Heavy chain disease.

Thomas Elmer Witzig, Dietlind L. Wahner-Roedler

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)247-254
Number of pages8
JournalCurrent Treatment Options in Oncology
Volume3
Issue number3
StatePublished - Jun 2002

Fingerprint

Heavy Chain Disease
Non-Hodgkin's Lymphoma
Immunoglobulin Heavy Chains
Immunoproliferative Small Intestinal Disease
Monoclonal Gammopathy of Undetermined Significance
B-Lymphocytes
Anti-Bacterial Agents
Drug Therapy
B-Cell Lymphoma
Hygiene

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Witzig, T. E., & Wahner-Roedler, D. L. (2002). Heavy chain disease. Current Treatment Options in Oncology, 3(3), 247-254.

Heavy chain disease. / Witzig, Thomas Elmer; Wahner-Roedler, Dietlind L.

In: Current Treatment Options in Oncology, Vol. 3, No. 3, 06.2002, p. 247-254.

Research output: Contribution to journalArticle

Witzig, TE & Wahner-Roedler, DL 2002, 'Heavy chain disease.', Current Treatment Options in Oncology, vol. 3, no. 3, pp. 247-254.
Witzig TE, Wahner-Roedler DL. Heavy chain disease. Current Treatment Options in Oncology. 2002 Jun;3(3):247-254.
Witzig, Thomas Elmer ; Wahner-Roedler, Dietlind L. / Heavy chain disease. In: Current Treatment Options in Oncology. 2002 ; Vol. 3, No. 3. pp. 247-254.
@article{fedbcd864c1f4e538659df1d6bb74615,
title = "Heavy chain disease.",
abstract = "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.",
author = "Witzig, {Thomas Elmer} and Wahner-Roedler, {Dietlind L.}",
year = "2002",
month = "6",
language = "English (US)",
volume = "3",
pages = "247--254",
journal = "Current Treatment Options in Oncology",
issn = "1527-2729",
publisher = "Springer New York",
number = "3",

}

TY - JOUR

T1 - Heavy chain disease.

AU - Witzig, Thomas Elmer

AU - Wahner-Roedler, Dietlind L.

PY - 2002/6

Y1 - 2002/6

N2 - 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.

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.

UR - http://www.scopus.com/inward/record.url?scp=0036616817&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0036616817&partnerID=8YFLogxK

M3 - Article

C2 - 12057070

AN - SCOPUS:0036616817

VL - 3

SP - 247

EP - 254

JO - Current Treatment Options in Oncology

JF - Current Treatment Options in Oncology

SN - 1527-2729

IS - 3

ER -