Background Pure Red CEll Aplasia (PRCA) is characterised by normochromic normocytic anaemia, reticulocytopenia and aplasia or severe hypoplasia of the red cell line with normal white cell and megakaryocyte lines in the bone marrow.1 PRCA may be associated with other conditions,2 but the association with systemic lupus erythematosus (SLE) is rare. Here we summarise our case and the previous cases of PRCA and SLE reported in the English literature.
Objectives To evaluate the clinical and laboratory features of all the patients reported with both SLE and PRCA.
Methods In addition to case, cases were identified from the English literature through a search using the Medline from the year 1966 till December 2000, and also through references of relevant publications.
Results Twenty-four cases, including our case were identified. The association between SLE and PRCA is relatively rare. However it may be underdiagnosed. In most of the cases SLE was diagnosed prior to or concomitantly with the diagnosis of PRCA. The clinical and laboratory features relevant to SLE in our patients are similar to the general patients of SLE, except for significantly less pleuritis and a trend for less nephritis, hallucinations, thrombopenia and leukopenia. Most of the patients had no clinical or laboratory activity at the time of PRCA diagnosis. In some patients as in the general patients with PRCA, inhibitory effect of the patient’s serum against erythroid progenitor cells and against erythropoietin (EPO) were identified. In most of the patients PRCA responded to corticosteroid (CS) treatment, and in some of the non-responders other modalities of treatment like intravenous gammaglobulins and even EPO were helpful.
Conclusion The association between PRCA and SLE is relatively rare. The clinical and laboratory features relevant to SLE in our patients are similar to the general patients with SLE, and the spectrum of inhibitory effect on the erythroid progenitor cells and EPO are similar to the general patients with PRCA. Most patients had favourable response to CS treatment.
Schmid JR, Kiely JM, Pease GL, Hargraves MM. Acquired pure red cell aplasia. Acta Haematol. 1963;30:325
Amus SS, Yunis AA. Aqcuired pure red cell aplasia. Am J Med. 1987;24:311–26
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