Glucocorticoid-resistant Evans' syndrome successfully controlled with low-dose cyclosporine.

OBJECTIVE: To present a patient suffering from Evans' syndrome (ES), whose bouts of severe cytopenia were prevented by low-dose cyclosporine maintenance therapy. CASE SUMMARY: A boy suffering from frequent mild respiratory infections, first time evaluated in a tertiary care pediatric center at age 4, was found to have lymphadenopathy and mild splenomegaly. The thrombocytopenia was first noted at age 6. He was diagnosed to have ES at the age of 8, during another bout of thrombocytopenia, this time associated with Coombs-positive hemolytic anemia. Immunoglobulin concentration in the plasma was measured repeatedly, and was in the normal range, or even increased. Lymphocyte subpopulation numbers were in the normal range, with decreased CD4+/ CD8+ ratio (0.6). Autoimmune lymphoproliferative syndrome was excluded by the absence of CD4-CD8- T lymphocytes. Since the patient failed to respond to standard therapy with prednisolon 2 mg/kg, high dose intravenous methylprednisolone (10 mg/ kg/d for 3 days) and high dose intravenous immunoglobulin (1 g/kg/d for 2 days), cyclosporine treatment was initiated (6 mg/ kg/d) and resulted in normalization of platelet count and resolution of hemolysis. Two attempts to withdraw cyclosporine therapy resulted in life-threatening hemolytic crisis with severe thrombocytopenia, requiring the re-institution of cyclosporine. The dose of cyclosporine was eventually tapered to the present 0.5 mg/kg, corresponding to drug serum levels of 5 - 8 mg/ml. The patient is now free of manifestations of Evans' syndrome but, after 20 years of cyclosporine treatment, has slightly impaired kidney function. CONCLUSION: Low-dose cyclosporine therapy given to our patient appears to have subdued the autoimmune process thought to underlie the manifestations of ES, albeit at the cost of some toxicity to the kidneys.

Clinical features of the most common fusion genes in childhood acute lymphoblastic leukemia.

Contemporary protocols ensure high-remission rate and long-term free survival in children with acute lymphoblastic leukemia (ALL), but small percentage of patients is still incurable. Molecular genetic methods helped to establish submicroscopic classification as well as minimal residual disease follow-up, considered to be responsible for relapse. Our study enrolled 70 pediatric patients with de novo ALL, analyzed using reverse transcriptase-polymerase chain reaction for the presence of four major risk-stratifying translocations (BCR/ABL, MLL/AF4, TEL/AML1, and E2A/PBX1). Bone marrow samples were collected at diagnosis, at the end of induction phase, and after intensive chemotherapy with the aim to establish the correlation between chromosomal aberration, clinical features, and treatment response. Presenting the results of this study, we offer another evidence of variable incidence and clinical characteristics of ALL subtypes.

Collection strategies and cryopreservation of umbilical cord blood.

The aim of this study was to compare (a) two different umbilical cord blood (UCB) collection methods while the placenta is still in the uterus (in utero), and (b) to evaluate the efficacy of four cryopreservation protocols based on UCB haematopoiestic stem cell (HSC) recovery. We analysed UCB samples collected with our original collection system designed for active Syringe/Flush/Syringe method or by standard in utero method. For comparing different cryopreservation procedures, dimethyl sulphoxide (DMSO) at final concentration of 5 and 10% was used and combined with our own controlled-rate or uncontrolled-rate cryopreservation. A total of 99 samples were collected. A significantly higher UCB volume, total nucleated cell and mononuclear cell were seen following the first collection strategy (n= 49; mean +/- SD, 103 +/- 35.4 mL; 12.34 +/- 5.27 x 10(8); 595 +/- 3.47 x 10(6)) vs. the second strategy (n= 50; 86 +/- 29.3 mL; 9.87 +/- 4.47; 424 +/- 2.82 x 10(6)) respectively (P < 0.01). The discard rate was 14% for the first and 36% for the second collection strategy (P < 0.01). It was shown that the most efficient procedure was the controlled-rate protocol combined with lower (5%) DMSO concentration. Using active Syringe/Flush/Syringe method, we collected UCB with greater volumes and with lower discard rate compared to the standard by gravity technique. The data presented also showed much better recovery of UCB cells when controlled-rate freezing procedure and 5% DMSO were combined.