Mr.S, 54 yrs old gentleman, is on regular dialysis for his chronic renal failure due to Diabetic nephropathy. He was known to have Ischemic heart disease and underwent single vessel angioplasty, few months before. He was on dual anti platelet therapy post angioplasty.

His chronic renal failure was managed by thrice weekly dialysis, dietary support, erythropoietin thrice weekly for his anaemia, calcium supplementation, and sugars were controlled with insulin.

He maintained haemoglobin at 10 grams and there was gradual drop in his haemoglobin. He complained of epigastric pain but there was no hemetemesis or malena.  His stool occult blood tested repeatedly was negative and upper and lower GI scopy studies were normal. His serum ferritin was normal, so as Transferrin saturation. His corrected reticulocyte count was < 0.8 % indicating bone marrow dysfunction.

He underwent a bone marrow biopsy and that was consistent with PRCA – Pure Red Cell Aplasia.

Management : He underwent screening for Parvovirus ( negative), and his anti EPO antibody (EPO – Erythropoietin) result was low positive.. He has been initiated on Cyclosporin and steroids and he didn’t need blood transfusion subsequently.

What is PRCA ?

Pure Red Cell Aplasia is a condition where there is absolute decrease in the production of red cells in the bone marrow. Contrary to Aplastic anemia, here the WBC and the platelet productions are normal. There is marked decrease in reticulocyte numbers, the red cells are normocytic and normochromic and significant reduction or absence of erythroid precursors on bone marrow biopsy.(1)

PRCA –Etiology

It can be Congenital( Diamond- BlackfanAnemia(2)   – associated with physical malformations due to multiple ribosomal gene abnormalities and Pearson syndrome (3)– a congenital mitochondrial gene defect associated with pancreatic insuffucuency and PRCA ) OR Acquired.

Acquired PRCA can be primary that is due to antibodies to red cell precursors. Secondary PRCA could be due to auto immune diseases like lupus, haematological malignancies like lymphomas and leukaemia, infections – classically Parvovirus B 19 (4) , hepatitis B &C, infectious mono nucleosis, variety of drugs, thymomas and sold tumours.

Clinical manifestations 

Are predominantly due to anemia – easy fatiguability, exertional breathlessness, chest discomfort, palpitations etc. Manifestations could be present related to the underlying secondary causes in secondary PRCA.

Investigations :

The routine haematological evaluation for anaemia including complete blood count, peripheral smear, reticulocyte count, endoscopy, coomb’s test etc need to be done. The confirmatory incestigation of  PRCA is bone marrow study.

This will reveal normal white cell and platelet series but grossly diminished red cell precursors.

Management of PRCA (Primary)

This involves immuno suppressive medications as it is due to antibody against EPO or erythroid precursors. Among the various drugs tried the best response is obtained with Cyclosporin (5) either alone or in combination with steroids. Other agents tried are Rituximab (6), IVIG, cyclophosphamide.

For refractory disease, plasmapharesis is tried. In patients with chronic kidney disease, renal transplantation is recommended if medical management .

1. Sawada K, Fujishima N, Hirokawa M. Acquired pure red cell aplasia: updated review of treatment. Br J Haematol. 2008;142(4):505-514
2. Ruggero D, Shimamura A. Marrow failure: a window into ribosome biology. Blood. 2014;124(18):2784-2792.
3. Fleming MD. The genetics of inherited sideroblasticanemias. SeminHematol. 2002;39(4):270-281.
4. Frickhofen N, Chen ZJ, Young NS, Cohen BJ, Heimpel H, Abkowitz JL. Parvovirus B19 as a cause of acquired chronic pure red cell aplasia. Br J Haematol. 1994;87(4):818-824
5. Means RT, Dessypris EN, Krantz SB. Treatment of refractory pure red cell aplasia with cyclosporine A: disappearance of IgG inhibitor associated with clinical response. Br J Haematol. 1991;78:114-119.
6. Auner HW, Wölfler A, Beham-Schmid C, Strunk D, Linkesch W, Sill H. Restoration of erythropoiesis by rituximab in an adult patient with primary acquired pure red cell aplasia refractory to conventional treatment. Br J Haematol. 2002;116(3):727-728.

Dr.R.Balasubramaniyam
Chief Of Nephrology
Kauvery Hospital