Altered immunophenotypic features of peripheral blood platelets in myelodysplastic syndromes.

BACKGROUND: Multiparameter flow cytometric analysis of bone marrow and peripheral blood cells has proven to be of help in the diagnostic workup of myelodysplastic syndromes. However, the usefulness of flow cytometry for the detection of megakaryocytic and platelet dysplasia has not yet been investigated. The aim of this pilot study was to evaluate by flow cytometry the diagnostic and prognostic value of platelet dysplasia in myelodysplastic syndromes. DESIGN AND METHODS: We investigated the pattern of expression of distinct surface glycoproteins on peripheral blood platelets from a series of 44 myelodysplastic syndrome patients, 20 healthy subjects and 19 patients with platelet alterations associated to disease conditions other than myelodysplastic syndromes. Quantitative expression of CD31, CD34, CD36, CD41a, CD41b, CD42a, CD42b and CD61 glycoproteins together with the PAC-1, CD62-P, fibrinogen and CD63 platelet activation-associated markers and platelet light scatter properties were systematically evaluated. RESULTS: Overall, flow cytometry identified multiple immunophenotypic abnormalities on platelets of myelodysplastic syndrome patients, including altered light scatter characteristics, over-and under expression of specific platelet glycoproteins and asynchronous expression of CD34; decreased expression of CD36 (n = 5), CD42a (n = 1) and CD61 (n = 2), together with reactivity for CD34 (n = 1) were only observed among myelodysplastic syndrome cases, while other alterations were also found in other platelet disorders. Based on the overall platelet alterations detected for each patient, an immunophenotypic score was built which identified a subgroup of myelodysplastic syndrome patients with a high rate of moderate to severe alterations (score>1.5; n = 16) who more frequently showed thrombocytopenia, megakaryocytic dysplasia and high-risk disease, together with a shorter overall survival. CONCLUSIONS: Our results show the presence of altered phenotypes by flow cytometry on platelets from around half of the myelodysplastic syndrome patients studied. If confirmed in larger series of patients, these findings may help refine the diagnostic and prognostic assessment of this group of disorders.

Large-volume leukapheresis for peripheral blood progenitor cell collection in low body weight pediatric patients: a single center experience.

Peripheral blood progenitor cells (PBPC) have became the preferred source of stem cells for autologous transplantation because of easier accessibility, rapid engraftment, and lower tumor cell contamination. In pediatric patients is very important to optimize peripheral blood stem cells (PBSC) harvesting to obtain a sufficient number of cells with a reduced number of leukapheresis. In this study we prospectively analyzed data on 43 large volume leukapheresis (LVL) from 20 consecutive low body weight pediatric patients with various malignancies. Patients' mean body weight was 16.6 kg (range, 8.9-32.0 kg), and the median age was 4 years (range, 1-10 y ears). Instead of saline, it was used irradiated and leukoreduced red blood cell (RBC) units to prime the machine in 15 patients weighting 25 kg or less. The median number of LVL was 2 (range, 1-4) and a mean of 5.2 patient's blood volume was processed per session lasting 165 min (range, 118-239). The mean number of CD34+ cells, one day before leukapheresis was 49 mm(-3) (range, 9-219). The PBPC collection yielded 24.7 x 10(8) total nucleated cells/kg (range, 6.2-74.0), 10.7 x 10(6) kg(-1) CD34+ cells (range, 3.6-53.7); 49.8 x 10(4) CFU-GM/kg (range, 6.4-198.1), and 65.6 x 10(4) BFU-E/kg (range, 7.6-198.1). The platelet count decreased significantly after each procedure 39.8 +/- 9.1 x 10(9) mm(-3) (range, 18.000-76.000) (p < 0.001). In conclusion, our data show that LVL for collection of PBPC in low weight pediatric patients is a safe and efficient procedure, but it may expose the patient to the risk of thrombocytopenia.