PMID- 16026747 OWN - NLM STAT- MEDLINE DCOM- 20051006 LR - 20111117 IS - 1521-6926 (Print) IS - 1521-6926 (Linking) VI - 18 IP - 4 DP - 2005 TI - Heavy chain diseases. PG - 729-46 AB - 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. FAU - Wahner-Roedler, Dietlind L AU - Wahner-Roedler DL AD - Division of General Internal Medicine, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA. wahnerroedler.dietlind@mayo.edu FAU - Kyle, Robert A AU - Kyle RA LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Best Pract Res Clin Haematol JT - Best practice & research. Clinical haematology JID - 101120659 RN - 0 (Immunoglobulin Heavy Chains) SB - IM MH - Clinical Laboratory Techniques MH - Heavy Chain Disease/*diagnosis/drug therapy/genetics/pathology MH - Humans MH - Immunoglobulin Heavy Chains/genetics MH - Lymphoproliferative Disorders/etiology MH - Prognosis RF - 38 EDAT- 2005/07/20 09:00 MHDA- 2005/10/07 09:00 CRDT- 2005/07/20 09:00 PHST- 2005/07/20 09:00 [pubmed] PHST- 2005/10/07 09:00 [medline] PHST- 2005/07/20 09:00 [entrez] AID - S1521-6926(05)00030-7 [pii] AID - 10.1016/j.beha.2005.01.029 [doi] PST - ppublish SO - Best Pract Res Clin Haematol. 2005;18(4):729-46. doi: 10.1016/j.beha.2005.01.029.