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]

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]

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]

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]

• Cold Intolerance

  No h/o cold intolerance /constipation /voice change ; No h/o polyuria/polydipsia /polyphagia; No h/o loose stools /vomiting/altered bowel habits; No h/o yellowish discoloration of urine ; No h/o abdominal distension/swelling of legs; No h/o weakness of [slideshare.net]

• Buffalo Hump

  Long-term use of high-dose prednisone results in significant toxicity, including some combination of growth retardation, cushingoid facies, buffalo hump, osteoporosis, aseptic necrosis of the hip and fractures, diabetes, hypertension, and cataracts. [patient-help.com]

• Cushingoid Facies

  Long-term use of high-dose prednisone results in significant toxicity, including some combination of growth retardation, cushingoid facies, buffalo hump, osteoporosis, aseptic necrosis of the hip and fractures, diabetes, hypertension, and cataracts. [patient-help.com]

• Cushingoid

  Long-term use of high-dose prednisone results in significant toxicity, including some combination of growth retardation, cushingoid facies, buffalo hump, osteoporosis, aseptic necrosis of the hip and fractures, diabetes, hypertension, and cataracts. [patient-help.com]

• Intermittent Fever

  Report of Case A 7 year Hindu female first child, born of a non-consanguineous marriage, presented with increasing pallor noticed for 6-7 days and intermittent fever with chills and rigor for 4 days. [ijmstrust.in]

• Orthopnea

  […] aplasia. [15] [16] [17] [18] [19] Acutely presenting in children 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 [explainmedicine.com]

  Velu; 42/m; Korukkupet- Manual labourer 3. c/o :- breathlessness on exertion 6months cough HOPI :- patient was apparently normal 6 months before ; h/o breathlessness present; grade 1; no h/o PND/orthopnea/chest pain /palpitation; h/o dry cough ; intermittent [slideshare.net]

• Dry Cough

  Velu; 42/m; Korukkupet- Manual labourer 3. c/o :- breathlessness on exertion 6months cough HOPI :- patient was apparently normal 6 months before ; h/o breathlessness present; grade 1; no h/o PND/orthopnea/chest pain /palpitation; h/o dry cough ; intermittent [slideshare.net]

• Pulsatile Tinnitus

  Symptoms of Acquired Pure Red Cell Aplasia Symptomatic anemia in the older patient may manifest as pallor, fatigue, lassitude, pulsatile tinnitus, and anginal chest pain. [patient-help.com]

• Sternal Tenderness

  General examination : conscious oriented afebrile pallor + no icterus/ cyanosis clubbing pedal edema lymphadenopathy sternal tenderness 7. [slideshare.net]

• Suggestibility

  Family history was also not suggestive of any bleeding disorder. [ijmstrust.in]

  Access full-color images and more than 90 online-only topics at Expert Consult, as well as EBMs, Suggested Reading, Patient Teaching Guides, and additional algorithms. [books.google.com]

  Our case and few other reports suggest that rituximab is a viable alternative in treating PRCA due to the neutralizing anti-epoetin antibody. [indianjnephrol.org]

  In conclusion, we suggest that PRCA should be added to the list of immunohaematological associations of chronic GVHD. References 1 Dessypris EN. Pure red cell aplasia. In Hoffman R, Bonn EJ, Senthil SJ (eds) Hematology. [nature.com]

  Blood film showed absolute lymphocytosis many small mature lymphocytes with many smudge cells are seen suggestive of chronic lymphocytic leukemia (CLL). [ijhonline.org]

• Impulsivity

  Vitals: PR- 88/mt; felt in all peripheral pulses equally; BP- 110/80 mmHg; System examination : CVS: ApicaI impulse – normal in position; S 1 S 2 heard ; haemic murmur heard in all areas ; no thrill ; 8. [slideshare.net]

• Involuntary Movements

  RS:- trachea in midline; normal vesicular breath sounds heard in all areas;no added sounds ; ABDOMEN: soft, no organomegaly; no free fluid; CNS: higher functions/motor,sensory,cranial nerves- normal; no involuntary movements; spine cranium normal 9. [slideshare.net]

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]

• Mediastinal Mass

  Final diagnosis MEDIASTINAL MASS s/o THYMOMA ANEMIA PURE RED CELL APLASIA 24. [slideshare.net]

  Figure 1: Chest X-ray with deviation of the mediastinum to the right suggesting left superior mediastinal mass Click here to view Figure 2: Whole body positron imaging tomography-computed tomography reveals splenomegaly with multiple enlarged faintly [ijhonline.org]

• Staphylococcus Aureus

  Saturating amounts of formalin-fixed Staphylococcus aureus were added, and the tubes were incubated for another 20 minutes while being stirred continuously. [nejm.org]

• Lymphocytic Infiltrate

  Anemia due to PRCA in CLL patients must be carefully evaluated and differentiated from other causes (autoimmune hemolytic anemia, neoplastic lymphocyte infiltration of bone marrow, chemotherapy) that require a different therapeutic approach. [moh-it.pure.elsevier.com]

  A lymphocyte infiltrate was also found, consisting of small, mature lymphocytes without villi (Figure 2A ) and with paratrabecular, pericapillary and intrasinusoidal distribution in the marrow. [casesjournal.biomedcentral.com]

  The rash was characterized pathologically by focal spongiosis and dermal lymphocytic infiltration, and it cleared completely and did not recur after a 3-week course of oral steroid treatment. [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]

Antibody-mediated pure red cell aplasia (PRCA) treatment and re-treatment: multiple options. Nephrol Dial Transplant. 2005 May;20 Suppl 4:iv23–26. 6.McKoy JM, Stonecash RE, Cournoyer D, et al. [nzma.org.nz]

[…] 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]

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]

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]

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]

There are multiple etiologies that can cause PRCA. [en.wikipedia.org]

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]

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]

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 pure red cell aplasia: implications for therapy. Blood 1996; 87 : 4831–4838. 9 Dincol, G, Aktan M, Nalcaci M et al. Clonality of acquired primary pure red cell aplasia: effectiveness of antithymocyte globulin. [nature.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]

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]