[Diagnosis and treatment of aquired aplastic anemia].
OBJECTIVE
To analyse the results of diagnosis and treatment of patients with aquired aplastic anemia (AA) in one center.


METHODS
All AA patients, diagnosed and treated in one clinic in 1998-2005, were included in the trial. In severe and very severe AA (SAA/VSAA) the patients (n = 19) received combined immunosuppressive therapy (IST) with antithymocytic globulin (ATG) and cyclosporin A (CsA), in non-severe AA (NSAA) the patients (n = 9) were given monotherapy with CsA. Allogenic transplantation of bone marrow (alloTBM) was made in 4 young patients with SAA/VSAA.


RESULTS
The diagnosis of AA was established in 33 patients (19 males and 14 females): NSAA in 9, SAA in 19, VSAA in 5, idiopathic--in 26, posthepatic--in 5, associated with pregnancy--in 2 patients. Age median was 20 years (13-53). The clone of paroxysmal nocturnal hemoglobinuria (PNH) was identified in 7 of 33 patients (21%), antigen HLA-DRB1 *15 in 6 of 11 patients (55%). In median of 26-month follow-up 31 patients (94%) were alive. In IST, complete or partial remissions were obtained in 88% patients. Median of the interval to achievement of transfusion independence made up 2.5 months. All the patients after alloTBM are in complete remission, chronic extensive transplant against host reaction was observed in one case.


CONCLUSIONS
Introduction of updated protocols provides long-term survival of more than 80% AA patients. To optimize treatment outcomes, it is necessary to include newly diagnosed AA patients into ongoing multicenter studies.
Kulagin
A D
AD
Lisukov
I A
IA
Kriuchkova
I V
IV
Sizikova
S A
SA
Gilevich
A V
AV
Denisova
V V
VV
Filimonov
P N
PN
Meniaeva
E V
EV
Pronkina
N V
NV
Kozhevnikov
V S
VS
Chernykh
E R
ER
Kozlov
V A
VA
D000741:Anemia, Aplastic / Q000175:diagnosis* / Q000188:drug therapy / Q000628:therapy*; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D016026:Bone Marrow Transplantation; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007165:Immunosuppression Therapy; D007166:Immunosuppressive Agents; D008297:Male; D016896:Treatment Outcome
D000961:Antilymphocyte Serum; D007166:Immunosuppressive Agents; D016572:Cyclosporine