scholarly journals Pure red cell aplasia of long duration complicating major ABO- incompatible bone marrow transplantation [see comments]

Blood ◽  
1990 ◽  
Vol 75 (1) ◽  
pp. 290-295 ◽  
Author(s):  
JP Gmur ◽  
J Burger ◽  
A Schaffner ◽  
K Neftel ◽  
O Oelz ◽  
...  

Abstract In 3 of 15 consecutive patients receiving a human leukocyte antigen (HLA)-identical but major ABO incompatible bone marrow transplant (BMT), pure red cell aplasia (PRA) lasting 5 to 8 months was observed. Titers of the incompatible anti-A agglutinin before infusion of the red blood cell (RBC)-depleted BMT was very high in one, and in the usual range in two patients. Decrease of agglutinin titers during the first 4 weeks after BMT were comparable between PRA patients and those of ABO- incompatible BMT recipients with timely RBC recovery. However, in PRA patients, agglutinin titers rose again and remained elevated for 19 to 28 weeks. RBC engraftment and reticulocyte recovery ultimately occurred spontaneously and coincided with the decrease of agglutinin titers below 16. These observations indicate that PRA is antibody-dependent in this setting. Furthermore, it is conceivable that cyclosporine facilitates recipient-derived antibody synthesis after major ABO- incompatible BMT.

Blood ◽  
1990 ◽  
Vol 75 (1) ◽  
pp. 290-295 ◽  
Author(s):  
JP Gmur ◽  
J Burger ◽  
A Schaffner ◽  
K Neftel ◽  
O Oelz ◽  
...  

In 3 of 15 consecutive patients receiving a human leukocyte antigen (HLA)-identical but major ABO incompatible bone marrow transplant (BMT), pure red cell aplasia (PRA) lasting 5 to 8 months was observed. Titers of the incompatible anti-A agglutinin before infusion of the red blood cell (RBC)-depleted BMT was very high in one, and in the usual range in two patients. Decrease of agglutinin titers during the first 4 weeks after BMT were comparable between PRA patients and those of ABO- incompatible BMT recipients with timely RBC recovery. However, in PRA patients, agglutinin titers rose again and remained elevated for 19 to 28 weeks. RBC engraftment and reticulocyte recovery ultimately occurred spontaneously and coincided with the decrease of agglutinin titers below 16. These observations indicate that PRA is antibody-dependent in this setting. Furthermore, it is conceivable that cyclosporine facilitates recipient-derived antibody synthesis after major ABO- incompatible BMT.


2021 ◽  
pp. 55-56
Author(s):  
G Srivani ◽  
D Roja Aishwarya ◽  
P. V. S. Kiran

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.


2017 ◽  
Author(s):  
Nancy Berliner ◽  
John M Gansner

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.


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.


2018 ◽  
Vol 05 (01) ◽  
pp. 050-052 ◽  
Author(s):  
C. Mansoor ◽  
Laksmi Priya

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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