Acquired pure red cell aplasia
Encyclopedia
Pure red cell aplasia or erythroblastopenia refers to a type of anemia
affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow
ceases to produce red blood cells. The condition has been first described by Paul Kaznelson
in 1922.
treatment such as ciclosporin
in many patients, though this approach is not without risk.
It has also been shown to respond to treatments with Rituxan.
Anemia
Anemia is a decrease in number of red blood cells or less than the normal quantity of hemoglobin in the blood. However, it can include decreased oxygen-binding ability of each hemoglobin molecule due to deformity or lack in numerical development as in some other types of hemoglobin...
affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow
Bone marrow
Bone marrow is the flexible tissue found in the interior of bones. In humans, bone marrow in large bones produces new blood cells. On average, bone marrow constitutes 4% of the total body mass of humans; in adults weighing 65 kg , bone marrow accounts for approximately 2.6 kg...
ceases to produce red blood cells. The condition has been first described by Paul Kaznelson
Paul Kaznelson
Paul Kaznelson was a Polish-born Czech scientist is credited with describing the first case of pure red cell aplasia. He is also known for his contribution to the discovery of the therapeutic role of splenectomy in idiopathic thrombocytopenic purpura.Much of his academic work appeared in the 1920s...
in 1922.
Causes
Causes include:- Autoimmune diseaseAutoimmune diseaseAutoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. In other words, the body actually attacks its own cells. The immune system mistakes some part of the body as a pathogen and attacks it. This may be restricted to...
.
- ThymomaThymomaThymoma is a tumor originating from the epithelial cells of the thymus. Thymoma is an uncommon tumor, best known for its association with the neuromuscular disorder myasthenia gravis. Thymoma is found in 15% of patients with myasthenia gravis. Once diagnosed, thymomas may be removed surgically...
or
- ViralVirusA virus is a small infectious agent that can replicate only inside the living cells of organisms. Viruses infect all types of organisms, from animals and plants to bacteria and archaea...
infections such as HIVHIVHuman immunodeficiency virus is a lentivirus that causes acquired immunodeficiency syndrome , a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thrive...
, herpesHerpes simplex virusHerpes simplex virus 1 and 2 , also known as Human herpes virus 1 and 2 , are two members of the herpes virus family, Herpesviridae, that infect humans. Both HSV-1 and HSV-2 are ubiquitous and contagious...
, parvovirus B19Parvovirus B19The B19 virus, generally referred to as parvovirus B19 or sometimes erythrovirus B19, was the first known human virus in the family of parvoviruses, genus erythrovirus...
(Fifth diseaseFifth diseaseErythema infectiosum or fifth disease is one of several possible manifestations of infection by erythrovirus, previously called parvovirus B19. The disease is also referred to as slapped cheek syndrome, slapcheek, slap face or slapped face. In Japan the disease is called 'apple sickness' or...
), or hepatitis.
- Lymphoproliferative. Association of pure red cell aplasia with T large granular lymphocyte leukemia is well recognized, especially in China.
- IdiopathicIdiopathicIdiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...
. Many cases of PRCA are considered idiopathic in that there is no discernible cause detected.
- DrugsDRUGSDestroy Rebuild Until God Shows are an American post-hardcore band formed in 2010. They released their debut self-titled album on February 22, 2011.- Formation :...
, e.g., erythropoietinErythropoietinErythropoietin, or its alternatives erythropoetin or erthropoyetin or EPO, is a glycoprotein hormone that controls erythropoiesis, or red blood cell production...
or mycophenolic acidMycophenolic acidMycophenolic acid INN or mycophenolate is an immunosuppressant drug used to prevent rejection in organ transplantation. It inhibits an enzyme needed for the growth of T cells and B cells. It was initially marketed as the prodrug mycophenolate mofetil to improve oral bioavailability. More...
.
- Congenital. The term "hereditary pure red cell aplasia" has been used to refer to Diamond-Blackfan anemiaDiamond-Blackfan anemiaDiamond–Blackfan anemia , also known as Blackfan–Diamond anemia and Inherited erythroblastopenia, is a congenital erythroid aplasia that usually presents in infancy. DBA patients have low red blood cell counts . The rest of their blood cells are normal...
.
Treatment
PRCA is considered an autoimmune disease as it will respond to immunosuppressantImmunosuppressant
An immunosuppressant is any substance that performs immunosuppression of the immune system. They may be either exogenous, as immunosuppressive drugs, or endogenous, as ,e. g., testosterone...
treatment such as ciclosporin
Ciclosporin
Ciclosporin , cyclosporine , cyclosporin , or cyclosporin A is an immunosuppressant drug widely used in post-allogeneic organ transplant to reduce the activity of the immune system, and therefore the risk of organ rejection...
in many patients, though this approach is not without risk.
It has also been shown to respond to treatments with Rituxan.
Popular culture
In an episode of House, an athlete is diagnosed with pure red cell aplasia as a manifestation of a thymoma.See also
- Diamond-Blackfan anemiaDiamond-Blackfan anemiaDiamond–Blackfan anemia , also known as Blackfan–Diamond anemia and Inherited erythroblastopenia, is a congenital erythroid aplasia that usually presents in infancy. DBA patients have low red blood cell counts . The rest of their blood cells are normal...
(genetic red cell aplasia) - aplastic anemiaAplastic anemiaAplastic anemia is a condition where bone marrow does not produce sufficient new cells to replenish blood cells. The condition, per its name, involves both aplasia and anemia...
(aplasia affecting other bone marrow cells as well)