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Blood. 2017 Nov 16;130(20):2215-2223. doi: 10.1182/blood-2016-12-755777. Epub 2017 Sep 20.

Degree of anemia, indirect markers of hemolysis, and vascular complications of sickle cell disease in Africa.

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Department of Internal Medicine, Assistance Publique des Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Internal Medicine, Paris, France.
INSERM, UMR_S970, Paris Descartes University, Sorbonne Paris Cité, Paris, France.
INSERM UMR_S1134, Paris Diderot University, Sorbonne Paris Cité, Institut National de la Transfusion Sanguine, Paris, France.
Laboratoire d'Excellence sur le Globule Rouge, Sorbonne Paris Cité, Paris, France.
Department of Hematology, Centre Hospitalo-Universitaire de Yopougon, Hematology, Abidjan, Ivory Coast.
Centre de Recherche et de Lutte contre la Drépanocytose, Bamako, Mali.
Centre National de Transfusion Sanguine, Dakar, Senegal.
Department of Pediatry, Hôpital Pédiatrique Albert Royer, Pediatry, Dakar, Senegal.
Department of Hematology, Centre Pasteur du Cameroun, Yaoundé, Cameroon.
Department of Pediatry, Hôpital Laquintinie, Douala, Cameroon.
Department of Pediatry, Centre Hospitalier d'Essos, Yaoundé, Cameroon.
Department of Hematology, Hopital Central de Yaoundé, Yaoundé, Cameroon.
Department of Cardiology, Hôpital Mère Enfant le Luxembourg, Bamako, Mali.
Centre Biomédical d'Abidjan, Abidjan, Ivory Coast.
Department of Cardiology, Fondation Mère Enfant Chantal Biya, Yaoundé, Cameroon.
Centre Cardiologique d'Abidjan, Abidjan, Ivory Coast.
Department of Cardiology, Centre Hospitalo-Universitaire de Fann, Dakar, Senegal; and.
Department of Cardiology, Assistance Publique des Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France.


The hyperhemolysis paradigm that describes overlapping "hyperhemolytic-endothelial dysfunction" and "high hemoglobin-hyperviscous" subphenotypes of sickle cell disease (SCD) patients is based on North American studies. We performed a transversal study nested in the CADRE cohort to analyze the association between steady-state hemolysis and vascular complications of SCD among sub-Saharan African patients. In Mali, Cameroon, and Ivory Coast, 2407 SCD patients (1751 SS or sickle β-zero-thalassemia [Sβ0], 495 SC, and 161 sickle β+-thalassemia [Sβ+]), aged 3 years old and over, were included at steady state. Relative hemolytic intensity was estimated from a composite index derived from principal component analysis, which included bilirubin levels or clinical icterus, and lactate dehydrogenase levels. We assessed vascular complications (elevated tricuspid regurgitant jet velocity [TRV], microalbuminuria, leg ulcers, priapism, stroke, and osteonecrosis) by clinical examination, laboratory tests, and echocardiography. After adjustment for age, sex, country, and SCD phenotype, a low hemoglobin level was significantly associated with TRV and microalbuminuria in the whole population and with leg ulcers in SS-Sβ0 adults. A high hemolysis index was associated with microalbuminuria in the whole population and with elevated TRV, microalbuminuria, and leg ulcers in SS-Sβ0 adults, but these associations were no longer significant after adjustment for hemoglobin level. In conclusion, severe anemia at steady state in SCD patients living in West and Central Africa is associated with elevated TRV, microalbuminuria, and leg ulcers, but these vascular complications are not independently associated with indirect markers of increased hemolysis. Other mechanisms leading to anemia, including malnutrition and infectious diseases, may also play a role in the development of SCD vasculopathy.

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