Presentation
Acute acquired form is predominantly present in children. [1] Chronically presenting in adults Chronic form is predominantly present in adults. [1] Features of acute heart failure i.e. orthopnea, paroxysmal nocturnal dyspnea, exertional intolerance. [explainmedicine.com]
We present this report in accordance with the CARE checklist for case reports 2016. Case presentation A 35-year-old Nepalese male presented to his primary care physician with lethargy and abdominal discomfort in June 2013. [esmoopen.bmj.com]
In parvovirus B19 infection, giant pronormoblasts may be present. Chest CT is indicated to evaluate for thymoma in the absence of another identified cause. [merckmanuals.com]
[…] and diagnosis Patients present with severe normochromic, normocytic to slightly macrocytic anemia and marked reticulocytopenia Occult parvovirus B19 infection must be excluded by PCR testing in all cases of secondary PRCA Evaluation for any underlying [pathologyoutlines.com]
Clinical course and treatment The anemia of TEC typically resolves spontaneously 1-2 months from presentation, and steroids have not been shown to alter the course of the disease. [pedclerk.bsd.uchicago.edu]
Cardiovascular
- Vascular Disease
In our patient, we ruled out other causes for PRCA such as collagen vascular disease, viral infections and other malignancies with appropriate radiological and laboratory tests. [esmoopen.bmj.com]
Acquired PRCA, rare in children, may be primary or secondary to conditions like Parvovirus B19 infection, thymoma, myelodysplatic diseases, drugs (including alpha-interferons, diphenylhydantoin, isoniazid, lamivudine), collagen vascular diseases and infections [jpgmonline.com]
Acquired Chronic (Sustained) Thymoma Chronic Lymphocytic Leukemia (B-Cell Type) Autoimmune disorders such as rheumatoid arthritis, systemic lupus erythematosus, autoimmune haemolytic anemia, chronic active hepatitis, collagen-vascular diseases, and chronic [ijmstrust.in]
The list includes thymoma, collagen vascular diseases, lymphoproliferative disorders, solid tumors, infections, severe renal failure, medications, pregnancy, severe nutritional deficiencies and other, rarer causes [ 1 ]-[ 5 ]. [casesjournal.biomedcentral.com]
It may be present as a primary hematologic disorder or secondary to various diseases like infection including parvovirus and hepatitis virus, collagen vascular disease, leukemia, lymphoma, thymoma and solid tumors. 13 A bone marrow aspirate is usually [e-cmh.org]
Skin
- Eruptions
Monoclonal antibody treatment was associated with little toxicity except for a cutaneous eruption that occurred 3 months after the last of 5 treatments in 2 patients. [annals.org]
In these patients, immunoglobulin infusions can induce fifth disease symptoms of variable severity, including cutaneous eruptions and arthritis. [patient-help.com]
Workup
A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. [e-cmh.org]
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]
Staging workup revealed Binet stage C/Rai stage IV. He was started on chemotherapy with the bendamustine + rituximab (BR) regimen [Figure 3] together with uricosuric drugs and hydration and received his first cycle on November 23, 2015. [ijhonline.org]
Serum
- Normocytic Anemia
Acquired pure red blood cell aplasia is a disorder of erythroid precursors that results in an isolated normocytic anemia. White blood cells and platelets are not affected. [merckmanuals.com]
Clinical signs: Normocytic normochromoc anemias. Histology: Peripheral blood: normochromic normocytic anemia. [atlases.muni.cz]
Case An otherwise well 18-month old male is noted on a screening CBC to have a normocytic anemia with Hb 7 g/dL and MCV 85, normal WBC and platelets. [pedclerk.bsd.uchicago.edu]
Keywords Thymoma Lymphoproliferative Disorder Antithymocyte Globulin Autoimmune Hemolytic Anemia Splenic Marginal Zone Lymphoma Introduction Acquired pure red cell aplasia (PRCA) is characterized by normochromic normocytic anemia, reticulocytopenia and [casesjournal.biomedcentral.com]
- Normocytic Normochromic Anemia
normochromic anemia, low hematocrit, reticulocytopenia and selective erythroid hypoplasia. [icd10data.com]
Laboratory Profound normocytic, normochromic anemia with reticulocytopenia and without polychromasia Other blood parameters generally unremarkable Treatment Steroids and immunosuppressants, antilymphocyte globulin, plasmapheresis, IV immunoglobulins [pathologyoutlines.com]
Clinical signs: Normocytic normochromoc anemias. Histology: Peripheral blood: normochromic normocytic anemia. [atlases.muni.cz]
A slow progressive normocytic-normochromic anemia and reticulocytopenia, without leukopenia and thrombocytopenia in a patient who, except pallor, does not generally show abnormal findings on physical examination, should arouse the suspicion of PRCA [ [ijmstrust.in]
Treatment
[…] an S 2018 5 Successful treatment of refractory/relapsed acquired pure red cell aplasia with sirolimus. ( 29982851 ) Long Z...Han B 2018 6 Successful high-dose dexamethasone treatment of acquired pure red cell aplasia following ABO major mismatched allogeneic [malacards.org]
Treatment The treatment goal is to restore red blood cell production and to treat any underlying disorder. The most common treatments are: Blood Transfusions to increase red blood cell levels. [aamds.org]
Rituximab: A viable treatment option for epoetin-induced pure red cell aplasia. [indianjnephrol.org]
Monoclonal antibody treatment was associated with little toxicity except for a cutaneous eruption that occurred 3 months after the last of 5 treatments in 2 patients. [annals.org]
[…] 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]
Prognosis
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]
Other treatment options: cyclophosphamide/ azathioprine cyclosporine ATG rituximab plasmapheresis splenectomy maintenance blood transfusions Prognosis : spontaneous remission – 5 to 10 % ; with immunosuppressives – 68 %; relapse within 2 years – 80%; [slideshare.net]
With the current use of immunosuppressive drugs and improved supportive care, the prognosis is no longer so grim. [medtextfree.wordpress.com]
Prognosis for Aplastic Anemia If stem cell transplantation is done early in the treatment cycle, prognosis is good and the risk of recurrence is relatively low. [lecturio.com]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Red cell aplasia as an acute and life-threatening complication of sickle cell disease and other hemolytic anemias was recognized in the 1940s, presaging the role of a specific virus in the etiology of both acute and chronic erythropoietic failure. [accessmedicine.mhmedical.com]
They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]
Etiology and Pathogenesis Clinical Features Laboratory Features Differential Diagnosis Therapy, Course, and Prognosis Chronic Pure Red Cell Aplasia—Acquired Definition and History Etiology and Pathogenesis Clinical Features Laboratory Features Differential [medtextfree.wordpress.com]
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]
Epidemiology
[…] 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]
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 Although it has been diagnosed in children as young as 1 month old, it usually presents in children 6 months – 6 years old, particularly between the ages of 1 and 4 years. It is more common in males than in females. [pedclerk.bsd.uchicago.edu]
Epidemiology Pure red cell aplasia (PRCA) is an uncommon disorder. The idiopathic form is the most common type of PRCA. [emedicine.medscape.com]
Pathophysiology
Whether responsiveness to individual therapeutic regimens necessarily correlates with the pathophysiology of disease is unclear. [annals.org]
Halperin SD, Freedman HM: Diamond-Blackfan anemia: Etiology, pathophysiology, and treatment. Am J Pediatr Hematol Oncol 11:380, 1989. [PMID: 2694854] 8. [patient-help.com]
Young NS (1991) The pathogenesis and pathophysiology of aplastic anemia. In: Hoffman R, Benz JR, Shattil SJ, Furie B, Cohen HJ (eds) Hematology. Basic principles and practice. Churchill Livingstone, New York, pp 122–160 Google Scholar 18. [link.springer.com]
The pathophysiology of pure red cell aplasia: implications for therapy. Blood. 1996 Jun 1; 87(11):4831-8. Contributed by J Manuel Zarandona, MD, and Sandra S Kaplan, MD [path.upmc.edu]
The pathophysiology of cytopenias includes cytokine effects and direct antigen-specific cytotoxicity. Most patients present with neutropenia. PRCA and pancytopenia are less common. [clinicalgate.com]
Prevention
[…] 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]
Since, this drug induced PRCA is reversible if recognised early, it is worthwhile to be aware of this complication to prevent permanent morbidity due to generalised bone marrow hypoplasia. [Malipatil R A et al. [scopemed.org]
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]
Amongst the various modalities tried, cyclosporin A has been the most successful drug. [9] For patients on cyclosporine, monitoring is essential to prevent complications, especially, renal failure. We are grateful to Dr. A. R. [jpgmonline.com]
The goal of preventing symptoms of anemia is achievable in most patients if the nadir hemoglobin is greater than 7 g/dL (70 g/L). [patient-help.com]