How is heavy chain disease diagnosed?

Gamma heavy chain disease is diagnosed based on the symptoms, a clinical exam, and laboratory tests to analyze the urine, blood, and bone marrow. A bone marrow biopsy may be done to remove a small piece of bone marrow for examination under the microscope.

How do you treat heavy chain disease?

There is no standardized regimen for the treatment of alpha heavy chain disease, but doxorubicin-containing combination chemotherapy such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), CHVP (cyclophosphamide, doxorubicin, teniposide, and prednisone), or ABV (doxorubicin, bleomycin, and …

What is a heavy chain disease?

The heavy chain diseases (HCDs) are rare B cell proliferative disorders characterized by the production of a monoclonal (M) protein consisting of a portion of the immunoglobulin heavy chain without a bound light chain [1-6].

What is heavy chain myeloma?

The heavy chain diseases (HCDs) are rare B-cell malignancies characterized by the production of a monoclonal immunoglobulin heavy chain without an associated light chain. There are three types of HCD, defined by the class of immunoglobulin heavy chain produced: IgA (α-HCD), IgG (γ-HCD), and IgM (μ-HCD).

Is heavy chain disease a genetic disorder?

The heavy chain diseases (HCDs) are B-cell proliferative disorders characterized by production of abnormal, structurally incomplete, immunoglobulin heavy chains without the corresponding light chains. The abnormal HCD proteins are the result of gene mutations, deletions, or insertions.

What does the heavy chain of an antibody do?

Heavy-chain antibodies can bind antigens despite having only VH domains. This observation has led to the development of a new type of antibody fragments with potential use as drugs, so-called single-domain antibodies.

What is IgA heavy chain?

Alpha heavy chain disease (IgA heavy chain disease) affects mainly younger adults of Middle Eastern or Mediterranean ancestry. Infiltration of the intestinal tract wall by cancerous plasma cells often prevents proper absorption of nutrients from food (malabsorption ), resulting in severe diarrhea and weight loss.

What is Alpha heavy chain disease?

Disease definition. A type of HCD characterized by the production of incomplete monoclonal alpha-heavy chains without associated light chains. Alpha-HCD is considered to be a subtype of immunoproliferative small intestinal disease (IPSID).

What is the heavy chain of IgG?

IgG antibodies are large molecules, having a molecular weight of approximately 150 kDa, composed of two different kinds of polypeptide chain. One, of approximately 50 kDa, is termed the heavy or H chain, and the other, of 25 kDa, is termed the light or L chain (Fig. 3.2).

Which is the heaviest antibody?

IgM
IgM is the largest antibody and the first one to be synthesized in response to an antigen or microbe, accounting for 5% of all immunoglobulins present in the blood. IgM typically exists as polymers of identical subunits, with a pentameric form as the prevalent one.

Is IgA a heavy chain?

IgA heavy chain disease is the most common heavy chain disease and is similar to Mediterranean lymphoma (immunoproliferative small intestinal disease). IgA heavy chain disease usually appears between ages 10 and 30 and is geographically concentrated in the Middle East.