scholarly journals Possible complications of erythropoietin therapy in patients with chronic renal failure

2004 ◽  
Vol 57 (5-6) ◽  
pp. 254-257 ◽  
Author(s):  
Steva Pljesa
Keyword(s):  
Bone Marrow ◽  
Red Blood Cells ◽  
Blood Cells ◽  
Reticulocyte Count ◽  
Pure Red Cell Aplasia ◽  
Recombinant Erythropoietin ◽  
Red Cell ◽  
Serbia And Montenegro ◽  
Red Cell Aplasia ◽  
Sudden Appearance

Introduction There have been many publications in the past 20 years about positive effects of human recombinant erythropoietin, which is used in treatment of anemia, especially in patients on dialysis. Complications The most important complications in patients treated with erythropoietin include: hypertensive reactions; thrombosis of AV fistula in patients on hemodialysis and appearance of severe anemia as a part of Pure Red Cell Aplasia (PRCA). The first two complications were managed quite easily with adequate erythropoietin dosage, and slower establishment of normal hemoglobin kevel, hematocrit level and red blood cell count, (our "target" Hb varied between 100 and 110 g/dl). Pure Red Cell Aplasia (PRCA) Pure Red Cell Aplasia is a progressive, marked anemia with sudden appearance of signifacnt loss or complete absence of erythrocyte precursor cells in normal bone marrow. In patients with end stage renal disease treated with erythropoietin PRCA appears in acute form as a consequence of production of neutralizing antibodies to erythropoietin. Time period between the beginning of erythropoietin therapy and appearance of PRCA is from 3 weeks to approximately 9 months. Symptoms and signs PRCA is characterized by sudden appearance of anemia in patients who had a satisfactory response to erythropoietin therapy till that moment. In PRCA, anemia is normocytic, normochromic with normal survival of red blood cells, without deficit in components such as iron, folic acid or vitamin B12, low reticulocyte count, decrease in Hg and normal platelet count. Diagnosis Diagnosis is based on clinical data (marked anemia), bone marrow biopsy, which shows a lower number of precursor red blood cells and presence of antibodies against erythropoietin. Before PRCA is diagnosed, all other causes for erythropoietin resistance must be excluded. Therapy Therapy of PRCA is based on cessation of erythropoietin therapy (all kinds), and correction of anemia with blood transfusions. Incidence PRCA is very rare and occurs in less than 1:10.000 patient-years in patients treated with erythropoietin, not lethal by itself and generally reversible. Till December 31, 2002. PRCA has been diagnosed in 142 patients world wide. In Serbia and Montenegro till this moment there hasn't been a single case of this syndrome. Preventive measures Continuous follow-up of reticulocyte count is the first step. Although this is a very rare disease, most of European Societies of Nephrology made protocols that recommend only intravenous application of -epoetin. Considering this new situation, Nephrology Society of Serbia and Montenegro recommends that -epoetin should be given to patients on hemodialysis only intravenously, while subcutaneous application of -epoetin is recommended in patients before beginning the dialysis treatment and in patients on hemodialysis, or who had undergone kidney transplantation.

A Pilot Study Of Rituximab In Patients With Moderate Aplastic Anemia, Diamond-Blackfan Anemia and Pure Red Cell Aplasia

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 2479-2479 ◽  
Author(s):  
Sabrina Martyr ◽  
Arun Balakumuran ◽  
Aldemar Montero ◽  
Cynthia E. Dunbar ◽  
Elizabeth M. Kang ◽  
...  
Keyword(s):  
Monoclonal Antibody ◽  
Bone Marrow ◽  
Aplastic Anemia ◽  
Reticulocyte Count ◽  
Bone Marrow Failure ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Diamond Blackfan Anemia ◽  
Red Cell Aplasia ◽  
Bone Marrow Failure Syndromes

Abstract Background Pure red cell aplasia (PRCA), Diamond-Blackfan anemia (DBA) and moderate aplastic anemia (MAA) are all bone marrow failure syndromes that are immune-mediated or may respond to immunosuppressive therapies (IST). Anti-thymocyte globulin, cyclosporine and corticosteroids have been used with some success but have significant toxicities. The humanized monoclonal antibody to the interleukin-2 receptor on T cells, daclizumab, showed efficacy in MAA and PRCA patients with some patients achieving transfusion independence (Sloand et al, Haematologica 2010). However, this agent has since been withdrawn from the market. It is increasing recognized that the anti-CD20 chimeric monoclonal antibody, rituximab, may modulate T cell immunity in addition to its known depletion of B cells (Staci, Seminars in Hematology 2010). There are anecdotal case reports of rituximab, showing benefit in PRCA. Here, we summarize our experience using rituximab in PRCA, DBA and MAA. Design and Methods We enrolled 11 patients with PRCA (n = 7), DBA (n = 1), and MAA (n = 3) who had failed at least one prior immunosuppressive regimen to receive rituximab 375 mg/m2intravenous infusions weekly times 4 doses (NCT00229619). Responses were evaluated at 3, 6 and 12 months. Patients with MAA, DBA or PRCA were eligible for trial participation. MAA was defined as a hypocellular marrow without evidence of an underlying disease process and depression of at least two of three cell lines (an absolute neutrophil count (ANC) ≤ 1200/µL, a platelet count ≤ 70,000/µL, and a hemoglobin ≤ 8.5 g/dL or absolute reticulocyte count (ARC) ≤ 60, 000/µL in transfusion-dependent patients) but who do not fulfill criteria for severe aplastic anemia (i.e. bone marrow cellularity < 30% and depression of two of the three peripheral counts: ANC < 500/µL, a platelet count < 20,000/µL and an ARC < 60,000/µL). DBA and PRCA were defined as anemia, reticulocytopenia (ARC ≤ 50, 000/µL) and absent or decreased marrow erythroid precursors. Patients with Fanconi’s anemia, other congenital bone marrow failure syndromes, cytologic abnormalities indicating myelodysplasia or recent/ongoing parvovirus infection were excluded. Complete response (CR) was defined as return of blood counts to normal. Partial response (PR) for MAA was defined as improvement in two of the three depressed blood counts that qualified patient for participation. PR for DBA/PRCA was defined as an increase in hemoglobin by 1.5 g/dl of blood and or ARC ≥ 50,000/µL but not meeting criteria for normal counts. Results Overall, 5/11 (45%) patients responded to rituximab, all achieving PR. At 3 months, one patient with PRCA had responded. At 6 months, two additional patients responded (one with PRCA, one with MAA). At 12 months, an additional two responses were confirmed (one PRCA, one MAA). One PRCA patient lost his response between the 6 and 12 month endpoint. Among the three responding PRCA patients, the mean reticulocyte count at study initiation was 4400/µL; this increased to 54,000/µL at 6 months and further increased to 61,000/µL at 12 months (including patient who lost his response). The study was terminated early for poor accrual; many eligible patients received alternate treatments at home. Due to early study termination, the duration of responses for majority of the patients is unknown. Given the reports of daclizumab efficacy in these diseases, 90% of our patients were previously treated with daclizumab. Notably, 3 of the patients responding to rituximab had previously not responded to daclizumab. Safety The most common toxicity of rituximab observed was an infusion related reaction affecting (8/11) 73% of patients with the first infusion of rituximab. One patient developed serum sickness after the third cycle which precluded the administration of the last dose. An expected decrease in quantitative immunoglobulin levels was observed; at the 6 month evaluation there was an 11% decrease in IgG and IgA; a greater decrease (48%) was observed in IgM. Conclusions Rituximab is a viable treatment option in the armamentarium for patients with PRCA and MAA. Rituximab is safe, effective, and easily administered. Responses can be delayed to beyond 6 months therefore we suggest observation for at least 6 months after rituximab administration. Disclosures: Off Label Use: Rituximab is not FDA approved for the treatment of Pure Red Cell Aplasia, Diamond-Blackfan Anemia or Moderate Aplastic Anemia.

Pure red cell aplasia of the bone marrow in combination with thymoma. A literature review and own data

2019 ◽  
Vol 91 (7) ◽  
pp. 121-126
Author(s):  
A A Petrenko ◽  
A V Pivnik ◽  
G A Dudina ◽  
M G Dubnitskaya
Keyword(s):  
Bone Marrow ◽  
Literature Review ◽  
Blood Cells ◽  
Spindle Cell ◽  
Pure Red Cell Aplasia ◽  
Morphological Type ◽  
Red Cell ◽  
Coombs Test ◽  
Red Cell Aplasia ◽  
Type A Thymoma

Eight patients were observed with a rare combination of thymoma and pure red cell aplasia of bone marrow (PRCA), of which seven women were between 44 to 68 years old. The diagnosis of PRCA was established before the detection of thymoma in 1 patient, simultaneously in 3, after - in 4. Seven patients underwent timomectomy. The weight of removed thymomas was from 200 to 780 grams. Morphological type A thymoma variant (spindle cell) was installed in 2 patients, type B1 - in 2, type B2 - in 2, type B3 - in 2. Complete remissions were obtained using cyclophosphamide and cyclosporin in 5 patients, lasting from 6 months to 7 years. The results of immunological studies with the identification of non - hemolytic antibodies to the proteolytic antigen (Pr1d) on the erythrocyte membrane in 4 patients are presented. Of these, two studied patients simultaneously detected antibodies to the Pr1d antigen and the interspecific antigen of mammalian erythroblasts (IAME). It is shown that the lifespan of red blood cells are not changed. The direct Coombs test was negative in 5 patients, but with the help of aggregate hemaglutination test and enzyme immunoassay, antibodies were detected on the surface of erythrocytes. The pathogenesis of this combination of diseases remains unclear and needs to be elucidated.

PURE RED CELL APLASIA : A CASE REPORT

2021 ◽  
pp. 55-56
Author(s):  
G Srivani ◽  
D Roja Aishwarya ◽  
P. V. S. Kiran
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Failure ◽  
Bone Marrow Aspiration ◽  
Oral Prednisolone ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Normocytic Normochromic Anemia ◽  
Pure Cell ◽  
Normochromic Anemia

Pure cell aplasia is a rare bone marrow failure that affects erythroid lineage characterized by normocytic normochromic anemia with reticulocytopenia in the peripheral blood and absent or infrequent erythroblasts in the bone marrow. It can be congenital or acquired. Acquired can be primary when no cause is identied or secondary-due to underlying or associated pathology. Herein we report a case of a 28 year old female with Primary Acquired Pure Red cell aplasia. The patient presented with severe anemia (Hb-1.9gm%) and low reticulocyte count 0.1%. Bone marrow aspiration shows normocellular marrow with Decreased erythropoiesis with M:E ratio of 20:1..Patient was started on oral prednisolone and improvement was seen and the patient became transfusion independent.

Anemia: Production Defects Generally Associated with Marrow Aplasia or Replacement

2017 ◽  
Author(s):  
Nancy Berliner ◽  
John M Gansner
Keyword(s):  
Bone Marrow ◽  
Differential Diagnosis ◽  
Aplastic Anemia ◽  
Blood Smear ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Normal Bone Marrow ◽  
Normal Bone ◽  
Red Cell Aplasia

This review focuses on anemia resulting from production defects generally associated with marrow aplasia or replacement. The definition, epidemiology, etiology, pathogenesis, diagnosis, differential diagnosis, management, complications, and prognosis of the following production defects are discussed: Acquired aplastic anemia and acquired pure red cell aplasia. Figures depict a leukoerythroblastic blood smear, a biopsy comparing normal bone marrow and bone marrow showing almost complete aplasia, and a marrow smear. A table lists the causes of aplastic anemia. This review contains 3 figures; 1 table; 108 references.

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.

scholarly journals Carbamazepine-Induced Pure Red Cell Aplasia

2018 ◽  
Vol 05 (01) ◽  
pp. 050-052 ◽  
Author(s):  
C. Mansoor ◽  
Laksmi Priya
Keyword(s):  
Bone Marrow ◽  
Rare Disease ◽  
Pure Red Cell Aplasia ◽  
Normocytic Anemia ◽  
Red Cell ◽  
Normal Bone Marrow ◽  
Hematologic Disorders ◽  
Normal Bone ◽  
Red Cell Aplasia ◽  
Carbamazepine Therapy

AbstractAntiepileptic therapy is associated with various hematologic disorders. Pure red cell aplasia (PRCA) is a rare disease that may be congenital or acquired. Severe normocytic anemia, reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow should raise the suspicion of PRCA. A 32-year-old unmarried woman was admitted with fatigue for 4 months. She had been on carbamazepine therapy for 4 years (200 mg twice daily) for seizure disorder. On evaluation, she was diagnosed to have PRCA secondary to carbamazepine. We describe a patient with carbamazepine-induced PRCA that improved after discontinuation of the drug.

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