Author/Editor		Mlakar, Uroš
Title		Zdravljenje avtoimunske hemolitične anemije z rituksimabom
Translated title		Treatment of autoimmune hemolytic anaemia with rituximab
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 73, št. Suppl 1
Publication year		2004
Volume		str. I-55-8
Language		slo
Abstract		Background. Autoimmune haemolytic anaemia (AIHA) is characterised by the presence of autoantibodies directed against autologous erythrocytes. Depending on temperature at which they are active AIHA is classified as AIHA due to warm or cold-reacting antibodies. Glucocorticoids are the mainstay of treatment for patients with AIHA due to warm autoantibodies. If haemolysis cannot be controlled by a low dose of glucocorticoids, then splenectomy is indicated. Immunosuppressive therapy with cytotoxic drugs is reserved for those patients who fail to respond to glucocorticoids and splenectomy or for those who are poor surgical risks. Cold agglutinin disease is a chronic haemolytic anaemia that is refractory to the usual treatments for haemolytic anaemia mediated by a warm-reactive antibody. Innovative approaches are needed for patients with AIHA that does not respond to conventional treatment. Rituximab is a monoclonal antibody against CD20 antigens on B cells. It has been introduced for the treatment of B-cell lymphomas. The successful treatments of refractory idiopathic cold agglutinin disease with rituximab were reported recently. Methods and results. In this paper we present three cases of chronic AIHA refractory to immunosuppressive treatment. Two patients were with cold agglutinin disease secondary to lymhoplasmacytic lymphoma. One of two had mixed cold and warm-reacting antibodies. The third case was with idiopathic AIHA due to warm-reacting antibodies. Treatment of secondary cold agglutinin disease with rituximab was unsuccessfully, while in patient with idiopathic warm-reacting AIHA remission was achieved. The patient has been without anaemia and without laboratory signs of hemolysis more then 10 months. Conclusions. Treatment with rituximab appears promising for idiopathic refractory AIHA. We suggest that combination of rituximab with chemotherapy in secondary cold agglutinin disease due to lymphoid neoplasm is better choice than rituximab alone.
Summary		Izhodišča. Glede na temperaturo, pri kateri se protitelesa vežejo na antigene eritrocitov, razlikujemo avtoimunsko hemolitično anemijo (AIHA) s toplimi protitelesi od AIHA s hladnimi protitelesi. AIHA s toplimi protitelesi zdravimo z glukokortikoidi. Če zdravljenje z njimi ni uspešno, ali je učinkoviti odmerek prevelik, prihaja v poštev splenektomija. Imunosupresivno zdravljenje s citostatiki je primerno za bolnike, pri katerih opisano zdravljenje ni bilo uspešno ali je tveganje za splenektomijo preveliko. Bolezen zaradi hladnih aglutininov (BHA) je kronična hemolitična anemija, pri kateri našteti načini zdravljenja navadno niso učinkoviti. Zato pri AIHA, kjer običajno imunosupresivno zdravljenje ni uspešno, poskušamo zdraviti z novimi zdravili. Rituksimab je monoklonsko protitelo proti antigenu CD20, ki se nahaja na imunskih celicah B. Uporablja se za zdravljenje limfatičnih novotvorb celic B. Nedavno so poročali o uspešnem zdravljenju z rituksimabom pri bolnikih z idiopatično BHA. Bolniki in metode. V prispevku prikazujemo tri bolnike s kronično AIHA, neodzivno na običajno imunosupresivno zdravljenje. Dva bolnika sta imela bolezen zaradi hladnih aglutininov. Bolezen je bila sekundarna v sklopu limfoplazmocitnega limfoma. Eden od omenjenih bolnikov je imel poleg hladnih aglutininov v krvi tudi topla protitelesa. Pri tretjem bolniku je bila prisotna kronična idiopatična AIHA s toplimi protitelesi. Zdravljenje sekundarne BHA z rituksimabom ni bilo uspešno. Pri bolniku z idiopatično AIHA s toplimi protitelesi smo z rituksimabom dosegli remisijo. Bolnik je že več kot deset mesecev brez anemije in brez laboratorijskih znakov za hemolizo. Zaključki. Rituksimab je obetajoče zdravilo za zdravljenje idiopatične AIHA, neodzivne na običajno imunosupresivno zdravljenje. Predpostavljamo, da je pri sekundarni BHA zaradi limfatične novotvorbe primernejši izbor kombinacija rituksimaba s kemoterapijo kot zdravljenje samo z rituksimabom.
Descriptors		ANEMIA, HEMOLYTIC, AUTOIMMUNE AUTOANTIBODIES ANTIBODIES, MONOCLONAL ANTIGENS, CD20 LYMPHOMA, SMALL LYMPHOCYTIC MIDDLE AGE TREATMENT OUTCOME