Symptoma

Red Cell Aplasia

Transient erythroblastopenia of childhood

Here, we describe a case of CVID-like humoral immune deficiency in a child who initially presented with red cell aplasia and ultimately developed progressive bone marrow failure. [ncbi.nlm.nih.gov]

Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. [bloodjournal.org]

  • Fatigue

    A sixty-one-year-old male was diagnosed with thymoma with PRCA after he complained fatigue, tinnitus, and weakness for 1 month, he received therapy with recombinant erythropoietin (rhEPO) for 1 month after the tumor was totally resected and readmitted [ncbi.nlm.nih.gov]

    Symptoms related to anemia include fatigue, lethargy, decreased exercise tolerance and pallor. CBC, reticulocyte count Bone marrow examination Pure RBC aplasia presents with a normocytic anemia but normal WBC and platelet counts. [msdmanuals.com]

    Symptoms result from anemia and include fatigue, lethargy, decreased exercise tolerance and pallor. Diagnosis requires demonstration of peripheral normocytic anemia and a normocellular bone marrow biopsy with absence of erythroid maturation. [merckmanuals.com]

    Snapshot An 11-month-old Caucasian boy presents to the emergency department for severe fatigue. His symptoms progressively worsened to the point where he is unresponsive. [medbullets.com]

    Patients with PRCA may present with fatigue, lethargy, and/or abnormal paleness of the skin. In some cases, PRCA was found to be reversible with dose reduction or cessation of CellCept therapy. [empr.com]

  • Suggestibility

    These findings suggest that monitoring of PVB DNA is recommended for patients undergoing HSCT and having contact with individuals with documented PVB infection, even if they are asymptomatic. [ncbi.nlm.nih.gov]

    It was suggested that they dispersed if the product was administered by IV, but remained if given by the SC route. [gabionline.net]

The characteristics of PRCA (see Workup ) include the following: Severe anemia Reticulocyte count The presence of less than 0.5% mature erythroblasts in the bone marrow Normocellular bone marrow in most cases The etiology of PRCA is heterogeneous. [emedicine.medscape.com]

  • Nephrolithiasis

    In this case, a 72-year-old woman underwent left nephrectomy for severe nephrolithiasis in 1990. From 2007 until 2014 kidney function slowly deteriorated, reaching stage IV–V CKD. [gabi-journal.net]

  • Erythroblast

    Pure red cell aplasia is characterized by normochromic normocytic anemia, reticulocytopenia, and absence of erythroblasts from a normal bone marrow. Only few lymphoproliferative disorders have been associated with erythroid aplasia. [ncbi.nlm.nih.gov]

    This is due to an interruption in the red blood cell development stage that stops the precursor cells (Erythroblasts) being formed. In Pure Red Cell Aplasia the red blood cells are reduced but the white blood cell and platelet production is normal. [drugs.com]

Compared with healthy control, T reg levels in patients with aPRCA reduced significantly before sirolimus but recovered after successful treatment. Level of T reg cells correlated with hemoglobin level after effective sirolimus treatment. [ncbi.nlm.nih.gov]

[…] an autoimmune disease as it will respond to immunosuppressant treatment such as cyclosporin in many patients, [9] though this approach is not without risk. [10] It has also been shown to respond to treatments with rituximab and tacrolimus. [ citation [en.wikipedia.org]

Hemogram and treatment sequence of patient 2. Arrow ( ) represents fludarabine cycle, and ( ) represents rituximab treatment. [bloodjournal.org]

Treatment Treatment consists of: Blood transfusions to overcome the anaemia. These may have to be repeated. Discontinuation of EPO because it has become ineffective. Also further exposure to EPO might allow the antibody response to persist. [rarerenal.org]

Increased EPO and Fer levels might be the negative factors for prognosis of acquired PRCA. [ncbi.nlm.nih.gov]

What is the Prognosis of Pure Red Cell Aplasia? (Outcomes/Resolutions) The prognosis of Pure Red Cell Aplasia varies depending on its severity. [dovemed.com]

Prognosis Prognosis varies among the different types of pure red cell aplasia (PRCA). Transient erythroblastopenia and other PRCA disorders in children and adults are benign with an excellent prognosis. [emedicine.medscape.com]

[…] and macrophages with abundant iron deposits is increased. dysplastic changes in at least 10% of cells from two or more hematopoietic lineages the number of myeloblasts in bone marrow is 5 – 19% rAEB-1: 5 – 9% myeloblasts rAEB-2: 10 – 19% myeloblasts prognosis [atlases.muni.cz]

Introduction Clinical definition isolated anemia and reticulocytopenia due to a pure red cell aplasia (PRCA) Etiology may be due to congenital ( e.g., Diamond-Blackfan anemia) or acquired ( e.g., transient erythroblastopenia, paraneoplastic syndromes, [medbullets.com]

Chronic, B-Cell/complications* Leukemia, Lymphocytic, Chronic, B-Cell/diagnosis Male Middle Aged Prednisolone/administration & dosage Prednisolone/therapeutic use* Red-Cell Aplasia, Pure/diagnosis Red-Cell Aplasia, Pure/drug therapy Red-Cell Aplasia, Pure/etiology [ncbi.nlm.nih.gov]

Anti-erythroid antibodies may be detected in some patients Also due to allogeneic bone marrow transplant from ABO incompatible donor (treat with aggressive plasmapheresis) May be due to antibodies to recombinant erythropoietin in those with chronic renal failure Etiology [pathologyoutlines.com]

They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]

[…] marrow with associated anemia and reticulocytopenia Normal numbers of megakaryocytes and white blood cell precursors Terminology Called either erythroblastopenia or red cell aplasia (RCA) Sustained isolated RCA is called pure red cell aplasia (PRCA) Epidemiology [pathologyoutlines.com]

Epidemiology Pure red cell aplasia is a very rare condition. One of its causes of congenital origin, called Diamond-Blackfan anemia, has a yearly incidence in the general European population of 1 case in every 150,000 individuals. [xpertdox.com]

Anemia in HIV infected adults: epidemiology, pathogenesis and clinical management. Curr Hematol Rep 4, 95 –102. [Google Scholar] Forna, F., Liechty, C. C., Solberg, P., Asiimwe, F., Were, W., Mermin, J., Behumbiize, P., Tong, T., Brooks, J. [jmm.microbiologyresearch.org]

Department of Epidemiology, Merck Research Laboratories, 351 N. Sumneytown Pike, North Wales, PA 19454, USA. 2015 Quinlan et al; licensee Herbert Publications Ltd. [hoajonline.com]

Epidemiology Pure red cell aplasia (PRCA) is an uncommon disorder. The idiopathic form is the most common type of PRCA. [emedicine.medscape.com]

Whether responsiveness to individual therapeutic regimens necessarily correlates with the pathophysiology of disease is unclear. [annals.org]

[…] is involved, the target of the autoantibody is variable and may be at the erythroid precursor level. 4 Myelodysplastic primary acquired PRCA is an uncommon presentation of myelodysplasia morphologically characterized by erythroid hypoplasia, but is pathophysiologically [bloodjournal.org]

The pathophysiology of pure red cell aplasia: implications for therapy. Blood 87:4831–48381996. 2. Fisch P, Handgretinger R, Schaefer HE. Pure red cell aplasia. Br J Haematol 111:1010–10222000. 3. [kjim.org]

Pathophysiology In general, pure red cell aplasia (PRCA) is due to a selective injury, often immunological, that affects the early phase of erythrocyte maturation. [emedicine.medscape.com]

[…] the underlying cause(s) Avoiding medications and drugs that could potentially lead to PRCA could help prevent the disease from developing In many cases, Pure Red Cell Aplasia is not a preventable condition. [dovemed.com]

Prevention of pure red cell aplasia after major or bidirectional ABO blood group incompatible hematopoietic stem cell transplantation by pretransplant reduction of host anti-donor isoagglutinins. [archive-ouverte.unige.ch]

[…] infection can be prevented by prophylactic administration of acyclovir. [omicsonline.org]

Prevention There is some evidence ( Macdougall et al, 2012 ) that most cases of PRCA are related to poor handling and storage of the drug EPO. [rarerenal.org]

Patients diagnosed with human immunodeficiency virus infection were excluded from the cohort due to privacy issues that would have prevented validation of any PRCA cases identified among these patients. [hoajonline.com]