Author/Editor     Preložnik-Zupan, Irena; Pretnar, Jože; Zver, Samo; Fikfak, Nataša
Title     Zdravljenje avtoimunskih citopenij po alogenični presaditvi krvotvornih matičnih celic - prikaz dveh primerov
Translated title     Treatment of severe autoimmune cytopenias after allogeneic stem cell transplantation - report of two cases
Type     članek
Source     Zdrav Vestn
Vol. and No.     Letnik 73, št. Suppl 1
Publication year     2004
Volume     str. I-51-4
Language     slo
Abstract     Background. Autoimmune cytopenias, thrombocytopenia, anemia and neutropenia, are rare but serious complications after stem cell transplantation (SCT). There are only a few reports concerning their treatment. We performed splenectomy in two patients with severe autoimmune cytopenias after allogeneic SCT resistant to immunosuppressive treatment. Patients, methods and results. First patient had unrelated alloSCT at Royal Free Hospital London for chronic granulocytic leukemia (CGL) in July 2000. Post-transplant period was complicated with cytomegalovirus reactivation and septicemia. Seven months later RBC and platelet counts went dawn. Direct Coomb's test was intermittently positive. She was resistant to steroids and high dose immunoglobulin. Splenectomy was performed in February 2001. After splenectomy hemoglobin concentration and platelet count improved. Her blood counts remained stable with hemoglobin about 110 g/L and platelets over 100 x 10 9/L. She continued therapy with Itraconazol, Valacyclovir and Penicillin. Three months later she was readmitted for E. Coli fulminated septic infection with fatal outcome. Second patient had related alloSCT at University Medical Centre Ljubljana for CGL in January 2003. Past-transplant course was uneventful. Seven months later he was readmitted for retinal bleeding with severe thrombocytopenia with positive anti-platelet antibodies. He was resistant to steroids and high dose inamunoglobulin. Splenectomy was performed in September 2003. His platelet count normalized and remains stable so far. He continues therapy with Itraconazol, Valacyclovir and Penicillin and didn't experience any serious infection. Conclusions. We assume that splenectomy is an efficient treatment for resistant immune cytopenias after alloSCT. However, severe late infections may compromise the outcome.
Summary     Izhodišča. Kot redek zaplet po presaditvi krvotvornih matičnih celic (PKMC) se lahko pojavijo avtoimunske citopenije: trombocitopenija, anemija in nevtropenija. V literaturi je malo poročil o njihovem zdravljenju. Po neuspešnem imunosupresivnem zdravljenju smo opravili splenektomijo pri dveh bolnikih s hudo avtoimunsko citopenijo po alogenični PKMC. Bolniki, metode in rezultati. Prva bolnica je bila zdravljena z nesorodno alogenično PKMC zaradi kronične mieloične levkemije (KML) v Royal Free Hospital v Londonu julija 2000. V obdobju po presaditvi je imela težave zaradi reaktivacije citomegalovirusa in sepse. Po sedmih mesecih so še vedno opažali nizke vrednosti eritrocitov in trombocitov v periferni krvi. Direkten Coombsov test je bil občasno pozitiven. Zdravljenje z glukokortikoidi in imunoglobulini ni bilo učinkovito. Februarja 2001 so opravili splenektomijo, po kateri se je povečala koncentracija hemoglobina v krvi in število trombocitov. Vrednosti hemoglobina so bile 110 g/L in število trombocitov nad 200 x 10 9/L. Bolnica je nadaljevala zdravljenje z itrakonazolom, valaciklovirjem in penicilinom. Devet mesecev po splenektomiji je bila ponovno sprejeta v bolnišnico zaradi okužbe z E. Coli, zaradi katere je bolnica tudi umrla. Drugega bolnika smo zdravili s sorodno alogenično PKNLC zaradi KML v Kliničnem centru v Ljubljani v januarju 2003. V obdobju po presaditvi ni imel večjih težav. Po sedmih mesecih se je pojavila krvavitev v očesno ozadje. V krvni sliki smo ugotovili hudo trombocitopenijo. Trombocitna protitelesa so bila pozitivna. Zdravljenje z glukokortikoidi in imunoglobulini je bilo neučinkovito. Septembra 2003 smo opravili splenektomijo, po kateri se je število trombocitov normaliziralo. Nadaljuje zdravljenje z itrakonazolom, valaciklovirjem in penicilinom. Do sedaj ni imel resnejše okužbe. (Izvleček skrajšan pri 2000 znakih).
Descriptors     HEMATOPOIETIC STEM CELL TRANSPLANTATION
THROMBOCYTOPENIA
ANEMIA, HEMOLYTIC
NEUTROPENIA
SPLENECTOMY
PLATELET COUNT