Hydroxyurea is associated with later onset of acute splenic sequestration crisis in sickle cell disease: Lessons from the European Sickle Cell Disease Cohort-Hydroxyurea (ESCORT-HU) study

被引：8

作者：

Allali, Slimane ^{[1
]}

Galacteros, Frederic ^{[2
]}

Oevermann, Lena ^{[3
,4
]}

Cannas, Giovanna ^{[5
]}

Joseph, Laure ^{[6
]}

Loko, Gylna ^{[7
]}

Elenga, Narcisse ^{[8
]}

Benkerrou, Malika ^{[9
]}

Etienne-Julan, Maryse ^{[10
]}

Castex, Marie-Pierre ^{[11
]}

Brousse, Valentine ^{[1
,9
]}

de Montalembert, Mariane ^{[1
]}

机构：

[1] Univ Paris Cite, Necker Enfants Malad Hosp, Assistance Publ Hop Paris AP HP, Dept Gen Pediat & Pediat Infect Dis,Reference Ctr, Paris, France

[2] UPEC, Henri Mondor Univ Hosp, AP HP, Dept Internal Med,Sickle Cell Referral Ctr, Creteil, France

[3] Charite, Dept Pediat Oncol & Hematol, Berlin, Germany

[4] Berlin Inst Hlth, Berlin, Germany

[5] Hop Edouard Herriot, Hosp Civils Lyon, Ctr Reference Constitutif Syndromes Drepanocytair, Med Interne, Lyon, France

[6] Univ Paris Cite, Necker Enfants Malad Hosp, Assistance Publ Hop Paris AP HP, Biotherapy Dept, Paris, France

[7] Ctr Hosp Univ Martinique, Ctr Reference Constitutif Syndromes Drepanocytair, Le Lamentin, Martinique, France

[8] Ctr Hosp Cayenne, Ctr Reference Constitutif Syndromes Drepanocytair, Cayenne, French Guiana, France

[9] Univ Paris Cite, Hop Robert Debre, AP HP,Inserm,ECEVE, Serv Hematol Immunol,Ctr Reference MCGRE,UMR 1123, UMR-1123 ECEVE, Paris, France

[10] CHU Pointe A Pitre, Ctr Reference Malad Rares Drepanocytose Antilles, Unite Transversale Drepanocytose, Pointe A Pitre, Guadeloupe, France

[11] Toulouse Univ Hosp, Childrens Univ Hosp, Pediat Oncol Immunol Hematol Unit, Toulouse, France

来源：

AMERICAN JOURNAL OF HEMATOLOGY | 2024年 / 99卷 / 04期

关键词：

CHILDREN; ANEMIA; HYDROXYCARBAMIDE; THERAPY; SPLEEN; ADULT;

D O I：

10.1002/ajh.27214

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication of sickle cell disease (SCD), typically occurring in young patients under 5 years of age, with a median age at first episode of less than 2 years. Because a beneficial effect of hydroxyurea (HU) on spleen perfusion and splenic function has been suspected, we hypothesized that HU treatment might be associated with later onset of ASSC in patients with SCD. To investigate this hypothesis, we analyzed data from the ESCORT-HU study on a large cohort of patients with SCD receiving HU, enrolled between January 2009 and June 2017 with a follow-up of 7309 patient-years of observation. The median age at ASSC of the 14 patients who experienced a first episode of ASSC during the study period was 8.0 [IQR: 5.0-24.1] years. The median age at HU initiation was significantly lower in these 14 patients (4.8 [IQR: 3.3-18.7] years) compared to the 1664 patients without ASSC (19.9 [8.8-33.4] years, p = .0008). These findings suggest that ASSC may occur at an unusually late age in patients receiving HU, possibly reflecting longer preservation of spleen perfusion and function secondary to early initiation of HU. Further studies are needed to better characterize the effects of HU on spleen perfusion/function and on the occurrence of ASSC in patients with SCD

引用

页码：555 / 561

页数：7