Pure red cell aplasia (PRCA) is a recognized but rare complication of systemic lupus erythematosus (SLE) and is characterized by the near absence of red blood cell precursors in the bone marrow but with normal megakaryocyte and granulocytes. We report a novel case of acquired PRCA occurring simultaneously with immune thrombocytopenia in the context of active SLE. Both syndromes were refractory to conventional treatment but responded to rituximab and cyclophosphamide.
HochbergMCUpdating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum1997; 409: 1725–1725.
2.
WaltonTKarimYWrightDKhamashtaMHughesGThe association of pure red cell aplasia with the antiphospholipid syndrome. Lupus2001; 1012: 899–901.
3.
ElimelakhMDaytonVParkKSRed cell aplasia and autoimmune hemolytic anemia following immunosuppression with alemtuzumab, mycophenolate, and daclizumab in pancreas transplant recipients. Haematologica2007; 928: 1029–1036.
4.
CasadevallNDupuyEMolho-SabatierPTobelemGVaretBMayeuxPAutoantibodies against erythropoietin in a patient with pure red-cell aplasia. N Engl J Med1996; 33410: 630–633.
5.
SawadaKFujishimaNHirokawaMAcquired pure red cell aplasia: Updated review of treatment. Br J Haematol2008; 1424: 505–514.
6.
GiannouliSVoulgarelisMZiakasPDTzioufasAGAnaemia in systemic lupus erythematosus: From pathophysiology to clinical assessment. Ann Rheum Dis2006; 652: 144–148.
7.
LiuHOzakiKMatsuzakiYAbeMKosakaMSaitoSSuppression of haematopoiesis by IgG autoantibodies from patients with systemic lupus erythematosus SLE. Clin Exp Immunol1995; 1003: 480–485.
8.
NarraKBorghaeiHAl-SaleemTHoglundMSmithMRPure red cell aplasia in B-cell lymphoproliferative disorder treated with rituximab: Report of two cases and review of the literature. Leuk Res2006; 301: 109–114.
9.
GhazalHSuccessful treatment of pure red cell aplasia with rituximab in patients with chronic lymphocytic leukemia. Blood2002; 993: 1092–1094.
10.
DungarwallaMMarshJCToozeJALack of clinical efficacy of rituximab in the treatment of autoimmune neutropenia and pure red cell aplasia: Implications for their pathophysiology. Ann Hematol2007; 863: 191–197.
11.
CournoyerDToffelmireEBWellsGAAnti-erythropoietin antibody-mediated pure red cell aplasia after treatment with recombinant erythropoietin products: Recommendations for minimization of risk. J Am Soc Nephrol2004; 1510: 2728–2734.
12.
SchettGFirbasUFurederWDecreased serum erythropoietin and its relation to anti-erythropoietin antibodies in anaemia of systemic lupus erythematosus. Rheumatology (Oxford)2001; 404: 424–431.
