Successful treatment of warm-type haemolytic anaemia with bortezomib in a rituximab-failed systemic lupus erythematosus patient.

Successful treatment of warm-type haemolytic anaemia with bortezomib in a rituximab-failed systemic lupus erythematosus patient SIR, Autoimmune haemolytic anaemia (AIHA) is classified as either warm or cold type, based on the characteristics of the autoantibodies involved in the pathogenesis of the disease. Each type of AIHA has a different underlying cause, which has a great impact on the treatment and outcome [1]. Glucocorticoid is the first-line treatment in warm AIHA. In some patients resistant to glucocorticoids, treatment may include immunosuppressive agents, rituximab or even splenectomy. Many immunosuppressive drugs have been used in attempts to treat refractory warm-type AIHA patients, including AZA, CYC, ciclosporin and MMF. Unfortunately the clinical response to these treatments is unpredictable and the response rate is relatively low. Therefore novel therapies for patients with steroid-refractory AIHA are urgently needed [2]. Here we report the case of a 65-year-old woman with SLE and a history of severe anaemia for 21 years. She had a strongly positive direct antiglobulin test with both IgG and C3d deposition on red blood cells (RBCs). She had oral ulcers and polyarthritis intermittently, which responded well to glucocorticoid treatment. The other laboratory findings were as follows: ANA was positive (1:320), anti-dsDNA and anti-ENA were negative, C3 (normal <12 RU/ml). Cryoglobulin test was negative. She was diagnosed as SLE according to 1997 ACR classification criteria [3]. The anaemia was so severe that the patient had to receive intensive blood transfusion support (1–3 U of RBCs/ day). At first, high-dose prednisone (1 mg/kg) had a good clinical response. However, the anaemia has repeatedly relapsed in the past 5 years when prednisone is tapered. Pulse therapy (1 g/day every 3 days), AZA, CYC and dana-zol showed poor response. Rituximab (375 mg/m 2 /week four times) in combination with prednisone 1 mg/kg/day was started in January 2012 and her haemoglobin was stable for $ 6 months. Unfortunately, when prednisone was tapered to 20 mg/day, the haemolytic anaemia relapsed again with elevated reticulocytes in September 2012. This time the patient rejected increasing the dosage of prednisone due to lumber fracture and diabetes. Since AIHA is an antibody-mediated disease, especially in autoimmune disease, we measured the lymphocytes and plasma cell counts in her peripheral blood and bone marrow. A small (27%) restricted plasma cell population that was CD138 + CD19 À , expressing CD117 was found in bone marrow. There was no monoclonal band in the immunofixation electro-phoresis examination. Plasma …