scholarly journals The pathophysiology of pure red cell aplasia: implications for therapy

Blood ◽  
1996 ◽  
Vol 87 (11) ◽  
pp. 4831-4838 ◽  
Author(s):  
RJ Charles ◽  
KM Sabo ◽  
PG Kidd ◽  
JL Abkowitz
Keyword(s):  
Drug Exposure ◽  
Mononuclear Cells ◽  
Parvovirus B19 ◽  
Pure Red Cell Aplasia ◽  
Immunosuppressive Drugs ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Chi Square Analysis

To determine the utility of marrow culture in defining the natural history and therapeutic response of pure red cell aplasia we have studied 37 patients. Patients were evaluated at the University of Washington before specific therapies (n = 21) or at the time of treatment failure in = 16). Evaluation included a medical and drug exposure history, a physical examination, a chest x-ray or computed tomography to rule out thymoma, lymphocyte immunophenotype studies, anti-nuclear antibody and rheumatoid factor determinations, marrow cytogenetics, and marrow progenitor cell cultures. Retrospective Southern analyses to detect human parvovirus B19 was performed in the 27 patients for whom sera was stored. Clinical follow-up was obtained to document therapeutic responses. Normal burst forming unit-erythroid (BFU-E) growth (>30 bursts/10(5) marrow mononuclear cells [MMNC]) in culture proved an outstanding predictor of clinical response, as 27 of 29 individuals with normal frequencies of erythroid bursts in culture responded to immunomodulating therapies (sensitivity 96%, specificity 78%, predictive value 93%, P = .0001 with two-tailed chi square analysis). Overall, 28 patients responded to either immunomodulating therapies or drug withdrawal. Twenty-four patients obtained a normal hematocrit (complete response [CR] and 4 additional patients became transfusion independent (partial response). Although responding patients often required several therapies, 20 of 24 (83%) patients who obtained a CR have sustained a normal hematocrit without maintenance therapy at the time of last follow-up (median 5 years). In contrast, of 8 patients with poor in vitro BFU-E growth (<6 bursts/10(5) MMNC), 7 failed to respond to any therapy and all died (median survival time 17 months). Our data suggest that in individuals, from whom BFU-E mature appropriately in culture, immunosuppressive drugs should be used sequentially until a CR is obtained and a durable remission is the expected outcome.

Pure red cell aplasia due to parvovirus B19 in a patient treated with rituximab

Blood ◽  
2000 ◽  
Vol 96 (3) ◽  
pp. 1184-1186 ◽  
Author(s):  
Vivek R. Sharma ◽  
Donald R. Fleming ◽  
Stephen P. Slone
Keyword(s):  
Monoclonal Antibody ◽  
B Lymphocytes ◽  
Parvovirus B19 ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Humoral Immune ◽  
Red Cell Aplasia ◽  
Point Of Interest ◽  
Humoral Immune System ◽  
Parvovirus B19 Infection

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.

scholarly journals Diphenylhydantoin-induced pure red cell aplasia

Blood ◽  
1985 ◽  
Vol 65 (4) ◽  
pp. 789-794 ◽  
Author(s):  
EN Dessypris ◽  
S Redline ◽  
JW Harris ◽  
SB Krantz
Keyword(s):  
Colony Formation ◽  
Cytotoxic Effect ◽  
Autologous Blood ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Erythroid Progenitors ◽  
Red Cell Aplasia ◽  
Serum Igg ◽  
Allogeneic Marrow

Abstract The pathogenesis of diphenylhydantoin-induced pure red cell aplasia was investigated in the case of a 32-year-old man who developed pure red cell aplasia while he was under treatment with diphenylhydantoin. The patient's serum IgG purified from serum drawn at the time of diagnosis suppressed normal allogeneic marrow colony-forming (CFU-E) and burst- forming (BFU-E) and autologous blood BFU-E growth in vitro only in the presence of diphenylhydantoin. This IgG-diphenylhydantoin complex had no effect on CFU-GM growth in vitro. Normal IgG or patient's IgG purified from serum drawn after the remission of red cell aplasia had no effect on erythroid colony formation in vitro in the presence of diphenylhydantoin. The IgG-diphenylhydantoin complex exerted no direct cytotoxic effect on normal marrow erythroblasts, CFU-E, and BFU-E, nor did it interfere with the action of erythropoietin on marrow erythroblasts. These studies suggest that diphenylhydantoin-induced red cell aplasia is immunologically mediated through an IgG inhibitor, which requires the presence of the drug to suppress erythroid colony formation in vitro. This inhibitor seems to exert its effect on erythroid progenitors at or beyond the stage of differentiation of CFU- E, but not on erythroblasts.

scholarly journals Pure red cell aplasia due to parvovirus B19 in pediatric malignancies – a report of two cases

2017 ◽  
Vol 2 (2) ◽  
pp. S19-S20
Author(s):  
Putun Patel ◽  
Vibha Bafna ◽  
Sandip Bartakke ◽  
Priya Gupta ◽  
Sanjay Mankar ◽  
...  
Keyword(s):  
Parvovirus B19 ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Pediatric Malignancies ◽  
Red Cell Aplasia

scholarly journals Ten year follow up of erythropoietin induced autoimmune pure red cell aplasia

2013 ◽  
Vol 23 (4) ◽  
pp. 323 ◽  
Author(s):  
R Ram ◽  
G Swarnalatha ◽  
PNageswar Reddy ◽  
CShyam Sundar Rao ◽  
GDiwakar Naidu ◽  
...  
Keyword(s):  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Red Cell Aplasia

scholarly journals Pure red cell aplasia caused by Parvo B19 virus in a kidney transplant recipient

2012 ◽  
Vol 52 (186) ◽  
Author(s):  
A Baral ◽  
B Poudel ◽  
R K Agrawal ◽  
R Hada ◽  
S Gurung
Keyword(s):  
Bone Marrow ◽  
Transplant Recipient ◽  
Intravenous Immunoglobulin ◽  
Kidney Transplant ◽  
Parvovirus B19 ◽  
Kidney Transplant Recipient ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Erythroid Progenitor ◽  
Red Cell Aplasia

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.

Pure Red Cell Aplasia Related to Parvovirus B19 Infection in Simultaneous Pancreas and Kidney Recipient: A Case Report

2020 ◽  
Vol 52 (8) ◽  
pp. 2539-2543 ◽  
Author(s):  
Ewa Nowacka-Cieciura ◽  
Ewa Karakulska-Prystupiuk ◽  
Anna Żuk-Wasek ◽  
Wojciech Lisik ◽  
Grzegorz Władysław Basak ◽  
...  
Keyword(s):  
Case Report ◽  
Parvovirus B19 ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Parvovirus B19 Infection ◽  
Kidney Recipient

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
Keyword(s):  
Response Rate ◽  
Parvovirus B19 ◽  
Pure Red Cell Aplasia ◽  
Adult Patients ◽  
Red Blood Cell Transfusion ◽  
Red Cell ◽  
Treatment Burden ◽  
Intravenous Immunoglobulin Therapy ◽  
Red Cell Aplasia ◽  
Blood Disorder

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.

Thymoma Associated with Pure Red-Cell Aplasia: Clinical Features and Prognosis

2002 ◽  
Vol 10 (2) ◽  
pp. 150-154 ◽  
Author(s):  
Tomohiro Murakawa ◽  
Jun Nakajima ◽  
Hajime Sato ◽  
Makoto Tanaka ◽  
Shinichi Takamoto ◽  
...  
Keyword(s):  
Myasthenia Gravis ◽  
Clinical Features ◽  
Surgical Intervention ◽  
Pure Red Cell Aplasia ◽  
The Other ◽  
Entire Group ◽  
Red Cell ◽  
Extended Thymectomy ◽  
Red Cell Aplasia

As information on the clinical features and prognosis of thymoma complicated by pure red-cell aplasia is limited, follow-up data on thymoma patients who had a thymectomy between 1954 and 1999 were analyzed retrospectively. Six of 166 cases were complicated by pure red-cell aplasia. In 3 of these, the pure red-cell aplasia appeared after surgical intervention. Remission was observed in 2 patients who underwent extended thymectomy. The other 4 patients subsequently died from pure red-cell aplasia. The outcome in patients with pure red-cell aplasia was poorer than that in the entire group of patients with thymoma and in those with thymoma complicated by myasthenia gravis. The possible onset of pure red-cell aplasia after thymectomy should be kept in mind during follow-up.

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