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Factor XI (FXI) deficiency is a rare inherited bleeding disorder that is highly prevalent in Ashkenazi Jewish ancestry but sporadically observed in most ethnic groups. It is heterogeneous both in clinical presentation and in genetic causality. Although a large spectrum of mutations associated with this disorder has been reported in several populations, genetic data of FXI deficiency in Tunisia are poorly described. The purpose of this study was to determine the molecular basis of FXI deficiency among Tunisian patients. Fourteen index cases from nine unrelated families with FXI deficiency, referred to Hemophilia Treatment Center of Aziza Othmana Hospital, were included in this study. The patients' F11 genes were amplified by PCR and subjected to direct DNA sequencing analysis. Sequencing analysis of F11 genes identified three distinct mutations; the Jewish type II nonsense mutation E117X, one previously reported missense mutation E602Q and one novel missense mutation V271M, which led to the disruption of the third apple domain structure of FXI. Furthermore, seven polymorphisms previously described, were also detected: C321F, c. 294AG, -138 AC, p.D125D, p.T249T, p.G379G, p.D551D. This report represents the first genetic study analyzing the molecular characteristics of factor XI deficiency within Tunisian population. Identification of the Jewish type II mutation in two families, as well as one missense previously reported mutation and one novel mutation confirmed the genetic heterogeneity of this disorder. Screening a large number of Tunisian factor XI deficient would reveal the spectrum mutations causing factor XI deficiency in Tunisia.

Congenital factor VII (FVII) deficiency is an autosomal recessive bleeding disorder characterized by a weak phenotypic and genotypic correlation. This study aimed to determine the genetic alterations of 40 Tunisian patients and to evaluate their relationships with the collected clinical and biological data. Forty FVII-deficient Tunisian patients have been included in this study. First, diagnosis of the FVII deficiency was made on the basis of FVII coagulant activity (FVII:c) levels performed using the prothrombin time assay. Then, clinical and anamnesis data were set up and filed out from the regional registry of bleeding disorders and the medical file of each patient. Finally, genetic alterations were determined by direct sequencing of the coding regions, intron/exons boundaries of the F7 gene. Clinical heterogeneity was noticed, and the direct sequencing allowed the identification of 13 F7 gene mutations of which one was a novel mutation. The clinical manifestations are variably associated with FVII activity FVII:c levels. Lack of relations between severity of clinical manifestations and genotypes was observed; however, a relationship between the nonpathogeneous mutations and clinical phenotypes was noticed. A wide phenotypic inter-individual variability was detected, which suggests the presence of other extra-genetic components influencing the expressivity of the deficiency.

Prophylaxis has become standard of care for persons with severe phenotype haemophilia (PWsH). However, 'standard prophylaxis' with either factor or non-factor therapies (emicizumab) is prohibitively expensive for much of the world. We sought to evaluate whether haemophilia care can be provided at a lower cost yet achieve good results using Lower dose/Lower frequency prophylaxis (LDP) and with increasing use of antifibrinolytics (Tranexamic acid and Epsilon amino caproic acid).We identified 12 studies that collectively included 335 PWsH using LDP. Additionally, we undertook a literature search regarding the benefits of antifibrinolytics in haemophilia care.Identified studies show that LDP is far superior to no prophylaxis (On demand [OD] therapy) resulting in significant patient benefits. Patients on LDP showed (in comparison to patients OD) on average: 72% less total bleeds; 75% less joint bleeds; 91% less days lost from school; 77% less hospital admission days; and improved quality of life measures. These benefits come at similar or only slightly higher ( 2-fold greater) costs than OD therapy. Antifibrinolytics are effective adjunctive agents in managing bleeds (oral, nasal, intracranial, possibly other) and providing haemostasis for surgeries (particularly oral surgeries). Antifibrinolytics can substitute for more expensive factor concentrates or can reduce the use of such concentrates. There is evidence to show that antifibrinolytics may be used in conjunction with factor concentrates/emicizumab for more effective/less costly prophylaxis.The use of LDP along with appropriate and increased use of antifibrinolytics offers less resourced countries good options for managing patients with haemophilia.

Factor XI deficiency is a rare coagulation disorder with variable bleeding manifestations.To evaluate the correlation between the degree of factorXI deficiency and the clinical expression of the disease.Retrospective study, spanning 10 years from January 1, 2010 to December 31, 2019, concerning patients followed at the Hemophilia Center at Aziza Othmana Hospital in Tunis. The data were collected from the medical records. The determination of PT, APTT, fibrinogen level and coagulation factors are performed by coagulometric technique on STA® compact / ACL TOP®. FactorXI deficiency was confirmed on two different samples. Statistical analysis of the clinical-biological correlation was performed using the chi-square test. The significance level was 0.05.Twenty patients were collected. The mean age of discovery was 25 years with a sex ratio (M/F) =0.33. The circumstances of discovery were incidental in 14 patients. A family history of bleeding was reported in 30% of cases. Eight patients underwent surgery, six of whom had a simple postoperative course. The APTT was prolonged and isolated in 75% of cases. The hemostasis test was normal in 5 cases. The average FactorXI level was 24%. The tendency to bleed did not seem to be correlated with FactorXI levels.Prospective multicenter studies including molecular study would be necessary to better elucidate this rare disorder.

Heavy menstrual bleeding (HMB) is the commonest bleeding symptom among women with inherited bleeding disorders (IBD). Since HMB starts at the very onset of menarche and continues throughout the reproductive life, the health related quality of life of these women is affected and they are at an increased risk of developing iron-deficiency anemia. Because of the entrenched stigma and taboos, women and girls are often reluctant to discuss the problem of HMB within their families and do not seek medical advice. Increased awareness and multidisciplinary management approach for the management of these women are essential in ensuring an optimal outcome. It is important to take a careful history and undertake a thorough gynecological assessment to exclude other underlying/concomitant causes of HMB. Iron supplementation is essential. Strategies for decreasing menstrual blood flow are similar to those used for HMB in general with the addition of desmopressin and replacement therapy and the exclusion of non-steroidal anti-inflammatory drugs. Tranexamic acid and/or hormonal intervention are usually recommended as first-line therapy. Treatment choice should be individualized taking into account whether the woman wishes to preserve her fertility, if she requires contraception, the type of IBD, the severity of bleeding, and her social and religious background as well as acceptability and availability of the treatment options.

Factor V deficiency is a rare hemostatic disorder. It may present with a diverse spectrum of symptoms due to a variety of mechanisms including development of autoantibodies associated with a number of conditions. We report a first case of factor V deficiency in Tunisian hemodialysis patient due to an autoantibody most likely secondary to antibiotic exposure responsible for an arteriovenous shunt thrombosis rather than bleeding. We discuss here the clinical and biological features of acquired factor V inhibitor and provide a short review of the current literature.

Factor VII (FVII) deficiency is the most common among all rare inherited bleeding disorders. However, acquired FVII deficiency (aFVIID) is uncommon. Only few cases in the literature have been reported. Herein, we present a case of an aFVIID associated with acute myeloid leukemia (AML), along with a literature review regarding this condition. A 50 year old Arab male patient was diagnosed with AML at the hematology department of our institution. At admission, coagulation tests showed a prolonged prothrombin time (PT) with a normal activated partial thromboplastin time (aPTT) and a slightly elevated fibrinogen level. Prothrombin complex coagulation factors dosing (PCCFD) revealed a decrease only in FVII levels. The patient, however, did not experience any bleeding. The evolution of the health of the patient was marked by a normalization of PT and FVII levels and complete remission.

BACKGROUND: Plasma-derived von Willebrand factor (VWF) (Wilfactin(®), LFB, France) was developed for prophylaxis and treatment of haemorrhages in both adults and adolescents with von Willebrand disease (VWD). Replacement therapy in paediatric patients is a key element of the clinical trial programme. MATERIAL AND METHODS: Patients aged

Flow cytometry is the gold standard for the detection of paroxysmal nocturnal hemoglobinuria (PNH) clones. Several antibody panels have been used with varying sensitivities and specificities. The CD157 is one of the glycosylphosphatidylinositol-anchored molecules tested and widely used. The CD157 deficiency is rare. We report a case of an isolated CD157 deficiency discovered during the search for the PNH clone in a patient with a plastic anemia. The interpretation of the results in this case poses a problem of false positive. We discuss how to deal with these difficulties encountered by the biologist, detailing the various possible causes. This observation also underlines the importance of following international guidelines before making the diagnosis of the PNH clone which has significant implications.

Prophylaxis is the gold standard treatment for haemophilia but requires more amount of clotting factor concentrates, than on demand therapy. Low dose prophylaxis is an alternative for countries with limited resources. There are data of evidence showing the superiority of low-dose prophylaxis than episodic treatment. Studies from China, India, Tunisia, Thailand and Indonesia reported experiences with low dose prophylaxis using outcome assessment. These studies have shown the effectiveness of various protocols regimen with once, twice or thrice injection of 10-15 UI Kg-1 per injection. These protocols allow reduction of joint bleeds and at least delay of joint damages. There is not enough long-term data nowadays, but low dose prophylaxis is certainly better than on demand therapy and should be considered as a first step of prophylaxis in some countries but not the final goal.

Background: The Glanzmann’s thrombasthenia (GT) is a rare autosomal-recessive bleeding disorder with uncommon neonatal revelation. It is due to abnormalities of quantitative and/or qualitative α IIb β 3 integrin. This cell adhesion receptor is essential for platelet aggregation and allows the formation of a hemostatic plug if the vessel is damaged by injury. The clinical picture of GT is variable, with mucocutaneous bleeding due to non-functional platelets. Management requires a good expertise in bleeding disorders. We describe the clinical and the epidemiological data of GT in Aziza Othmana Hospital Hemophilia Center. Methods: This was a retrospective study of all patients with GT monitored and treated in our hemophilia center during the period of 2011 - 2015. Results: Twenty-seven patients among the 35 patients included in our hemophilia center registry were studied. The most common sign encountered is the gingival bleeding. In our women cohort, one completed her pregnancy. The consanguinity is present with a frequency of 62%. Treatments used depending on the case are tranexamic acid, platelet transfusion, packed red blood cells and rFVIIa, respectively. Conclusion: GT is relatively frequent in Tunisia and especially in the North of the country which can be explained by the high consanguinity in our population. J Hematol. 2017;6(2-3):44-48 doi: https://doi.org/10.14740/jh330e

Background Replacement donors are considered as having a major risk of transmission of infections to recipients mainly by the World Health Organisation. Study design and methods Seroprevalency of HBV, HCV, HIV and syphilis were determined in 19,783 whole blood donations collected in the Tunisian National Blood Transfusion Centre during the year 2010 (12,968 [65.55%] replacement donations and 6815 [34.44%] voluntary blood donations). For HBV, HCV and syphilis, we performed a univariate analysis to determine whether age, sex and type of donation were risk factors, then multivariate logistic regression, to see if these factors were independent. Results Mean age of donors was 30.1 years (replacement donors 34.5 years, first time non-remunerated donors 34.5 years, P

ResumeObjectif Evaluer le taux d’immunisation anti-erythrocytaire et anti-HLA dans les hemoglobinopathies. Patients et methodes Etude transversale (octobre 2009–mars 2010) concernant 83 patients suivis pour hemoglobinopathie. La recherche d’anticorps irreguliers est realisee par deux techniques : Coombs indirect et technique enzymatique sur carte gel. La recherche des anticorps anti-HLA de classe I est faite par la technique de lymphocytotoxicite complement dependante. Resultats L’âge moyen est de 30 ans (14–64 ans), le sex-ratio M/F est de 0,84. Il s’agit de 42 cas de syndromes drepanocytaires majeurs (29 drepanocytoses homozygotes et 13 drepano-thalassemies) et de 41 cas de syndromes thalassemiques (26 thalassemies majeures et 15 intermediaires). La frequence d’allo-immunisation anti-erythrocytaire est de 22,89 % sans difference entre les syndromes drepanocytaires et thalassemiques (7,31 %). La frequence d’auto-immunisation anti-erythrocytaire est de 24,8 %. Elle est plus elevee dans les syndromes thalassemiques (41,46 %) que dans les syndromes drepanocytaires (7,14 %) (p

La recherche des anticorps irreguliers (RAI) chez la femme enceinte est fondamentale. Le but etait d’analyser les RAI pour evaluer la prevalence de l’AI antierythrocytaire en milieu obstetrical. L’etude etait retrospective (09/2016-01/2019). Les renseignements cliniques recueillis : Groupe sanguin (GS) ABO RH1, terme gestationnel, antecedents obstetricaux et transfusionnels, injection d’immunoglobulines anti RH1. La RAI etait realisee par coombs indirect (CI) sur carte gel. Deux panels (depistage et identification) ont ete utilises. Le titrage des anticorps immuns etait fait par CI en microfiltration. L’analyse des donnees etait faite par SPSS. Trois milles six-cents soixante RAI (moyenne :1[1–7]) etaient realisees pour 3543 femmes. Quatre cent quatre-vingt six cas etaient documentes. Les GS etaient essentiellement : O (49 %). 71 % etaient RH1 positif. Les RAI etaient realisees au 2e trimestre dans 35 % des cas. 37 % des femmes etaient nullipares. Les antecedents obstetricaux releves etaient avortement (13 %), toxemie gravidique (9 %), mort fœtale in utero (3 %). La transfusion etait notee chez 1 % des femmes. La RAI etait positive dans 35 cas (1 %) identifiant 40 anticorps (Ac) ou associations d’Ac : 15 allo-Ac (anti RH1 :2 cas, anti RH1 + anti RH2 :2 cas, antiRH3 + anti RH4 :2 cas, anti RH3 :1cas, anti RH2 :1 cas, antiMNS 3 :1 cas, anti FY1 :1cas et un anti HTLA :1cas), 22 anti-RH1 passifs, 2 auto-Ac (IgG) et un Ac anti MNS1. Le titre d’anti-RH1variait de 16 a 2048 et celui de l’anti-MNS1 etait de 64. Une transfusion in utero a ete realisee dans 2 cas. L’AI antiRH1est relativement faible, neanmoins l’identification d’autres Ac incite la mise en place d’un calendrier bien definie de surveillance obstetricale en Tunisie.

Resume En Tunisie, la transfusion de globules rouges s’inscrit dans un cadre reglementaire mais reste sujette a des defaillances en raison de la meconnaissance de la legislation et des regles de securite. L’objectif de notre travail est d’evaluer les connaissances des medecins en matiere de securite immunologique en transfusion erythrocytaire avant et apres une lecture d’un CD-ROM d’auto-enseignement. Il s’agit d’une evaluation d’une intervention. Quatre-vingts medecins hospitaliers ont repondu a un questionnaire anonyme, comportant sept questions a choix multiple (QCM) de 29 items. Le taux de bonnes reponses (TBR) calcule par question et par item a pris en compte l’impact du CD-ROM sur l’amelioration des reponses apres lecture. La note globale moyenne est de 2,9/7. Les reponses par questions sont variables (25 % a 76 %). Tous les participants ont repondu juste a plus de 50 % des items. Deux participants ont repondu correctement a tous les items. Parmi les participants, 31,3 % ont repondu correctement aux cinq items « importants » concernant les regles de compatibilite dans le systeme ABO et l’epreuve ultime au lit du malade. Quatre-vingt-trois pour cent ont visionne le CD-ROM et accepte de participer a l’evaluation finale. L’impact du CD-ROM a ete statistiquement significatif. La note globale a augmente de 2,9 a 5,8/7, 31,5 % ont repondu correctement a toutes les questions (vs 2 %) et 95,5 % ont repondu correctement aux cinq items importants (vs 31,3 %). Ce travail a revele une meconnaissance des medecins en matiere de securite immunologique erythrocytaire. Le CD-ROM a significativement ameliore les reponses. L’instauration d’une formation continue est imperative dans notre pays.

Introduction Low dose prophylaxis could be recommended in countries with limited resources. Aim We report our single centre experience in children with haemophilia. Patients Fifty-five children were included in our study with a weekly median dose of 30 UI kg−1 given once, twice or thrice a week. Age of initiation of prophylaxis is 5.32 years (0.64–11.44). Outcome assessment used were number of bleeding before and after initiating prophylaxis, haemophilia joint health score (HJHS), functional independence score in haemophilia (FISH) and quality of life with the Haemo-QoL. Results Reduction of number of bleeding was clear in all patients; HJHS, FISH and Haemo-QOL were satisfactory. Conclusion Low dose prophylaxis is effective and better than on-demand therapy. It should be the starting point for prophylaxis in countries with limited resources.

Von Willebrand's disease (VWD) is the most commonly inherited bleeding disorder. It is characterized by clinical, biological and molecular heterogeneity. In certain types of the disease, diagnosis can be difficult.We report the clinico-biological characteristics of VWD's patients and analyze diagnosis difficulties.33 cases were diagnosed in the laboratoryfrom February to May, 2011. Screening hemostasis included the measuring ofC, VWF: Ag and VWF: RCo. Blood cell count and blood group were performed in all cases.Mean age at diagnosis is 13 years [10 months -43 years]. The sex ratio M/F is 0.5. The patients are classified type 3 VWD in 52% of the cases, type 2 VWD in 30 % of the cases and type 1 VWD in 18 % of the cases. The diagnosis of type 2B VWD suspected in combination of the ratio VWF:RCo / VWF: Ag0,7 and thrombocytopenia in one case. Required tests for positive diagnosis and distinction between the primary categories of VWD are available. Specialized tests will allow a best characterization variants type 2 VWD for a better therapeutic approach.

Resume Objectif La determination avec precision de la lignee cellulaire au cours de la leucemie aigue (LA) est une etape cruciale dans le diagnostic et la conduite therapeutique ulterieure. En Tunisie, faute de cytometrie de flux (CMF), certaines LA continuent d’etre traitees sur la base de la cytologie seule. Notre objectif est de confronter les resultats de la cytologie a ceux de l’immunophenotypage et d’analyser les degres de discordance. Patients et methodes L’etude concerne 100 cas de LA. Elle consiste a effectuer une deuxieme lecture a posteriori en double insu de lames de moelle de LA deja diagnostiquees par deux cytologistes differents et de confronter aux resultats de l’immunophenotypage. Resultats Deux cas classes cytologiquement en LAM ont ete reclasses a l’immunophenotypage en LAL T et leucemie aigue biphenotypique. Trois cas classes cytologiquement LAL ont ete reclasses leucemies aigues indifferenciees (2 cas) et leucemie aigue biphenotypique (1 cas). Quatre cas non identifies a la cytologie ont ete classes LAL B (3 cas), leucemie aigue biphenotypique (1 cas). Dans les deux cas de discordance entre les deux cytologistes, la cytometrie de flux a permis de retenir le diagnostic de leucemie aigue biphenotypique dans un cas et de LAM dans l’autre. Conclusion L’etude cytologique demeure insuffisante dans le diagnostic de lignee meme avec des cytologistes experimentes. L’immunophenotypage est un examen incontournable pour confirmer ou redresser le diagnostic de LA.

Summary. Combined factor V (FV) and factor VIII (FVIII) deficiency (F5F8D) is a rare autosomal recessive disorder caused by mutations in LMAN1 or MCFD2 genes which encode proteins that form a complex involved in the transport of FV and FVIII from the endoplasmic reticulum to Golgi apparatus. We report two novel mutations in MCFD2 gene and one recurrent mutation in LMAN1 gene that caused combined FV and FVIII deficiency in two unrelated Tunisian Muslim families. For the first family two patients were homozygous for a new missense mutation Asp81His in exon 3 of MCFD2 and heterozygous for a second new missense mutation Val100Asp in the same exon. Replacement respectively of the hydrophilic Asp residue with hydrophobic positively charged His and of the hydrophobic neutral Val residue with the Asp residue most likely disrupts the MCFD2–LMAN1 interaction, thus leading to the disease phenotype. For the second family a reported Arg202X mutation in exon 5 in the LMAN1 gene was identified in the homozygous state.

Acute promyelocytic leukemia (APL) has now become the most curable of all subtypes of acute myeloid leukemia. A cure rate of 75-80% can be anticipated with a combination of all-trans retinoic acid (ATRA) and anthracyclines. In Tunisia, the ATRA era began in 1998 with the use, consecutively, of two regimens of a combination of ATRA with anthracycline and cytarabine (APL93), and without cytarabine (LPA99). From 2004, 39 patients with confirmed APL either by t(15;17) or PML/RARA were treated by the PETHEMA LPA 99 trial. The rationale of this protocol by avoiding cytarabine is to reduce death in complete remission (CR) without increasing the incidence of relapse. Thirty-three patients achieved CR (84.6%). The remaining six patients were considered as failure due to early death: three caused by differentiation syndrome (DS) and three died from central nervous system hemorrhage. Baseline blood cell count (WBC) >10 x 10(9)/l (P=0.26) and creatinine >1.4 mg/dl (P=0.42) were not predictive of mortality. DS was observed in 11 patients (30.5%) with a median onset time of 12 days (range: 3-23 days) and median WBC of 29 x 10(9)/L (range: 1.2 x 10(9)-82.7 x 10(9)/l). DS was severe in seven cases, moderate in four, and fatal in three cases. Body mass index > or =30 (P=0.044) and baseline WBC > or =20 x 10(9)/l (P=0.025) are independent predictors of DS. The median follow-up of this study is 36 months. Thirty patients are alive in continuous complete remission; two patients died in CR from septic shock and secondary myelodysplastic syndrome respectively; one patient died 47 months after achieving two relapses. Event free survival from diagnosis was 80% and overall survival was 82%. Our results are quite acceptable and can be improved by reducing mortality rate.

Auteur(s) : Wijden El Borgi1, Emna Gouider1, Nour Louati1, Nawel Ben Salah1, Halima Senana2, Ramzi Jeddi3, Ali Saad2, Raouf Hafsia1 1Service d’hematologie biologique, Hopital Aziza Othmana, Tunis 2Service de cytogenetique, Hopital Farhat Hached, Sousse 3Service d’hematologie clinique, Hopital Aziza Othmana, Tunis, Tunisie L’hemophagocytose par les cellules blastiques est rarement observee au cours des leucemies aigues (LA) myeloides, elle est surtout rapportee dans les [...]

The combination of all-trans-retinoic acid (ATRA) and chemotherapy has made acute promyelocytic leukemia (APL) a highly curable leukemia. However, several complications are reported with this treatment the most serious and life threatening being Retinoic Acid Syndrome (RAS). We aimed at identifying factors that could predict complications caused by ATRA during induction treatment of APL.Forty-two patients with confirmed APL (by t(15;17) and/or PML/RARA) treated at our institution (University hospital of Tunis) between January 1998 and June 2006 using two consecutive protocols: European APL93 trial (24 patients) until February 2004 and Spanish PETHEMA LPA99 trial (18 patients) more recently. Induction regimen consisted of ATRA 45 mg/m(2)/d until CR combined to DNR 60 mg/m(2)/d x 3+Cytarabine 200 mg/m(2)/d x 7 (APL93) and Idarubicin 12 mg/m(2) d2, 4, 6, 8 (LPA99). Prednisone (0.5 mg/kg d1-d15) was added if WBC10 x 10(9)/L to prevent RAS in LPA 99.Median age was 36 yr (7-64 yr), M/F=16/26 (0.61), median WBC was 2.4 x 10(9)/L (range 0.6-100 x 10(9)/L). WBC10 x 10(9)/L was noted in 14 patients (33%). Additional cytogenetic abnormalities were seen in 12/42 (28%). Median body mass index (BMI=weight/height(2):N 20-25) was 24 kg/m(2) (range 16-40 kg/m(2)), BMI30 was noted in nine patients (8F and 1M). Thirty-three patients achieved CR (78.57%):18/24 (75%) in APL93 versus 15/18 (83%) in LPA99. Nine patients (21.42%) had early death. Causes of early death were: RAS (6) and CNS hemorrhage (3). Complications due to ATRA were: RAS (10), Scrotal ulcerations (3), Sweet syndrome (2), Perineal ulcerations (1), and Pseudotumor cerebri (1). Prognostic factors for complications of ATRA (Fisher exact test) were: BMI35 (p=0.055), induction treatment without cytarabine (LPA99 trial) (p=0.047), whereas age (p=0.74), gender (p=0.51), initial WBC (p=0.47), and additional cytogenetic abnormalities (p=0.83) were not predictive. Retinoic Acid Syndrome was more reported in patients with initial WBC10 x 10(9)/L (p=0.08).We found high BMI (35) in female and treatment without Cytarabine to increase the risk of developing complications with ATRA.

Despite legislative acts develloped, many deficiencies were identified in blood requests at the National Blood TransfusionCenter impedding board and blood safety.to evaluate the conformity of the different topics of packed red blood cells requests to the legislation.Our study was prospective descriptive lasting six months (March-August 2011). It assessed all packed red blood cells requests which reached the national blood transfusion center.16064 packed red blood cells requests from 21 public institutions and 28 private institutions were studied. There was different deficiencies in each item.The absence of birth date in 67.18% of request represented the largest non-compliance within administrative information. A predominance of shortcomings related to transfusion and obstetric history was recorded for clinical information with absence of date of the last transfusion in 91.72% cases, lack of accuracy of any previous transfusion reactions in 88.63% cases and absence of the number of previous pregnancies in 93.15% of transfusion requests prescribed to women. Non-conformities related to the prescribing physician concerned mainly the phone number which was absent in 55.82% of cases.This study revealed a significant lack of awareness of physicians in relation to the law governing transfusion. It is therefore essential to develop training for prescribers to improve transfusion safety.

Background: Replacement donors are considered as having a major risk of transmission of infections to recipients mainly by the World Health Organisation. Study design and methods: Seroprevalency of HBV, HCV, HIV and syphilis were determined in 19,783 whole blood donations collected in the Tunisian National Blood Transfusion Center during the year 2010 (12,968 (65.55%) replacement donations and 6815 (34.44%) voluntary blood donations). For HBV, HCV and syphilis, we performed a univariate analysis to determine whether age, sex and type of donation were risk factors, then multivariate logistic regression, to see if these factors were independent. Results: Mean age of donors was 30.1 years (Replacement donors 34.5 years, First Time Non Remunerated Donors 34.5 years, p

Summary Some progress has been made regarding availability of recombinant factor VIII concentrates and prophylaxis for haemophilia A in emerging countries, where plasma-derived concentrates were used in the vast majority. Clinical studies to document their introduction and effectiveness are so far not widely available in literature. This non-interventional study evaluates the real-life effectiveness and safety of prophylactic and on-demand treatment with recombinant factor VIII formulated with sucrose (rFVIII-FS) for bleed control and preservation of joints in emerging countries from Eastern Europe, North Africa and Middle East area. One hundred and eighty-six patients from 11 countries were enrolled, mean ± SD age 12.8 ± 12.7 years. At enrolment, majority (79.6%) had severe haemophilia A (

International audience; The aims of this study are to determine seroprevalence of Hepatitis E virus (HEV) in Tunisian blood donors and to evaluate its risk of parenteral transmission. Sera collected from 426 blood donors were tested for HEV IgG by indirect ELISA. Individuals were recruited from two national transfusion centers, in the North and the South of the country. Seroprevalence of HEV IgG was then compared with two other groups with increased risk of exposure to parenterally transmitted agents: 80 hemophiliac and 286 hemodialysis patients. Among blood donors, the seroprevalence was estimated to be 4.5%. It was significantly higher in the hemophiliac and hemodialysis groups with 7.5% and 10.2%, respectively, (P = 0.002). No significant correlation was observed for this IgG 1 seroprevalence between age and sex among three studied groups. These results suggest that HEV has a high risk of parenteral transmission and confirm that the low endemicity of hepatitis E in Tunisia was observed.

We report two cases of atypical defibrination syndromes in patients with respectively acute monoblastic leukemia (chronic myeloid leukemia initially) and acute lymphoblastic leukemia. Hemostasis studies show low fibrinogen level, elevated D-dimers, decreased alpha 2 antiplasmin and factor V, normal antithrombin III values. Plasminogen is below the normal range in one patient. Soluble complexes, which are an important argument for diagnosis of intravascular coagulation disease, are not detected in both patients. Primary or secondary hyperfibrinolysis seems also excluded since euglobulin clot lysis time was normal. Enzymatic proteolysis of fibrinogen (or fibrin) by the blast cells has been reported by some authors; this mechanism could account for the hemostasis abnormalities observed in these two patients.

Les leucemies aigues lymphoblastiques (LAL) sont des hemopathies malignes se definissant par l'envahissement de la moelle par une population lymphoide immature et monoclonale. L'etude de la maladie residuelle (MRD) au cours de ces hemopathies permet d'evaluer la reponse therapeutique. L'objectif de notre travail est l'evaluation de la masse tumorale residuelle a la fin du traitement d'induction (J35). Cette etude porte sur 59 patients suivis au service d'hematologie de l'Hopital Aziza Othmana. A J35, l'estimation d'une remission complete est basee sur une etude cytologique. Elle est definie par un taux de blastes < 5 %. L'etude immunophenotypique a objective 42 cas de LAL B et 17 cas de LAL T. La MRD est < 10 -4 dans 29 % des cas seulement. Ce taux reste inferieur a celui rapporte dans les differentes series de la litterature (60-70%). L'immunophenotypage permet de quantifier les blastes non detectables en cytologie, d'evaluer la masse tumorale residuelle et d'adapter les protocoles therapeutiques. La cytometrie en flux est une technique simple, rapide, tres sensible et moins couteuse que la biologie moleculaire dans l'evaluation de la maladie residuelle et permet ainsi un suivi therapeutique rigoureux des patients.

Immunophenotyping is a major tool for the diagnosis of the chronic lymphoid leukaemia (CLL). Its interest remains limited in the classification of the other B chronic lymphoproliferative syndromes (B-CLPS). We evaluate the place of the flow cytometry (CMF) in the diagnosis and classification of the non CLL B-CLPS. The cases with Matutes score of 4 or more are excluded. A confrontation of the results to the histology is made. 28 cases of non CLL B-CLPS are diagnosed. CMF shows a κ monoclonal population in 15 cases and λ in 13 cases. A co-expression CD19+CD5 + is found in 11 cases concording with an atypic CLL or a mantel cell lymphoma in 6 cases with Matutes score of 3. In 5 cases, we concluded to non CLL B-CLPS (Matutes

Menstruations, by their abundance and their duration, can be a source of impaired quality of life. Women with inherited bleeding disorders appear to be, specially at risk.Assess the impact of menstrual blood loss on the quality of life for women with inherited bleeding disorders.31 women with various inherited bleeding disorders were interviewed. They completed a quality of life questionnaire.Von Willebrand disease was the most frequent inherited bleeding disorder in our population (38.7%). 54.8% of patients had a menstrual period more than 6 days 61.3% of them consider their menstrual flow to be normal. The general condition apart of the menstrual period was considered medium to poor in 35.5% of patients. The average score assessing the impact of menstruation on daily life was of 5.00 ± 3.47. Only 19.35% of patients felt that dysmenorrhea significantly affect their quality of life. Impaired quality of life was seen in 64.5% of patients according to score Aand in 41.9% of them according to score B. During menstruation 22.6% of the patients didn't do to work or to school because of the menstrual flow. On the other hand, 48.4% of patients were hospitalized at least once for a heavy menstrual flow.The quality of life during menstruation, in women with an inherited bleeding disorder, according to the different scores appear altered. Although because of the small size of our study population, we could not prove correlation between the importance of menstrual blood loss and the impairment of quality of life.

Immunophenotyping is an essential step in the diagnosis of acute myeloid leukemia. Its prognostic value remains controversial with contradictory results.To assess prognostic impact of the immunophenotyping in AML.Our study is retrospective (October, 2005 - July, 2007) concerning 56 cases of AML (AML3 excluded) of the adult from 18 to 55 years old diagnosed and treated in Tunis Aziza Othmana Hospital. The immunophenotyping was performed by flow cytometry (Beckman Coulter EPICS XL MCL®). We studied clinical and biological characteristic, immunophenotypic expressions, and parameters of the response to the treatment: complete remission (CR), overall survival (OS), relapse free survival (RFS) and relapse in a delay of 1 year and 2 years. SPSS software was used to perform the statistical analysis.The median age of the patients is of 37,7±11.8 years. Sex ratio (M/F) is 1.33. Among individual antigenic expressions, only CD7 is associated to lower CR rates (p=0.044). We did not find any statistically significant association between immunophenotypic expressions and OS nor with relapse or RFS.The impact of immunophenotyping in AML remains controversial because of contradictory results. The research of molecular changes would be an interesting alternative in our context.