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Pure red cell aplasia (PRCA) or erythroblastopenia refers to a type of
aplastic anemia Aplastic anemia is a cancer in which the body fails to make blood cells in sufficient numbers. Blood cells are produced in the bone marrow by stem cells that reside there. Aplastic anemia causes a deficiency of all blood cell types: red bloo ...
affecting the precursors to
red blood cells Red blood cells (RBCs), also referred to as red cells, red blood corpuscles (in humans or other animals not having nucleus in red blood cells), haematids, erythroid cells or erythrocytes (from Greek ''erythros'' for "red" and ''kytos'' for "hol ...
but usually not to
white blood cells White blood cells, also called leukocytes or leucocytes, are the cells of the immune system that are involved in protecting the body against both infectious disease and foreign invaders. All white blood cells are produced and derived from mult ...
. In PRCA, the
bone marrow Bone marrow is a semi-solid biological tissue, tissue found within the Spongy bone, spongy (also known as cancellous) portions of bones. In birds and mammals, bone marrow is the primary site of new blood cell production (or haematopoiesis). It i ...
ceases to produce red blood cells. There are multiple etiologies that can cause PRCA. The condition has been first described by
Paul Kaznelson Paul Kaznelson (1898–1959) was a Polish-born Czech scientist 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 thromb ...
in 1922.


Signs and symptoms

Signs and symptoms may include: * Pale appearance * Rapid heart rate * Fatigue


Causes

Causes of PRCA include:


Treatment

PRCA is considered an
autoimmune In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an "autoimmune disease". ...
disease as it will respond to
immunosuppressant Immunosuppressive drugs, also known as immunosuppressive agents, immunosuppressants and antirejection medications, are drugs that inhibit or prevent activity of the immune system. Classification Immunosuppressive drugs can be classified in ...
treatment such as
cyclosporin Ciclosporin, also spelled cyclosporine and cyclosporin, is a calcineurin inhibitor, used as an immunosuppressant medication. It is a natural product. It is taken orally or intravenously for rheumatoid arthritis, psoriasis, Crohn's disea ...
in many patients, though this approach is not without risk. It has also been shown to respond to treatments with rituximab and
tacrolimus Tacrolimus, sold under the brand name Prograf among others, is an immunosuppressive drug. After allogeneic organ transplant, the risk of organ rejection is moderate. To lower the risk of organ rejection, tacrolimus is given. The drug can also ...
.


See also

* Diamond–Blackfan anemia (genetic red cell aplasia) *
Aplastic anemia Aplastic anemia is a cancer in which the body fails to make blood cells in sufficient numbers. Blood cells are produced in the bone marrow by stem cells that reside there. Aplastic anemia causes a deficiency of all blood cell types: red bloo ...
(aplasia affecting other bone marrow cells as well)


References


External links

{{DEFAULTSORT:Pure Red Cell Aplasia Aplastic anemias