The condition has been first described by paul kaznelson in 1922. Pure red cell aplasia an overview sciencedirect topics. Pure red cell aplasia prca, also known as erythroblastopenia, is characterized by a suppression of erythrocytes in the bone marrow. These results suggest indeed that durable maintenancefree remission of. Cellmediated suppression of erythropoiesis as a possible pathogenesis of pure red cell aplasia. Pure red cell aplasia prca is an uncommon disorder in which maturation arrest occurs in the formation of erythrocytes. Lipopolysaccharideresponsive beigelike anchor lrba protein deficiency is a recently described immunodeficiency syndrome that has been associated with inflammatory bowel disease and. The acute pure red cell aplasia is a transient disorder and is seen primarily in childhood but can occur at all ages. Rational management approach to pure red cell aplasia. This signs and symptoms information for pure red cell aplasia has been gathered from various sources, may not be fully accurate, and may not be the full list of pure red cell aplasia signs or pure red cell aplasia symptoms. Acquired pure red cell aplasia prca is a rare condition of profound anemia characterized by a very low reticulocyte count and the virtual absence of erythroid precursors in the bone marrow. Find details on pure red cell aplasia in dogs including diagnosis and symptoms, pathogenesis, prevention, treatment, prognosis and more. Pdf pure red cell aplasia prca is an uncommon disorder in which maturation arrest occurs in the formation of erythrocytes.
Pure red cell aplasia prca is a rare bone marrow failure syndrome defined by a. The onset of pure red blood cell anemia usually is insidious, often occurring over weeks or months. Pure red cell aplasia prca is a rare disorder of erythropoiesis characterized. Pure red cell aplasia prca is a rare bone marrow disorder characterized by. Pure red cell aplasia pcra is a rare disorder of erythropoiesis characterized by a selective and severe decrease in erythrocyte precursors in an otherwise normal bone marrow. Acquired pure red blood cell aplasia is a disorder of erythroid precursors that results in an isolated normocytic anemia. Acquired pure red cell aplasia due to antierythropoietin antibodies in a patient with end stage chronic kidney disease. Pure red cell aplasia following autoimmune hemolytic. Pure red cell aplasia prca is a rare hematological condition and a reason for severe anemia under immunosuppression. A case of pure red cell aplasia linkedin slideshare. Treatment of primary, idiopathic prca is immunosuppressive therapy. This is a very rare condition and usually affects adults. Pure red cell aplasia fisch 2000 british journal of haematology.
Here, we report a case of a 74yearold female who presented with severe proximal weakness without pain and was found to have pwca with. Acquired pure red cell aplasia is characterized by a decrease in the number of red blood cells produced in the bone marrow. Pure red cell aplasia and lymphoma jama jama network. Pure red blood cell aplasia, acquired, usually presents as a severe condition. Acquired prca may be either a primary disorder or secondary to some other disorder or agent. Pure red cell aplasia prca is a rare condition that affects the bone marrow. Pure white cell aplasia pwca is a rare hematologic disorder characterized by the absence of neutrophil lineages in the bone marrow with intact megakaryopoiesis and erythropoiesis. Daclizumab for pure redcell aplasia annals of internal. Pure red cell aplasia prca is a rare syndrome that only affects the erythroid lineage. A 72yearold caucasian male, who was started on isoniazid after the. The pathology of pure red cell aplasia rho chi post. Pure red cell aplasia prca is a hematologic syndrome characterized by severe normochromic, normocytic anemia associated with reticulocytopenia and.
Anemia is highly prevalent in patients after kidney transplantation. Pure red cell aplasia prca is characterized by a severe normocytic, normochromic, nonregenerative anemia along with erythroid hypoplasia and increased lymphocyte numbers in the bone marrow. Bone marrow contains stem cells which develop into the red blood cells that carry oxygen through the body, the white blood cells that fight infections, and the platelets that help with blood clotting. It is associated with many disorders, including parvovirus b19 infection, lymphoproliferative disorders rheumatologic diseases, thymoma, and rarely erythropoietin. Shortly after discontinuation of the procainamide preparation, a reticulocytosis and increasing hemoglobin level were observed. Pure red cell aplasia prca is a rare disease characterized by a severe normocytic anemia, reticulocytopenia and absence of erythroblasts from the bone marrow. Pure red cell aplasia prca is an uncommon cause of anaemia and was originally described in 1922. Levels of the hormone erythropoietin that stimulates the bone marrow to produce red blood cells are usually elevated. Pure red cell aplasia and lymphoproliferative disorders. Three patients with severe, deforming, and longstanding rheumatoid arthritis developed pure red cell aplasia that did not remit after withdrawal of medications, ran a chronic course, and in two patients remitted only after cytotoxic immunosuppressive treatment. Pure red cell aplasia hematology american society of. In prca, the bone marrow ceases to produce red blood cells.
It is known to cause hepatotoxicity, but in rare instances, it can lead to prca. Individuals with this disorder are deficient in the number of precursors of red blood cells erythroblasts. Tohda s, nara n, tanikawa s, imai y, murakami n, aoki n. Pure red cell aplasia prca red blood cell bone marrow. Pure red cell aplasia developed in a patient with smallcell lung cancer who was also taking sustainedrelease procainamide.
All other cell lines are present and seem quantitatively and morphologically normal. Pure red cell aplasia is caused by a selective destruction or inhibition of erythroid progenitor or precursor cells. Histopathology images of pure red cell aplasia pra by. Additional and relevant useful information for pure red cell aplasia. Pure red cell aplasia prca is defined as an acquired anemia secondary to absence or nearabsence of erythroid precursors in the bone marrow. Pdf pure red cell aplasia and cyclosporine fernando. Diamondblackfan anemia is a congenital form of prca.
It is defined by a normocytic, normochromic anemia with a marked reticulocytopenia and severe reduction or. All other cell lineages are present in normal numbers and appear morphologically normal. There are five treatment regimens that have established efficacy for patients with chronic prca. Pure red cell aplasia after kidney transplantation. Acquired prca, which is distinguished from congenital forms of prca, is associated with autoimmune, infections, solid tumors, lymphoproliferative disorders, autoimmune vascular diseases, or specific medications. Hemal ms senanayake, kanapathipillai thirumavalavan. Pure red blood cell aplasia hematology and oncology. The condition may be congenital but is usually acquired and associated with haematological disorders including leukaemia. Pure red cell aplasia prca, a disorder first described in 1922 kaznelson, 1922, can be characterized as an anaemia with the almost complete absence of red cell.
Learn vocabulary, terms, and more with flashcards, games, and other study tools. Symptoms of pure rbc aplasia are generally mild and relate to the degree of the anemia or to the underlying disorder. Pure red cell aplasia prca is a hematologic syndrome characterized by severe normochromic, normocytic anemia associated with reticulocytopenia and absence of erythroblasts from an otherwise normal marrow. Pure red cell aplasia following autoimmune haemolytic anaemia. Pure red cell aplasia in dogs vetlexicon canis from. The disease also may be associated with a variety of other conditions, including paraproteinemia. In people with prca, the bone marrow makes a reduced number of red blood cells called anemia. Pure red cell aplasia blood american society of hematology. Detection of pvb19 in serum or bone marrow in the absence of. Free heme is toxic to cells, and it is hypothesized that the binding of felvc to. From 1980 through 1994, we identified 47 adult patients with acquired pure red cell aplasia median age, 64 years. Rheumatoid arthritis and pure red cell aplasia annals of. Chapter 32 pure red cell aplasia free medical textbook.
Isoniazid is a widely used drug for the treatment of tuberculosis all over the world. Acquired pure red cell aplasia and acquired amegakaryocytic thrombocytopenic purpura are rare in children. In patients with congenital hypoplastic anemia the best results have been reported using corticosteroids. Pure red cell aplasia prca is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Pure red cell aplasia prca, initially described by kaznelson in 1922, is a rare disorder, characterized by the presence of a severe normochromic, most frequently normocytic anaemia and reticulocytopenia free. Symptoms result from anemia and include fatigue, lethargy, decreased exercise tolerance, and pallor. Pure red cell aplasia prca free download as powerpoint presentation. Pdf antierythropoietin antibodies and pure red cell. Cureus isoniazid induced pure red blood cell aplasia. Approach to normocytic anemia pure red cell aplasia check reticulocyte count normocytic anemia increased is there evidence of hemolysis. Pure red cell aplasia prca can be inherited diamond blackfan anemia, dba or acquired aprca. Pure red cell aplasia is morphologically characterized by severe lack of erythroid precursors in the bone marrow whereas myeloid precursors and megakaryocytic elements are unaffected and are present in normal numbers. Acquired pure redcell aplasia prca is characterized by isolated severe anemia and reticulocytopenia and by absent or markedly diminished erythroid precursors in the bone marrow, which is otherwise normal. Differential diagnosis of pure red cell aplasia should include parvovirus b19 pvb19 infection, especially in immunologically incompetent hosts, like patients with nonsuppressed hiv.
It is characterized by an anemia and reticulocytopenia and occurs as an acute or chronic condition. It is a peculiar oddity that the bone marrows progenitor cells still differentiate into white blood cells and platelets. Pure red cell aplasia prca is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of. This is a rare syndrome 129 thought to be caused by immunemediated response against. We have found only one report of prca in the course of histiocytic lymphoma. Pure red blood cell aplasia prca is one of the uncommon causes of anemia. Pure red cell aplasia prca is a syndrome characterized by a.
The amounts of white blood cells and platelet remain normal transient or acute selflimited prca. Identification of mutations in patients with acquired pure. Cook md, phd, in hematopathology third edition, 2018. Although it is rare, isolated cytogenetic abnormalities can be seen in prca, and. It is defined by a normocytic, normochromic anemia with a marked reticulocytopenia and severe reduction or absence of erythroid precursors in the bone marrow. However, if it is properly managed from the early stages, it can be maintained and prevented from developing into its chronic form. The os and diseasefree probability at 510 years in our cohort were 0. It is characterized by an absence of red cell precursors reticulocytes in the marrow and a low red blood cell count. The association of pure red cell aplasia prca with thymoma 1 is well known. Frequent causes of anemia in transplant recipients range from simple hemorrhage to hematotoxicity of drugs, inflammatory conditions, and infectious diseases. Pure redcell aplasia and recombinant erythropoietin nejm.