PMID- 12057070
OWN - NLM
STAT- MEDLINE
DCOM- 20020709
LR  - 20191106
IS  - 1527-2729 (Print)
IS  - 1534-6277 (Linking)
VI  - 3
IP  - 3
DP  - 2002 Jun
TI  - Heavy chain disease.
PG  - 247-54
AB  - The heavy chain diseases (HCDs) are rare B-cell malignancies that are 
      distinguished by the production of a monoclonal immunoglobulin heavy chain (HC) 
      without an associated light chain by the malignant B-cells. There are three types 
      of HCD defined by the class of immunoglobulin (Ig) HC produced: IgA (alpha-HCD), 
      IgG (gamma-HCD), and IgM (mu-HCD). Alpha-HCD is the most common and occurs most 
      commonly as intestinal malabsorption in a young adult from a country bordering 
      the Mediterranean Sea. Treatment consists of antibiotics and improved nutrition 
      and hygiene. Surgery is occasionally required for patients with bulky masses at 
      risk for bowel perforation. If there is no response to antibiotics or if 
      aggressive non-Hodgkin's lymphoma (NHL) is diagnosed, the patient should be 
      treated with chemotherapy. Gamma- and mu-HCD are rare and essentially are found 
      in patients with a B-cell NHL that produces an abnormal Ig heavy chain. These 
      patients occasionally may be diagnosed with a monoclonal gammopathy of 
      undetermined significance (MGUS). Patients with MGUS with NHL should be 
      administered chemotherapy. Screening the serum and urine of patients with 
      lymphoplasmacytoid NHL would likely identify more patients with gamma- or mu-HCD.
FAU - Witzig, Thomas E
AU  - Witzig TE
AD  - Division of Hematology, Mayo Clinic and Mayo Foundation, 200 SW First Street, 
      Rochester, MN 55905, USA. witzig@mayo.edu
FAU - Wahner-Roedler, Dietlind L
AU  - Wahner-Roedler DL
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Curr Treat Options Oncol
JT  - Current treatment options in oncology
JID - 100900946
RN  - 0 (Immunoglobulin Heavy Chains)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Gene Rearrangement, B-Lymphocyte, Heavy Chain/genetics
MH  - Heavy Chain Disease/genetics/immunology/*therapy
MH  - Humans
MH  - Immunoglobulin Heavy Chains/genetics
MH  - Incidence
MH  - Middle Aged
MH  - Radiotherapy
RF  - 36
EDAT- 2002/06/12 10:00
MHDA- 2002/07/10 10:01
CRDT- 2002/06/12 10:00
PHST- 2002/06/12 10:00 [pubmed]
PHST- 2002/07/10 10:01 [medline]
PHST- 2002/06/12 10:00 [entrez]
AID - 10.1007/s11864-002-0014-3 [doi]
PST - ppublish
SO  - Curr Treat Options Oncol. 2002 Jun;3(3):247-54. doi: 10.1007/s11864-002-0014-3.