Heavy chain disease

Division of Hematology, Mayo Clinic and Mayo Foundation, 200 SW First Street, Rochester, MN 55905, USA.
Current Treatment Options in Oncology (Impact Factor: 3.24). 07/2002; 3(3):247-54. DOI: 10.1007/s11864-002-0014-3
Source: PubMed


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.

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    • "Alpha heavy chain disease is a rare disorder also known as Mediterranean lymphoma or immunoproliferative small intestine disease [3]. It is a rare malignancy that shows a distinct geographic epidemiology with most cases reported from northern Africa, Israel, and the Middle East in patients of low socioeconomic status and poor nutrition and who live in areas with poor hygiene [3] [4]. The disorder typically presents in patients < 30 y of age with symptoms that include intestinal malabsorption, diarrhea, weight loss, and abdominal pain [4]. "
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