Awa Oumar Touré

Antiphospholipid antibodies and systemic scleroderma.

Objective: Antiphospholipid antibodies (APLs) could be associated with an increased risk of vascular pathologies in systemic scleroderma. The aim of our study was to search for APLs in patients affected by systemic scleroderma and to evaluate their involvement in the clinical manifestations of this disease. Materials and Methods: We conducted a cross-sectional descriptive study, from January 2009 until August 2010, with patients received at the Department of Dermatology (Dakar, Senegal). Blood samples were taken at the hematology laboratory and were analyzed for the presence of APLs. Results: Forty patients were recruited. Various types of either isolated or associated APLs were found in 23 patients, i.e. 57.5% of the study population. The most frequently encountered antibody was IgG anti-β2 GPI (37.5% of the patients), followed by anticardiolipins (17.5%) and lupus anticoagulants (5%). No statistically significant association of positive antiphospholipid-related tests to any of the scleroderma complications could be demonstrated. Conclusion: A high proportion of patients showing association of systemic scleroderma and APLs suggests the presence of a morbid correlation between these 2 pathologies. It would be useful to follow a cohort of patients affected by systemic scleroderma in order to monitor vascular complications following confirmation of the presence of antiphospholipid syndrome. Conflict of interest:None declared.

Rosai Dorfman disease diagnosed by fine‐needle aspiration cytology in a young man with HIV infection

RDD (Rosai Dorfman disease) is a rare and benign histiocytic proliferative disorder of unknown etiology. FNAC (Fine‐needle aspiration cytology) is a useful and reliable tool for the diagnosis of RDD, and as such, biopsy is avoidable.

Molecular diagnosis of haemophilia A in patients from Senegal

M. SECK,* C. COSTA,† B. F. FAYE,* D. SY BAH,* S. A. TOUR E,* N. DIENG,* A. SALL,* M. GADJI ,* A. O. TOUR E,* D. LASNE,‡ C. ROTHSCHILD§ and S. DIOP* *Hematology Cheikh Anta Diop University, Dakar, Senegal; †Laboratory of Genetic and Molecular Biology Cochin Hospital, APHP; ‡Laboratory of Hemostasis, Hôpital Universitaire Necker-Enfants Malades, AP-HP; and §Haemophilia Treatment Centre, Department of Haematology, Hôpital Universitaire Necker-Enfants Malades, AP-HP, Paris, France

Challenges in the management of sickle cell disease during pregnancy in Senegal, West Africa

ABSTRACT Objectives: The aim of this study was to evaluate the maternal and fetal complications in pregnant patients with sickle cell disease (SCD) and find risk factors of stillbirth. Method: We conducted a prospective study in pregnant women with SCD. Demographic characteristics, maternal and fetal morbi-mortality, and outcome of pregnancies were described. Risk factors of fetal loss were evaluated by comparing the parameters of the pregnancies that led to a live birth with those interrupted. Results: We included 70 pregnancies in 58 women with SCD. The average age was 29.3 years. The average gestational age at the start of follow-up was 13 weeks. The occurrence of acute complications was significantly higher during pregnancy compared to the year before (p < 0.05). Maternal mortality was 0%. Live birth rate was 80%. Fetal loss rate was 3.9 times higher in previous pregnancies that had not been monitored in hematology (71.8 versus 18.6%). Stillbirth was associated with nulliparity, high leukocytes or platelet counts (p < 0.05). Conclusion: Pregnancy in SCD was associated with a high maternal morbidity and stillbirth. Nulliparity, high leucokocytes or platelet count were identified as risk factors of fetal loss.

Bleeding risk assessment in hemophilia A carriers from Dakar, Senegal

&NA; Hemophilia A carriers have an abnormal X chromosome with a molecular abnormality of FVIII gene. These carriers, long considered to be free of bleeding risk, could have the same symptoms as mild hemophiliacs. This study aim to assess bleeding risk of hemophilia A carriers monitored at the Clinical Hematology Department of Dakar. This is a prospective study of a period of 6 months including 22 hemophilia A carriers aged between 8 and 48 years. Hemophilia carriers were recruited using the genealogical tree of hemophiliacs followed in the service. Their diagnosis was carried out by long range PCR and Sanger sequencing method searching the molecular abnormality responsible for hemophilia in their family. Bleeding risk was determined using a questionnaire consisting of different bleeding symptoms quoted from −1 to 4 according to the severity. Total of different values allow to determine the bleeding score which was pathological if it was greater than or equal to 1. Medium age was 22.5 years (8–48) (SD = 9.28). Four hemophilia A carriers (18.1%) presented bleeding symptoms and had a bleeding score at least 1 (P = 0.02). Menorrhagia was predominant (13.6%) followed by epistaxis (9%), gingivorrhagia (9%), and prolonged bleeding after tooth extraction (9%). Factor VIII level was lower in hemophilia carriers who presented bleeding (42 ± 8.61 UI/l) versus hemophilia carriers without bleeding (100 ± 50.95 UI/l) (P = 0.001). There was no significant correlation between bleeding occurrence and age (P = 0.81), activated patial thromboplastin time value (P = 0.97) and FVIII/Von Willebrand Factor ratio (P = 0.12). One in five hemophilia carriers presented bleeding and the questionnaire was effective to identify hemophilia carriers who had a risk of bleeding.