Journal Information
Vol. 42. Issue S2.
Pages 41 (November 2020)
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Vol. 42. Issue S2.
Pages 41 (November 2020)
DOI: 10.1016/j.htct.2020.10.067
Open Access
A.C.S. Pintoa, A.S. Araujob, S.F.M. Gualandroc, C.T. Buenod, R.D. Cançadoe
a Centro Regional de Hemoterapia de Ribeirão Preto, Faculdade de Medicina de Ribeirão Preto (FMRP), Universidade de São Paulo (USP), Ribeirão Preto, SP, Brazil
b Fundação de Hematologia e Hemoterapia de Pernambuco (Hemope), Recife, PE, Brazil
c Departamento de Hematologia, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), São Paulo, SP, Brazil
d Novartis Oncology, Brazil
e Departamento de Hematologia Oncológica, Faculdade de Ciências Médicas da Santa Casa de São Paulo (FCMSCSP), São Paulo, SP, Brazil
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Background and aims: Sickle cell disease (SCD) is a multi-system disorder, and vaso-occlusive crisis (VOC) is a hallmark of the disease commonly leading to end-organ damage. This study aims to determine the frequency of different complications among Brazilian SCD patients and compare these with data from other countries. Material and methods: SWAY was a multi-country survey of unmatched SCD patients and HCPs developed by international SCD experts, patient advocacy groups, and Novartis. It is a cross-sectional study, conducted in 16 countries (Bahrain; Brazil; Canada; France; Germany; Ghana; India; Italy; Lebanon; Netherlands; Nigeria; Oman; Panama; Saudi Arabia; United Kingdom; United States). SCD patients aged >6 years were recruited and answered a questionnaire that included sections on demographics, disease treatment and clinical characteristics (those aged 6-11 years old completed the questionnaire with a caregiver/parent/legal guardian proxy). Subjects also reported on SCD complications that they had ever experienced in their lives. The population for this analysis was stratified by age and according with the country. Results: The analysis included 260 patients from Brazil and 1,885 from other countries. Regarding hydroxyurea (HU) use, 68% of Brazilian patients reported having previous HU use vs 38% in other countries. The absence of complications was reported by 1% of Brazilian patients and 6% of those from other countries. Disease complications ever experienced that significantly differed (p<0.05) between Brazilian patients vs patients from other countries were: fever 85% vs 61%, infection 79% vs 51%, required blood transfusions 74% vs 39%, joint issues 70% vs 54%, swollen or painful fingers/toes 58% vs 32%, tight chest pain 55% vs 40%, gallbladder stones 43% vs 20%, issues/damage of the spleen 36% vs 19%, vision problems 31% vs 19%, stroke 13% vs 7%, issues/damage of the liver 13% vs 9%, issues/damage of the kidneys 13% vs 8% and priapism 10% vs 7%. The sample was further stratified by age, and a similar pattern of complications occurrence was observed in both Brazilian and other countries samples. Considering patients aged 6-16 years, the frequency of fever, infections, required blood transfusions and joint issues were 92%, 81%, 74% and 57% in Brazil vs 60%, 47%, 31% and 39% in other countries, respectively. Among those aged >16 years, the frequencies were 79%, 77%, 75% and 79% in Brazilian patients vs 62%, 53%, 43% and 61% in patients from other countries. Discussion: Brazilian patients showed significantly higher frequencies of complications compared to patients in other countries. Reasons to explain such findings need to be further addressed. In addition, a higher frequency of HU use is observed among Brazilian patients. However, it is not possible to assess the association between HU use and complications since this study was not designed to address this issue. Conclusion: Brazilian patients experience significantly more complications than patients from other countries. Thus, the need for treatment strategies able to improve disease management may be highlighted.

Hematology, Transfusion and Cell Therapy

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