Pure Red Cell Aplasia and Myelofibrosis in B-cell Neoplasm

2005 ◽  
Vol 33 (4) ◽  
pp. 460-466 ◽  
Author(s):  
Y Hatta ◽  
Y Kura ◽  
T Yano ◽  
H Ushiyama ◽  
M Sugitani ◽  
...  

We describe an unusual case of B-cell neoplasm accompanied by pure red cell aplasia (PRCA) and myelofibrosis in a 67-year-old male presenting with severe anaemia. A few unclassified, myeloperoxidase-negative blastoid cells were seen on bone marrow aspiration, and erythroid cell hypoplasia and myelofibrosis on bone marrow biopsy. An autoimmune PRCA was suspected, as serum CH50, C3 and C4 levels were consistently low. Ciclosporin was effective in treating the anaemia, but anaemia returned when the drug was discontinued. Thirteen months later, the patient was admitted with pleural effusion and ascites that contained monoclonal CD19+ CD20+ immature blast cells with a complex karyotype, thought to be neoplastic B-cells. The unclassified blastoid cells seen earlier may therefore have been from the same origin. The patient deteriorated rapidly and died. Only one case of non-Hodgkin's lymphoma with PRCA and myelofibrosis has been reported previously. We discuss the possibility that dysregulated T-cells induced by neoplastic B-cells may have given rise to concomitant PRCA and myelofibrosis.

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.


1990 ◽  
Vol 14 (3) ◽  
pp. 279-286 ◽  
Author(s):  
Roxanne Alter ◽  
Shantaram S. Joshi ◽  
Joseph D. Verdirame ◽  
Dennis D. Weisenburger

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.


2002 ◽  
Vol 119 (1) ◽  
pp. 125-127 ◽  
Author(s):  
Kevin W. Song ◽  
Peter Mollee ◽  
Bruce Patterson ◽  
William Brien ◽  
Michael Crump

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