Response of AIHA to high dose intravenous immunoglobulins in a patient with ovarian teratoma

Abstract

Eur J Clin Invest 2009; 39 (6): 531-532Sir, A 15-year-old female was admitted at the Division of Haematology because of persisting pallor and anaemia; haemoglobin (Hb) level was 5AE4 g dL )1 , red blood cell count (RBC) 1AE480 x 10 9 L )1 ; reticulocyte count 36 · 10 9 L )1 ; white blood cell and platelet count was normal. Anysocytosis, macrocytosis, hyperchromasia and nucleated RBC were noted on peripheral blood smear. Serum lactate dehydrogenase was 192 IU L )1 (70-190 IU L )1 ), total bilirubin 0AE56 mg dL )1 (0AE4-1AE1), haptoglobin 60 mg dL )1 (58-356) and Vitamin B 12 345 pg mL )1 (200-900). Anti-nuclear antibody and anti-dsDNA antibody were negative. Direct antiglobulin test (DAT), performed with broad spectrum and monospecific antisera (anti-IgG+C3d, -IgG, -IgA, -IgM, -C3d and -C3b supplied by Ortho Clinical Diagnostics, Raritan, NJ, USA, and DiaMed, Cressier sur Morat, Switzerland), was strongly positive for the presence of an IgG autoantibody, which was present in the eluate and in patient's serum: autoimmune haemolytic anaemia (AIHA) was diagnosticated.Abdominal computed tomography and nuclear magnetic resonance revealed an ovarian cystic mass. Prednisolone (1 mg kg )1 day )1 ) was immediately implemented, with a poor response; after 3 weeks, intravenous immunoglobulins (IVIg) therapy (800 mg kg )1 per week for 1 month) was added. Haemoglobin reached 12 g dL )1 in 4 weeks; 7 weeks after admission, she underwent surgery: the specimen was ovarian teratoma showing soft material, hair and teeth. DAT became negative on the 14th day after surgery; 2 months later all serological tests were negative and Hb was 13AE7 g dL )1 (Fig. 1). Prudentially, the steroid therapy was gradually tapered until 5 mg three times a week for 3 months after surgery.A recent study identified a subset of more than 50 autoantigens on the surface of ovarian cancer cells that could share some structures with RBC, hence the production of crossreactive autoantibodies [1]. Glucocorticoids are the treatment of first choice in AIHA, but when this condition is associated with ovarian neoplasm, they are ineffective [2,3]. IVIg are used in many immuno-related diseases to accelerate clearance of autoantibodies by anti-idiotypic effect, to block Fc receptors and to inhibit antibody synthesis. IVIg treatment of AIHA in teratoma patients has not been reported previously; actually guidelines on the use of IVIg do not recommend this therapy in AIHA, except under certain life-threatening circumstances [4]. Other therapeutic options, which are used when a rapid reduction of pathogenic antibodies is required, e.g., plasma exchange (PE), are also not reported. Moreover, because of prompt rebound effect, the PE therapy in autoimmune diseases is not credited of significant benefits. Recent data have shown that pre-transfusion PE performed to improve transfusion efficacy also has no effect [5]. In our patient, IVIg had to be used with steroids to foster haemoglobin recovery and to allow the safe surgical excision, without blood support; the quick response ...