What is plasma cell dyscrasia?
Definition. Plasma cell dyscrasias are a monoclonal proliferation of plasma cells that produce a clonal immunoglobulin protein (i.e., monoclonal gammopathies or paraproteinemias). They are derived from malignant B lymphocytes. Common plasma cell dyscrasias include multiple myeloma and Waldenström’s macroglobulinemia.
What are the types of plasma cells?
The current findings suggest that after the process of affinity maturation in germinal centers, plasma cells develop into one of two types of cells: short-lived plasma cells (SLPC) or long-lived plasma cells (LLPC).
How is multiple myeloma classified?
In myeloma, all the abnormal plasma cells make the same antibody. Therefore, the myeloma can be classified by the type of light and heavy chains produced, such as IgG kappa, IgG lambda, IgA kappa, or IgA lambda, etc. The most common type of heavy chain produced in myeloma is IgG, followed by IgA and then IgD.
How is plasma cell dyscrasia diagnosed?
Flow cytometry is also useful in diagnosis of plasma cell dyscrasias. Plasma cells are CD38 positive (bright) and CD138 positive (although less sensitive). Plasma cells in the blood and tonsils are CD45 positive, whereas in the bone marrow, plasma cells may be either CD45 positive or negative.
What are the functions of plasma cells?
Plasma cells are professional secretory cells, which function as cellular factories for immunoglobulin synthesis and secretion. Being the sole cell type responsible for antibody secretion they play an essential role in the immune response against a broad spectrum of pathogens.
What are plasma cells derived from?
Plasma cells arise from antigen-activated B cells in secondary lymphoid organs such as the spleen and lymph nodes. Remarkably, shortly after their formation plasma cells tend to home primarily to the bone marrow where they may persist for months or even years.
What is the difference between myeloma and multiple myeloma?
There is no difference. The terms are used interchangeably. Myeloma is derived from the Greek words “myel” (meaning marrow) and “oma” (meaning tumor). Because malignant plasma cells almost always occur in more than one location, it is often referred to as multiple myeloma.
What is the meaning of blood dyscrasias?
Dyscrasia is a nonspecific term that refers to a disease or disorder, especially of the blood. The latter is called a blood dyscrasia. Before modern medicine, it meant an imbalance of four body fluids: blood, bile, lymph, and phlegm.
Which are characteristics of a plasma cell?
Plasma cells vary in size from 14 to 20 micrometers. They are round-to-ovoid cells containing abundant deep blue cytoplasm with a pale perinuclear area corresponding to the Golgi apparatus. They have a round, eccentrically placed nucleus with coarse chromatin arranged in a clock face (art wheel) pattern.
Where are plasma cells made?
bone marrow
Plasma cells are found in bone marrow, where blood cells are made. Normal bone marrow contains few plasma cells. A person with multiple myeloma often has many abnormal plasma cells (myeloma cells) in the bone marrow.
What are the three stages of multiple myeloma?
Stages
- Durie-Salmon Staging. In this system, there are three stages of myeloma: Stage I, Stage II, and Stage III.
- International Staging System (ISS)
- Monoclonal Gammopathy of Undetermined Significance (MGUS)
- Asymptomatic (smoldering/indolent) myeloma.
- Symptomatic (active) myeloma.
What are the types of blood dyscrasia?
The blood disorder list includes:
- Anemia.
- Hemophilia.
- Leukocytosis.
- Polycythemia vera.
- Sickle cell disease.
- Thalassemia.
- Von Willebrand disease.
What is an example of blood dyscrasia?
Examples include bone marrow disorders, some medications such as chemotherapy, certain viral infections, etc. Increased destruction of platelets. This can be caused by immune disorders (immune thrombocytopenia) such as idiopathic thrombocytopenic purpura). Blood loss such as from bleeding.
What is plasmacytic dyscrasia?
Plasma cell dyscrasias are a heterogeneous group of disorders caused by the monoclonal proliferation of lymphoplasmacytic cells in the bone marrow. Multiple myeloma is the most serious and prevalent plasma cell dyscrasia, with a median age of onset of 60 years.
What are the possible complications of plasma cell dyscrasia?
Serious and potentially life-threatening paraneoplastic complications can occur in plasma cell dyscrasias regardless of tumor cell burden, myeloma protein levels, or the presence of other criteria suggesting the dyscrasia has entered a malignant phase.
Which tests are performed in the workup of plasma cell dyscrasia?
The presence of a plasma cell dyscrasia must be investigated with serum and urine immunofixation electrophoresis; generally patients present with an IgG or IgA light-chain monoclonal gammopathy. Endocrinological abnormalities must be searched with assessment of thyrotropin, glucose, and sex hormone levels.
What are the types of plasma cell dyscrasia-associated amyloidosis?
There are two different types of plasma cell dyscrasia-associated amyloidosis syndromes: amyloid light chain amyloidosis (AL amyloidosis) in which amyloid deposits consist of free light chains and amyloid heavy chain amyloidosis (AH amyloidosis) in which amyloid deposits contain only free heavy chains.