Details

Autor(en) / Beteiligte

• Touzot, Fabien, MD, PhD
• Moshous, Despina, MD, PhD
• Cros, Guilhem, MD
• Frange, Pierre, MD, PhD
• Chomton, Maryline, MD
• Frémond, Marie-Louise, MD
• Neven, Bénédicte, MD, PhD
• Cavazzana, Marina, MD, PhD
• Fischer, Alain, MD, PhD
• Blanche, Stéphane, MD
• Helley, Dominique, MD, PhD

Titel

Circulating endothelial cells as markers of endothelial dysfunction during hematopoietic stem cell transplantation for pediatric primary immunodeficiency

Ist Teil von

• Journal of allergy and clinical immunology, 2014-11, Vol.134 (5), p.1203-1206

Ort / Verlag

United States: Elsevier Inc

Erscheinungsjahr

2014

Quelle

Elektronische Zeitschriftenbibliothek - Freely accessible e-journals

Beschreibungen/Notizen

• The CEC count was persistently higher at day 30 in patients with VE (median = 98/mL; range: 1-548) as compared to patients without VE (median = 4/mL; range: 2-52; P = .002) (Fig 1, A). [...]the increase of CEC counts was significantly higher in patients with VE during the first month post-transplant (median δD0-D30 = 92; range: 16.5-547; P = .007) as compared to patients without (Fig 1, B).  No vascular event (n = 23) Vascular event (n = 11) Total patients (n = 34) Sex ratio [female]/[male] 9/14 3/8 12/22 Age: median (range) 3 y (0.5-14 y) 0.9 y (0.25-9 y) 3 and 5 y (0.25-14 y) Diagnosis    SCID 6 2 8 CID 8 5 13 HLH 5 3 8 Neutrophil disorders 3 1 5 Donor    Matched sibling donor 7 0 7 MUD 7 5 12 MMUD 4 2 6 Haploidentical donor 5 4 9 Defibrotide conditioning regimen 8 9 17 Bu/Flu/ATG 16 6 22 Bu/Flu/ThioTEPA/ATG 6 5 11 Mel/Flu/Alemtuzumab 1 0 1 Graft characteristics    In vitro T-depletion 5 6 11 No in vitro T-depletion 18 5 23 CD34 cell dose (106/kg) 8.2 (1.4-24.2) 13 (5-24.2) 10.5 (1.4-24.2) Engraftment day 19 (9-49) 18.5 (13-31) 19 (9-49) Rejection 2 1 3 GVHD 8 3 11 Grade 1-2 6 1 7 Grade 3-4 2 2 4 Time onset (days) 16.5 (2-60) 27 (10-47) 17 (2-60) CMV systemic replication 4 3 7 Severe sepsis 2 1 3 Duration of hospitalization post-HSCT (days) 78 (38-212) 92 (56-155) 83 (38-212) Death 3 5 8 Table I Hematopoietic transplantation characteristics For CD34 number, engraftment, time of onset of GVHD, and duration of post-HSCT hospitalization, median time is indicated with range in brackets.ATG, Antithymocyte globulin; Bu, busulfan; CID, combined immunodeficiency; CMV, cytomegalovirus; Flu, fludarabine; HLH, hemophagocytic lymphohistiocytosis; Mel, Melphalan; MMUD, mismatched unrelated donor; MUD, matched unrelated donor; SCID, severe combined immunodeficiency.