Background: Hereditary spherocytosis (HS) is the most common cause of inherited chronic hemolysis.The various tests developed for HS screening have many shortcomings. The purpose of this study was to develop a new, simple, reliable test using flow cytometry.Methods: In this new test, deionized water, a hemolysis-inducing agent, is spiked to a red cell suspension during acquisition, and the count of red cells is measured sequentially in real-time using flow cytometry. In this study, the optimal parameters for this test were established, and the discriminatory power of the adopted protocol was verified among several groups with various degrees of osmotic fragility (OF).Results: The established protocol successfully discriminated between blood analyzed immediately and blood stored for 24 h (P < 0.00001). With healthy individuals as a reference, the OF measured by this protocol was significantly decreased in patients with iron deficiency anemia (P = 0.030) and was significantly increased in HS patients (P = 1.1 × 10 29 ). This new test found all 11 HS patients to have increased OF.Conclusions: This new test was verified to be simple, quantitative, objective, and cost-effective for the measurement of OF. We suggest this test as another effective approach for HS screening, although its assay performance will require further verification through more clinical trials. q 2008 Clinical Cytometry
MMF would appear to be effective and safe for treating refractory chronic GVHD, yet not as effective for treating refractory acute gut GVHD. Accordingly, a prospective randomized clinical trial is warranted to assess the impact of MMF in the treatment of refractory GVHD.
Summary:The current study defines the incidence and clinical manifestations of hyperacute graft-versus-host disease (haGVHD; fever, skin rash, diarrhea, and hepatic dysfunction) and analyzes the risk factor and the impact of haGVHD on the results of allogeneic stem cell transplantation (SCT). In all, 90 patients underwent allogeneic SCT from 71 matched siblings or 19 alternative donors. Immediate high-dose steroids were administered to 22 patients who met the criteria. The overall incidence of haGVHD was 36.7% (n ¼ 34) and haGVHD was also strongly correlated with acute (aGVHD) (Po0.001) and extensive chronic GVHD (cGVHD) (P ¼ 0.007), and found to be associated with decreased probability of relapse (P ¼ 0.0017). Early intervention with steroids within 7 days after the diagnosis of haGVHD might be associated with better survival. A survival analysis of the overall survival and disease-free survival did not reveal any difference between haGVHD þ and haGVHDÀ groups. In multivariate analysis, the use of an alternative donor (P ¼ 0.020) was identified as the only risk factor. Immediate high-dose steroids were effective in treating haGVHD. We conclude that in an allogeneic setting, haGVHD is not an uncommon manifestation, associated with the development of aGVHD or cGVHD. The only risk factor for haGVHD was the use of an alternative donor.
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