scholarly journals Etiologies and Treatment Burden in Adult Patients with Pure Red Cell Aplasia: A Single-Center Experience and Review of Literature

Anemia ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-5 ◽  
Author(s):  
Pimjai Niparuck ◽  
Wasana Kanoksil ◽  
Pathawut Wacharapornin ◽  
Pichika Chantrathammachart ◽  
Sarinya Boongird

Background. Pure red cell aplasia (PRCA) is less common blood disorder; the causes and the treatments of PRCA are varied. Methods. We conducted a retrospective study during January 2010–December 2017, to explore the etiologies and to evaluate the response and treatment burden in adult patients with PRCA. Results. Of 32 PRCA patients, median age was 57 years (18–90 years). Median hemoglobin level and reticulocyte count at the time of diagnosis were 5.6 g/dL (3.3–7.3 g/dL) and 0.3% (0.1–0.7%), respectively. Median time to hematologic recovery was 12 weeks (3–72 weeks), and median number of red blood cell transfusion (RBC) was 20 units (4–100 units). Causes of PRCA were erythropoiesis-stimulating agent (ESA) (47%), parvovirus B19 infection (19%), thymoma (13%), zidovudine (6%), primary autoimmune PRCA (6%), Kaposi’s sarcoma (3%), systemic lupus erythematosus (3%), and ABO-mismatched stem cell transplantation (3%). Only 9 out of 24 treated patients achieved hematologic response within 8 weeks of treatment. Intravenous immunoglobulin therapy provided 100% response rate in patients with parvovirus B19-associated PRCA and primary autoimmune PRCA. Low response rate was found in patients receiving immunosuppressants and chemotherapy for the treatment of ESA and thymoma-associated PRCA, respectively. Conclusions. Treatment outcome of PRCA depended upon the causes and the types of treatment, and the burden of RBC transfusion was very high in patients with ESA and thymoma-associated PRCA.

Blood ◽  
2000 ◽  
Vol 96 (3) ◽  
pp. 1184-1186 ◽  
Author(s):  
Vivek R. Sharma ◽  
Donald R. Fleming ◽  
Stephen P. Slone

Abstract Rituximab is a chimeric monoclonal antibody directed against CD20 and used in the treatment of B-cell non-Hodgkin's lymphoma. Due to its ability to deplete B lymphocytes, rituximab can interfere with humoral immunity, causing it to be suppressed for several months after treatment. The reported case depicts a serious consequence of this effect of rituximab therapy: pure red cell aplasia resulting from chronic parvovirus B19 infection. The point of interest in this case is not only the association between rituximab therapy and pure red cell aplasia, but the diagnostic and therapeutic utility of the knowledge of parvovirus B19 as the likely etiologic link between the two. Given the known efficacy of intravenous immunoglobulin (IVIg) in the treatment of chronic parvovirus B19 infection, this therapy can cure some of these patients and successfully render most others transfusion-independent until recovery of their own humoral immune system.


2017 ◽  
Vol 2 (2) ◽  
pp. S19-S20
Author(s):  
Putun Patel ◽  
Vibha Bafna ◽  
Sandip Bartakke ◽  
Priya Gupta ◽  
Sanjay Mankar ◽  
...  

2012 ◽  
Vol 52 (186) ◽  
Author(s):  
A Baral ◽  
B Poudel ◽  
R K Agrawal ◽  
R Hada ◽  
S Gurung

Parvo B19 is a single stranded DNA virus, which typically has affi nity for erythroid progenitor cells in the bone marrow and produces a severe form of anemia known as pure red cell aplasia. This condition is particularly worse in immunocompromised individuals. We herein report a young Nepali male who developed severe and persistent anaemia after kidney transplantation while being on immunosuppressive therapy. His bone marrow examination revealed morphological changes of pure red cell aplasia, caused by parvovirus B19. The IgM antibody against the virus was positive and the virus was detected by polymerase chain reaction in the blood. He was managed with intravenous immunoglobulin. He responded well to the treatment and has normal hemoglobin levels three months post treatment. To the best of our knowledge, this is the fi rst such case report from Nepal. Keywords: Intravenous immunoglobulin, kidney transplant recipient, Parvovirus B19, pure red cell aplasia.


2020 ◽  
Vol 52 (8) ◽  
pp. 2539-2543 ◽  
Author(s):  
Ewa Nowacka-Cieciura ◽  
Ewa Karakulska-Prystupiuk ◽  
Anna Żuk-Wasek ◽  
Wojciech Lisik ◽  
Grzegorz Władysław Basak ◽  
...  

2010 ◽  
Vol 90 (8) ◽  
pp. 975-978 ◽  
Author(s):  
Ja-Young Seo ◽  
Hee-Jin Kim ◽  
Sun-Hee Kim

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4818-4818 ◽  
Author(s):  
Xuemei Wu ◽  
Suli Wang ◽  
Wenyi Shen ◽  
Yu Zhu ◽  
Rui-Nan Lu ◽  
...  

Abstract Objective: Adult pure red cell aplasia (PRCA) is a syndrome characterized by a severe normocytic anemia, reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow. Immunosuppressive therapy has been used as the initial treatment for acquired chronic PRCA. This article aims to evaluate the efficacy of cyclosporine A, and/or corticosteroids, and possible factors influencing it. Methods: 34 cases of PRCA were retrospectively analyzed at our institution.Clinical data of 23 inpatient cases and 11 outpatient cases from 2009 October to 2016 March were collected. These patients were treated by cyclosporine A (CsA), and/or corticosteroids (CS). Results: The causes of PRCA were as follows (table 1): idiopathic (15 cases, 44.1%), thymoma-associated (5 cases, 14.7%), and large granular lymphocyte (LGL) leukaemia-associated (7 cases, 20.6%), virus infection (4 cases, 11.7%), major ABO-mismatched allogeneic haematopoietic stem cell transplantation (1 case, 2.94%), rheumatoid arthritis (1 case, 2.94%), antierythropoietin antibody-mediated (1 case, 2.94%). One patient was lost to follow-up. Two patients were not evaluable for response due to short observation period (<3 months). In idiopathic PCRA, complete remission (CR) was achieved in 4 of 14 cases (28.6%), and partial remission (PR) was achieved in 7 patients (50.0%), while 1 case (7.1%) was still transfusion dependent and 2 deaths(14.3%). In secondary PRCA, complete remission (CR) was achieved in 6 of 17 cases (35.3%, 3 LGL, 2 thymoma, 1 others), and partial remission (PR) was achieved in 3 patients (17.6%, 1 LGL, 2 others ), while 7 case (41.2%, 3 LGL, 2 thymoma, 2 others)) were still transfusion dependent and 1 death(5.9%, thymoma). It showed that the initial rate of CR in CsA-containing group was higher than CS alone (33.3% vs 0%, P=0.028). In 22 refractory and relapsed PRCA patients, 6 out of 10 (60%) refractory patients and 5 out of 12 (41.7%) relapsed patients achieved remission(table 2). The cumulative rate of CR of CsA alone, CS alone, CS+CsA and other immunosuppressive agents were 27.8%, 6.67%, 66.7%, 10%. The cumulative rate of CR of the combination of CS and CsA was higher than CS alone or CsA alone (66.7% vs 18.2%, P=0.028). The response rate of CS or CsA was higher than other immunosuppressive agents (69.2% vs 20%, P<0.01). The age, gender, the percent of reticulocyte and absolute number of lymphocyte in peripheral blood, and etiology did not influence the response rate. (table 3) Conclusion: Thymoma and LGL are the two common causes of secondary PRCA. CsA and/or CS are effective in treating PRCA.The combination of CS and CsA was more effective in achieving CR than CS alone or CsA alone. It was suggested that we might choose the therapy which contains CsA as the initial induction therapy. Other immunosuppressive agents (tacrolimus or mycophenolate mofetil) was less effective in refractory and relapsed PRCA patients. And it was still needed to explore a more effective therapy for refractory and relapsed PRCA patients. Disclosures No relevant conflicts of interest to declare.


2019 ◽  
Vol 21 (6) ◽  
Author(s):  
Neeraj Inamdar ◽  
Ritambhra Nada ◽  
Ranjana Minz ◽  
Deepesh Benjamin Kenwar ◽  
Sarabpreet Singh ◽  
...  

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