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Heavy chain disease. Heavy chain disease is a form of paraproteinemia and plasma cell dyscrasia that involves the proliferation of cells producing immunoglobulin heavy chains. [1] This disease is characterized by an excessive production of heavy chains that are short and truncated. These heavy chain disease proteins have various deletions ...
The immunoglobulin heavy chain (IgH) is the large polypeptide subunit of an antibody (immunoglobulin). In human genome, the IgH gene loci are on chromosome 14. A typical antibody is composed of two immunoglobulin (Ig) heavy chains and two Ig light chains. Several different types of heavy chain exist that define the class or isotype of an antibody.
Heavy Chain Deposition Disease (HCDD) features deposition of heavy chains only. Most commonly, this subtype is formed of monoclonal proteins of the IgG1 subclass of IgG. [2] The immunoglobulin heavy chain in HCDD is frequently a truncated heavy chain. [10] HCDD is the rarest subtype of MIDD. [10]
However, heavy chain diseases are still often classified with plasma cell dyscrasias. [46] The heavy chain diseases are classified as α, γ, and μ heavy chain diseases and are based respectively on >400, 130, and 30–40 case reports as reviewed in a 2014 publication. [11]
Light chains only (or Bence Jones protein). This may be associated with multiple myeloma or AL amyloidosis. Heavy chains only (also known as "heavy chain disease"); Whole immunoglobulins. If immunoglobulins tend to precipitate within blood vessels with cold, that phenomenon takes the name of cryoglobulinaemia.
Light chain deposition disease can affect any organ. [3] Renal involvement is always present and can be identified by microscopic hematuria and proteinuria.Due to the gradual buildup of light chains from plasma filtration, renal function rapidly declines in the majority of patients with LCDD as either acute tubulointerstitial nephritis or rapidly progressing glomerulonephritis.
In addition, light and or heavy chains (the building blocks of antibodies) may be secreted in isolation: κ- or λ-light chains or any of the five types of heavy chains (α-, γ-, δ-, ε- or μ-heavy chains). People without evidence of a monoclonal protein may have "nonsecretory" myeloma (not producing immunoglobulins); this represents about 3 ...
The rearrangements of heavy-chains are different from the light chains because DNA undergoes rearrangements of V-D-J gene segments in the heavy chains. These reorganizations of gene segments produce gene sequence from 5 prime to 3 prime ends such as a short leader exon, an intron, a joined VDJ segment, a second intron and several gene segments.