Author/Editor		Preložnik-Zupan, Irena; Zver, Samo; Pretnar, Jože
Title		Huda oblika akutne reakcije presadka proti gostitelju (aGVHD) po alogenični presaditvi krvotvornih matičnih celic
Translated title		Severe (grade III-IV) acute graft versus host disease after allogeneic haematopoietic stem cell transplantation
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 71, št. 9
Publication year		2002
Volume		str. 539-41
Language		slo
Abstract		Background. Beside greater susceptibility to infections, acute graft host disease is a consequence of the activation of donor T-cells against host antigens. Most common target organs are skin, liver and intestinal mucosis. December 2000, 49 patients were treated with allogeneic haematopoietic stem cell transplantation (allo-HSCT) in Transplant unit, Department of Hematology, Clinical Centre Ljubljana. The standard GVHD prophylaxis regimen consisted of cyclosporine and short-course methotrexate. Severe, grade 111-N aGVHD with skin and/or gastrointestinal and/or liver involvement appeared in 16 (32%) of the 49 patients. Results. Among the 16 patients with severe aGVHD, 14 had liver involvement, ten gastrointestinal and eight skin involvement. One patient had skin involvement only, the rest of them had combined involvement of two or three organ systems. Routine first-line treatment for aGVHD, given to all 16 pts with severe forms of the disease, was methylprednisolone (MP) 2mg/ kg. Six patients with predominant skin involvement responded to MP. Other ten patients with mainly liver and gastrointestinal involvement needed second or even third line aGVHD treatment. These were anti-thymocyte globulin (ATG) and/or monoclonal antibodies (OKT3) and/or mycophenolate mofetil (MMF) and/or FK506(tacrolimus). Seven patients died of advanced aGVHD and treatment related infection. Conclusions. Based on our experiences, we conclude that in critically ill patients with severe aGVHD, neutropenia and high risk for opportunistic infection, each day of ineffective MP therapy may have fatal consequences. Simultaneous institution of a combination of corticosteroids and a second-line drug mightprove more appropriate forpatients with a severe form of aGVHD.
Summary		Izhodišča. Akutna reakcija presadka proti gostiteju (aGVHD-acute graft versus host disease) je poleg zvečane dovzetnosti za okužbe glavni zaplet alogenične presaditve krvotvornih matičnih celic (alo-PKMC). Nastane kot posledica aktivacije darovalčevih limfocitov proti prejemnikovim antigenom in prizadene najpogosteje kožo, jetra in črevesno sluznico. Metode. V 6-letnem obdobju, med januarjem 1995 in decembrom 2000, smo v enoti za PKMC, Kliničnega oddelka za hematologijo, Kliničnega centra v Ljubljani opravili 49 aloPKMC. Izvajali smo standardno profilakso GVHD s ciklosporinom in kratkimi odmerki metotreksata. Hudo aGVHD, stadij 111-IV s prizadetostjo kože in/ali prebavnega trakta in/ali jeter, smo opazovali pri 16 (32%) bolnikih. Rezultati. Od 16 bolnikov s hudo aGVHD smo pri 14 bolnikih ugotovili prizadetost jeter, pri 10 bolnikih prizadetost prebavne cevi in pri 8 bolnikih prizadetost kože. Pri enem bolniku je bila prizadeta le koža, preostali pa so imeli prizadeta dva ali tri organske sisteme. Zdravilo prve izbire za zdravljenje aGVHD pri vseh 16 bolnikih je bil metilprednizolon (MP) v odmerku 2 mg/kg. Pri 6 bolnikih s pretežno prizadetostjo kože je prišlo do izboljšanja. 10 bolnikov s prizadetostjo jeter in prebave je potrebovalo zdravilo druge ali celo tretje izbire. Zdravili smo s protitimocitnim globulinom (ATG) in/ali monoklonskimi protitelesi (OKT3) in/ali mikofenolat mofetilom (MMF) in/ali FKSOG (takrolimus). Sedem bolnikov je umrlo zaradi napredovale aGVHD in/ali okužbe, povezane z zdravljenjem. Zaključki. Na osnovi izkušenj menimo, da je pri bolnikih s hudo obliko aGVHD, hudo nevtropenijo in veliko nevarnostjo za oportunistične okužbe vsak dan zdravljenja le zMP izgubljen. Istočasna uvedba MP in zdravila druge izbire bi bila morda primernejša odločitev za zdravljenje bolnikov s hudo aGVHD.
Descriptors		HEMATOPOIETIC STEM CELL TRANSPLANTATION GRAFT VS HOST DISEASE TRANSPLANTATION, HOMOLOGOUS TRANSPLANTATION CONDITIONING