scholarly journals Pure red cell aplasia (PRCA): Response of three patients of cyclophosphamide and/or antilymphocyte globulin (ALG) and demonstration of two types of serum IgG inhibitors to erythropoiesis

Blood ◽  
1975 ◽  
Vol 45 (2) ◽  
pp. 247-261 ◽  
Author(s):  
A Marmont ◽  
C Peschle ◽  
M Sanguineti ◽  
M Condorelli
Keyword(s):  
Type A ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Antilymphocyte Globulin ◽  
Type B ◽  
Erythroid Precursor ◽  
Red Cell Aplasia ◽  
Serum Igg ◽  
First Case

Abstract Three cases of adult pure red cell aplasia (PRCA) ARE REPORTED. All patients proved refractory to various combinations of androgens and corticosteroids. The first case, harboring a thymoma, showed a complete clinical remission following cyclophosphamide therapy. The second and third responded similarly to either a combined cyclophosphamide + antilymphocyte globulin (ALG) treatment or to ALG administration preceded by a small dosage of cyclophosphamide, which had proved ineffective when administered alone. Serum IgG inhibitors to erythropoiesis were demonstrated in all cases by means of in vivo and/or in vitro techniques. The inhibitor(s), although directed against the erythroid marrow in both the first and third patients (PRCA type A), apparently functioned as an antibody to circulating erythropoientin (Ep) in the second case (PRCA type B). The inhibitor(s) was always absent in postremission samples. Additionally, experimental models for both types of human PRCA were established in normal rodents. The present studies support the contention that adult PRCA is an autoimmune disease. The therapeutic role of cytotoxic-immunodepressive agents in PRCA patients is confirmed. It is emphasized that ALG may represent an additional therapeutic tool in cases resistant to cyclophosphamide and/or steroids. In addition, cyclophosphamide proved effective in a patient harboring a thymoma not amenable to surgery. Finally, it is postulated that IgG serum autoantibodies, directed against either an early erythroid precursor (PRCA type A) or, more rarely, circulating Ep (PRCA type B), play a major role in the pathogenesis of the disease.

scholarly journals Pure red cell aplasia (PRCA): Response of three patients of cyclophosphamide and/or antilymphocyte globulin (ALG) and demonstration of two types of serum IgG inhibitors to erythropoiesis

Blood ◽  
1975 ◽  
Vol 45 (2) ◽  
pp. 247-261
Author(s):  
A Marmont ◽  
C Peschle ◽  
M Sanguineti ◽  
M Condorelli
Keyword(s):  
Type A ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Antilymphocyte Globulin ◽  
Type B ◽  
Erythroid Precursor ◽  
Red Cell Aplasia ◽  
Serum Igg ◽  
First Case

Three cases of adult pure red cell aplasia (PRCA) ARE REPORTED. All patients proved refractory to various combinations of androgens and corticosteroids. The first case, harboring a thymoma, showed a complete clinical remission following cyclophosphamide therapy. The second and third responded similarly to either a combined cyclophosphamide + antilymphocyte globulin (ALG) treatment or to ALG administration preceded by a small dosage of cyclophosphamide, which had proved ineffective when administered alone. Serum IgG inhibitors to erythropoiesis were demonstrated in all cases by means of in vivo and/or in vitro techniques. The inhibitor(s), although directed against the erythroid marrow in both the first and third patients (PRCA type A), apparently functioned as an antibody to circulating erythropoientin (Ep) in the second case (PRCA type B). The inhibitor(s) was always absent in postremission samples. Additionally, experimental models for both types of human PRCA were established in normal rodents. The present studies support the contention that adult PRCA is an autoimmune disease. The therapeutic role of cytotoxic-immunodepressive agents in PRCA patients is confirmed. It is emphasized that ALG may represent an additional therapeutic tool in cases resistant to cyclophosphamide and/or steroids. In addition, cyclophosphamide proved effective in a patient harboring a thymoma not amenable to surgery. Finally, it is postulated that IgG serum autoantibodies, directed against either an early erythroid precursor (PRCA type A) or, more rarely, circulating Ep (PRCA type B), play a major role in the pathogenesis of the disease.

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 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

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 First Case Report of Acquired Pure Red Cell Aplasia Associated with Micafungin

2011 ◽  
Vol 50 (9) ◽  
pp. 1051-1054 ◽  
Author(s):  
Mayumi Yoshida-Hiroi ◽  
Masayuki Koizumi ◽  
Reiko Oka ◽  
Aki Mitsuda ◽  
Naoki Hiroi
Keyword(s):  
Case Report ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
First Case

scholarly journals TKI-induced pure red cell aplasia: first case report of pure red cell aplasia with both imatinib and nilotinib

ESMO Open ◽  
2016 ◽  
Vol 1 (3) ◽  
pp. e000058 ◽  
Author(s):  
Bishesh Sharma Poudyal ◽  
Sampurna Tuladhar ◽  
Bishal Gyawali
Keyword(s):  
Case Report ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
First Case

scholarly journals Acquired Amegakaryocytic Thrombocytopenia and Pure Red Cell Aplasia in Thymoma

2018 ◽  
Vol 2018 ◽  
pp. 1-3 ◽  
Author(s):  
Sumit Dahal ◽  
Eliza Sharma ◽  
Suyash Dahal ◽  
Binav Shrestha ◽  
Bikash Bhattarai
Keyword(s):  
Aplastic Anemia ◽  
Diagnostic Procedures ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Early Presentation ◽  
Erythroid Precursor ◽  
Red Cell Aplasia ◽  
History Of ◽  
Intravenous Steroids ◽  
Acquired Amegakaryocytic Thrombocytopenia

Association of thymoma with myasthenia gravis, pure red cell aplasia, and aplastic anemia is well documented. However, thymoma complicated by acquired amegakaryocytic thrombocytopenia (AAMT) is rarely reported. Here, we present a case of a 60-year-old male with past medical history of recurrent invasive thymoma who presented with cough and blood in sputum. He was found to have severe normocytic normochromic anemia and thrombocytopenia that did not improve with intravenous steroids or multiple transfusions of red cells and platelets. Subsequent bone marrow biopsy showed severely depleted megakaryocytes and erythroid precursor cells with relative myeloid hyperplasia suggestive of amegakaryocytic thrombocytopenia and red cell aplasia. He was started on oral cyclosporine but subsequently developed leukopenia and refused any further treatment or diagnostic procedures and left the hospital against medical advice. AAMT, thus, may be a very early presentation of impending aplastic anemia, and treating physicians need to be aware of this entity.

scholarly journals Erythroid progenitors in adult chronic pure red cell aplasia: relationship of in vitro erythroid colonies to therapeutic response

Blood ◽  
1984 ◽  
Vol 64 (1) ◽  
pp. 71-77 ◽  
Author(s):  
C Lacombe ◽  
N Casadevall ◽  
O Muller ◽  
B Varet
Keyword(s):  
Myeloproliferative Disorders ◽  
Pure Red Cell Aplasia ◽  
Type I ◽  
Red Cell ◽  
Type Ii ◽  
Erythroid Progenitors ◽  
Chronic Myeloproliferative Disorders ◽  
Type Iii ◽  
Red Cell Aplasia

Twenty-two cases of idiopathic chronic pure red cell aplasia (PRCA) in adults have been studied to evaluate their erythroid progenitors in vitro using the plasma clot technique. Three types of culture growth patterns were observed and classified as follows. Type I: showing a normal number of autologous CFU-E; type II: CFU-E and BFU-E were detectable but constantly decreased; type III: CFU-E and BFU-E were undetectable. The results were reproducible when patients were studied on two or more occasions. A strong correlation was found between the in vitro growth of autologous erythroid colonies and the results of immunomodulating therapy in 18 evaluable patients. A constant response to immunomodulating treatment was observed in type I patients. A constant failure of treatment was observed in type III patients, whereas results of therapy were unpredictable in type II patients. Two patients with chronic PRCA associated with thymoma and three with chronic myeloproliferative disorders were also studied. Patients with PRCA and thymoma behaved in vitro like type I patients. Patients with chronic myeloproliferative disorders exhibited very low numbers or no CFU-E or BFU-E (type II or III). These data support the hypothesis that at least two mechanisms are responsible for PRCA--one immunologically mediated and the other resulting from a stem cell defect. Moreover, they suggest that the study of erythroid progenitors in vitro might be useful in predicting the immunosuppressive therapy effect in adult chronic PRCA.

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