ABSTRACT
INTRODUCTION: Haemophilia treatment varies significantly between individuals, countries and regions and details of bleed rates, factor consumption and injection frequency are often not available. AIM: To provide an overview of the FVIII/FIX treatment practice and outcome for patients with haemophilia A (HA) or haemophilia B (HB) across Europe. METHODS: Non-interventional, 12-month retrospective study where anonymized data were retrieved from haemophilia centres/registers in Belgium, France, Germany, Italy, Spain, Sweden and the United Kingdom. Male patients (all ages) receiving coagulation factor treatment 24 months prior to the study, with basal FVIII/FIX levels ≤5 IU dL-1 , without inhibitors, were included. Data were summarized descriptively. RESULTS: In total, 1346 patients with HA and 312 with HB were included in the analysis; 75% and 57% had severe disease (FVIII/FIX < 1 IU dL-1 ) respectively. Prophylaxis was most common for severe haemophilia, especially for children, whereas on-demand treatment was more common for moderate haemophilia in most countries. The mean (SD) prescribed prophylactic treatment ranged from 67.9 (30.4) to 108.4 (78.1) (HA) and 32.3 (10.2) to 97.7 (32.1) (HB) IU kg-1 per week, across countries. Most patients on prophylaxis were treated ≥3 times/week (HA) or two times/week (HB). The median annual bleeding rate (ABR) for patients on prophylaxis ranged from 1.0 to 4.0 for severe HA, and from 1.0 to 6.0 for severe HB, while those with moderate haemophilia generally had slightly higher ABRs. Median ABRs for on-demand-treated severe HA ranged from 4.5 to 18.0, and for HB, 1.5 to 14.0. CONCLUSION: Treatment practice varied greatly between centres and countries and patients treated on-demand and prophylactically both experienced bleeds, emphasizing the need for further optimization of care.
Subject(s)
Hemophilia A/therapy , Adult , Europe , Humans , Male , Retrospective StudiesSubject(s)
Erythroblastosis, Fetal/prevention & control , Pregnancy Complications/therapy , Prenatal Care/statistics & numerical data , Rh Isoimmunization/therapy , Rho(D) Immune Globulin/therapeutic use , Drug Utilization/statistics & numerical data , Europe , Female , Humans , Infant, Newborn , Practice Guidelines as Topic , Practice Patterns, Physicians'/statistics & numerical data , Pregnancy , Pregnancy Complications/immunology , United StatesABSTRACT
PURPOSE: The purpose of this study is to demonstrate that flow cytometric measurement of DNA content and cell proliferation is useful and provides significant information about the diagnosis and prognosis of non Hodgkin lymphoma. PATIENTS AND METHODS: Clinical and histologic information of 66 lymphoma patients was registered at diagnosis. Fifty thick sections of lymphoma samples and 67 control samples were deparaffined, rehydrated, dissected and DNA stained using a modification of the method described by Hedley. We have studied the relationship between flow cytometric results and clinical, histologic, treatment outcome and prognosis of lymphoma. RESULTS AND CONCLUSIONS: No DNA aneuploid histograms were obtained from the control non neoplastic, lymphoid samples. Side scatter light values were significantly larger in high grade lymphomas. DNA content didn't show any correlation with clinical presentation and evolution of lymphoma, however, DNA aneuploidy was more frequent in high grade lymphomas (p = 0.0172), and in these patients, aneuploidy was related to with a lower percentage of complete remission achievement (p = 0.0248). A high S phase was associated with shorter survival in patients without remission post-treatment (p < 0.0001) and in low grade lymphomas (p = 0.0174). In conclusion, our data suggest that DNA flow cytometric study provides useful information for the diagnosis of lymphoma and identifies different prognostic groups of patients.
