What is a heavy chain disease?
What is a heavy chain disease?
Heavy chain diseases are plasma cell cancers in which a clone of plasma cells produces a large quantity of pieces of abnormal antibodies called heavy chains. (See also Overview of Plasma Cell Disorders. They begin when a single plasma cell multiplies excessively.
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 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 Franklin’s disease?
Franklin’s disease is a type of heavy chain disease (malignant plasma cell disorder) whose symptoms include fatigue, enlargement of the lymph nodes, and susceptibility to infections. It can be treated with chemotherapy.
What is the difference between heavy and light chains?
The light chain is made up of two such immunoglobulin domains, whereas the heavy chain of the IgG antibody contains four (see Fig. 3.1a). This suggests that the immunoglobulin chains have evolved by repeated duplication of an ancestral gene corresponding to a single domain.
What is IgA heavy chain?
IgA Heavy Chain Disease (alpha chain disease) 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.
What are heavy and light chains?
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.
What is Ipsid?
IPSID is a variant of the B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), which involves mainly the proximal small intestine resulting in malabsorption, diarrhea, and abdominal pain. Geographically, IPSID is most prevalent in the Middle East and Africa.
What is the difference between light and heavy chains?
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 Multiple Myeloma a heavy chain disease?
The most common type of heavy chain produced in myeloma is IgG, followed by IgA and then IgD. IgM myelomas are rare, but when IgM is elevated in the blood, the patient more likely has a related disorder, known as Waldenstrom’s Macroglobulinemia.
What is heavy light chain?
The heavy/light chain (HLC) immunoassay is an additional tool for measuring intact immunoglobulin monoclonal proteins. Therefore, we aimed to examine the clinical utility of the HLC assay for the disease monitoring of IgG and IgA multiple myeloma (MM) patients.
What are the different types of heavy chain diseases?
Heavy chain diseases are neoplastic plasma cell disorders characterized by overproduction of monoclonal immunoglobulin heavy chains. Symptoms, diagnosis, and treatment vary according to the specific disorder. Heavy chain diseases are plasma cell disorders that are typically malignant.
What kind of proteins are produced in heavy chain diseases?
In most plasma cell disorders, M-proteins (monoclonal immunoglobulin protein) are structurally similar to normal antibody molecules. In contrast, in heavy chain diseases, incomplete monoclonal immunoglobulins (true paraproteins) are produced.
What are the symptoms of gamma heavy chain disease?
IgG/γHCD. Franklin’s disease (gamma heavy chain disease) It is a very rare B-cell lymphoplasma cell proliferative disorder which may be associated with autoimmune diseases and infection is a common characteristic of the disease. It is characterized by lymphadenopathy, fever, anemia, malaise, hepatosplenomegaly, and weakness.
Is there a difference between heavy chain and malignant lymphoma?
IgG heavy chain disease is generally similar to an aggressive malignant lymphoma but is occasionally asymptomatic and benign. IgG heavy chain disease occurs primarily in older men but can occur in children.