Background Ovarian teratoma is a common ovarian neoplasm. West-Watson and Young described the first case of acquired immune haemolytic anemia (AIHA) in association with an ovarian tumor. Since then there are only sporadic reports of this association, and AIHA as a manifestation of ovarian teratoma remains a rare paraneoplastic syndrome of uncertain pathogenesis. This case report describes the management of a young patient affected by a severe haemolytic anemia. Case report A 15 years-old female was admitted at our hospital because of persisting pallor and anemia fortuitously found by peripheral blood examination. At the time of admission the Hb level was 5.4g/dL, the red blood cell (RBC) count was 1.480x109/L; the reticulocyte count was 3.6x109/L; white blood cell count and platelets value were normal. The serum lactate dehydrogenase was 192 IU/L (70-190 IU/L), total bilirubin 0.56 mg/dL (0.4- 1.1mg/dL), indirect 0.1 (0.1-0.4 mg/dL), haptoglobin 60mg/dL (58-356 mg/dL) and Vitamin B12 345 pg/mL. Anti-nuclear and anti-doublestranded DNA antibodies were negative. Immunohaematological study: direct antiglobulin test (DAT) was performed with a broad spectrum and monospecific antisera; eluate was performed according to Rubin’s method; indirect antiglobulin test with untreated and treated (ficin/papain) homologous red was performed with the patient’s serum.The DAT was positive for an autoAb of IgG class; the autoAb was present also in the patient’s serum and was detectable only with enzyme-treated RBC. The eluate, investigated using a broad panel of reagent RBCs, showed no specificity. Moreover, abdominal ultrasound sonography revealed a left ovarian cystis mass, confirmed by CT-scan and MR. A diagnosis of ovarian teratoma was made.We started therapy with prednisolone but with no response. Then, when the intravenous immunoglobulin therapy was added, the Hb level increased reaching 12 g/dL level and she underwent surgery to remove the ovarian teratoma and the appendix. The DAT remained weakly positive until 6 weeks after surgery,but two months later serological test became negative. The steroid therapy was gradually tapered and stopped three months after the surgical intervention. During this period, no transfusion with paked red cell was performed. Conclusion Glucocorticoids are the treatment of choice in AIHA, but in an ovarian neoplasias they are usually ineffective and the remission is linked to surgery; therefore early recognition of the teratoma is important, as only surgery excision is curative. In similar cases, intravenous immunoglobulins can be effectively associated with steroids to foster the recovery of hemoglobin level and allow the surgical excision without blood support.
A FURTHER CASE OF AUTOIMMUNE HEMOLYTIC ANEMA ASSOCIATED WITH AN OVARIAN TERATOMA / Coluzzi, S.; De Nicolò, M. C.; Giona, F.; Taralli, S.; Amendola, A.; Neri, A.; Girelli, G.. - In: BLOOD TRANSFUSION. - ISSN 1723-2007. - (2008). (Intervento presentato al convegno XXXVIII CONVEGNO NAZIONALE DI STUDI DI MEDICINA TRASFUSIONALE tenutosi a RIMINI).
A FURTHER CASE OF AUTOIMMUNE HEMOLYTIC ANEMA ASSOCIATED WITH AN OVARIAN TERATOMA
Coluzzi S.;Giona F.;Girelli G.
2008
Abstract
Background Ovarian teratoma is a common ovarian neoplasm. West-Watson and Young described the first case of acquired immune haemolytic anemia (AIHA) in association with an ovarian tumor. Since then there are only sporadic reports of this association, and AIHA as a manifestation of ovarian teratoma remains a rare paraneoplastic syndrome of uncertain pathogenesis. This case report describes the management of a young patient affected by a severe haemolytic anemia. Case report A 15 years-old female was admitted at our hospital because of persisting pallor and anemia fortuitously found by peripheral blood examination. At the time of admission the Hb level was 5.4g/dL, the red blood cell (RBC) count was 1.480x109/L; the reticulocyte count was 3.6x109/L; white blood cell count and platelets value were normal. The serum lactate dehydrogenase was 192 IU/L (70-190 IU/L), total bilirubin 0.56 mg/dL (0.4- 1.1mg/dL), indirect 0.1 (0.1-0.4 mg/dL), haptoglobin 60mg/dL (58-356 mg/dL) and Vitamin B12 345 pg/mL. Anti-nuclear and anti-doublestranded DNA antibodies were negative. Immunohaematological study: direct antiglobulin test (DAT) was performed with a broad spectrum and monospecific antisera; eluate was performed according to Rubin’s method; indirect antiglobulin test with untreated and treated (ficin/papain) homologous red was performed with the patient’s serum.The DAT was positive for an autoAb of IgG class; the autoAb was present also in the patient’s serum and was detectable only with enzyme-treated RBC. The eluate, investigated using a broad panel of reagent RBCs, showed no specificity. Moreover, abdominal ultrasound sonography revealed a left ovarian cystis mass, confirmed by CT-scan and MR. A diagnosis of ovarian teratoma was made.We started therapy with prednisolone but with no response. Then, when the intravenous immunoglobulin therapy was added, the Hb level increased reaching 12 g/dL level and she underwent surgery to remove the ovarian teratoma and the appendix. The DAT remained weakly positive until 6 weeks after surgery,but two months later serological test became negative. The steroid therapy was gradually tapered and stopped three months after the surgical intervention. During this period, no transfusion with paked red cell was performed. Conclusion Glucocorticoids are the treatment of choice in AIHA, but in an ovarian neoplasias they are usually ineffective and the remission is linked to surgery; therefore early recognition of the teratoma is important, as only surgery excision is curative. In similar cases, intravenous immunoglobulins can be effectively associated with steroids to foster the recovery of hemoglobin level and allow the surgical excision without blood support.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
