heavy chain disease

Heavy chain disease is a form of paraproteinemia with a proliferation of cells producing immunoglobulin heavy chains.

The most common type of heavy chain disease is the IgA type, known as αHCD.

Franklin's disease (gamma heavy chain disease) It is characterized by lymphadenopathy,fever,anemia ,malaise,hepatosplenomegaly and weakness.

These heavy chain disease proteins have various deletions, mainly in their amino-terminal part, which causes the heavy chains to lose the ability to form disulfide bonds with the light chains.

The Franklin's disease has been designated in his name as a result of his discovery of the heavy chain disease of which the Franklin disease is a variety of.

IgM and IgG heavy chain diseases, which are known as μHCD and γHCD respectively, are fairly common and are present in various tissues.

The IgM type of heavy chain disease, μHCD, is often misdiagnosed as chronic lymphoid leukemia (CLL) because the two diseases are often associated with each other and show similar symptoms.

Deletion of the N-terminal part of the heavy chain disease protein leads to aggregation and signaling of the B cell receptor, presumably due to the loss of the anti-aggregating properties of the light chain.

The disease variously known as Mediterranean abdominal lymphoma, heavy chain disease, or immunoproliferative small intestinal disease (IPSID), which occurs in young adults in eastern Mediterranean countries, is another version of MALT lymphoma, which responds to antibiotics in its early stages.