboratory Techniques; D006362:Heavy Chain Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics; D008232:Lymphoproliferative Disorders / Q000209:etiology; D011379:Prognosis
D007143:Immunoglobulin Heavy Chains