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Fifty-four carbapenem non-susceptible Klebsiella pneumoniae (CNSKP) isolates were collected from a Tunisian hospital over a period of 13 consecutive months. Carbapenemase production and the prevalence of carbapenemase-encoding genes were investigated using combined-disk test (CDT), modified Carba NP (mCarba NP) test, and UV-spectrophotometry method complemented by PCR experiments and sequencing. Carbapenemase production was detected by the mCarba NP test and CDT in 92.59% and 96.29% of the 54 CNSKP isolates, respectively; while imipenem hydrolysis was detected using UV-spectrophotometry in the crude extracts of 44 isolates. blaNDM, blaOXA-48-like, and blaKPC carbapenemase-encoding genes were found in 48, 31, and 22 isolates, respectively. Remarkably, blaNDM-9, blaKPC-20, and blaKPC-26 genes were reported. The co-occurrence of carbapenemase-encoding genes in a single isolate was detected in 62.96% of the isolates. The analysis of clonal relationships between the isolates by pulsed field gel electrophoresis revealed that the majority of them were genetically unrelated. Our investigation provides molecular data on enzymatic mechanism of carbapenem non-susceptibility among 54 CNSKP showing the dominance of blaNDM, and comprises the first identification of blaNDM-9, blaKPC-20, and blaKPC-26 genes in a Tunisia hospital.

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Introduction: Laboratory medicine has an important role in the management of COVID-19. The aim of this study was to analyze routinely available blood parameters in intensive care unit COVID-19 patients and to evaluate their prognostic value. Patients and methods: This is a retrospective, observational, single-center study including consecutive severe COVID-19 patients who were admitted into the intensive care unit of Ben Arous Regional Hospital in Tunisia from 28 September 2020 to 31 May 2021. The end point of the study was either hospital discharge or in-hospital death. We defined two groups based on the outcome: survivors (Group 1) and non-survivors (Group 2). Demographical, clinical, and laboratory data on admission were collected and compared between the two groups. Univariate and multivariate logistic regression analysis were performed to determine the predictive factors for COVID-19 disease mortality. Results: A total of 150 patients were enrolled. Eighty patients (53.3%) died and 70 (46.7%) survived during the study period. Based on statistical analysis, median age, Simplified Acute Physiology Score (SAPS II) with the serum levels of urea, creatinine, total lactate dehydrogenase (LDH), creatine kinase, procalcitonin and hs-troponin I were significantly higher in non-survivors compared to survivors. On multivariate analysis, LDH activity ≥ 484 U/L (OR=17.979; 95%CI [1.119-2.040]; p = 0.09) and hs-troponin I ≥ 6.55 ng/L (OR=12.492; 95%CI [1.691-92.268]; p = 0.013) independently predicted COVID-19 related mortality. Conclusion: Total LDH and hs-troponin I were independent predictors of death. However, further clinical investigations with even larger number of patients are needed for the evaluation of other laboratory biomarkers which could aid in assessing the prediction of mortality.

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Docimology has allowed the development of evaluative processes assuring valid, reliable and objective assessments. It was adopted within the faculty of Medicine of Tunis since  2007. The aim of this study was to analyze the docimological survey results of hematology-oncology exams, to evaluate the interest of this analysis in the elaboration of exams and the construction of an item bank, and propose some corrections  in order to improve assessment.     Methods :We have analyzed the hematology-oncology exams of SCMS1 (Second cycle of Medical Studies 1) from educational year 2008-2009 to 2013-2014. The data input was already done with Excel. The test includes 4 disciplines (Hematology, Oncology, Genetics and the Anatomic Pathology). We have calculated docimological parameters allowing global analysis, by discipline and by item. Results : A total of 3281 papers and 1004 questions were analyzed. The average success rate per year was 91,54% ± 7,12. The highest average success rate was found in hematology (80,51% ± 10,18). The lowest rate was found in the anatomic pathology (51,61% ± 23,76). The average rate of students succeeding the test without having average note in hematology was 5,36%. It was 42,29% in the anatomic pathology. Average difficulty index was 0,57 ± 0,05. Items analysis showed that 38,04% were easy and 19,02% were difficult. Average discrimination index was 0,25 ± 0,02. Discrimination was very good in 20,51% of items and good in 17,13%. Useless and bad discrimination items were about 40,53%. The average of Cronbach Alpha coefficient was 0,84 ± 0,03, showing a good internal-consistency. Conclusion :This study allowed an objective evaluation of "contributive disciplines" in multidisciplinary evaluation and showed the interest of integrating questions. Question analysis with teachers would be important to reevaluate and improve these items.

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BACKGROUND: The spectrum of thalassemias is wide ranging from thalassemia minor, which consists of mild hypochromic microcytic anemia without obvious clinical manifestations, to thalassemia major (TM), which is characterized by severe anemia since the first years of life and is transfusion dependent. Thalassemia intermedia (TI) describes those patients with mild or moderate anemia. OBJECTIVE: To describe the genetic features and major clinical complications of TI, and the therapeutic approaches available in the management of this disease. METHODS: Publications from potentially relevant journals were searched on Medline. RESULTS AND DISCUSSION: Over the past decade, the understanding of TI has increased with regard to pathophysiology and molecular studies. It is now clear that clinical presentation and specific complications make TI different from TM. It is associated with greater morbidity, a wider spectrum of organ dysfunction and more complications than previously thought. CONCLUSION: TI is not a mild disease. The interplay of three hallmark pathophysiologic factors (ineffective erythropoiesis, chronic anemia, and iron overload) leads to the clinical presentations seen in TI. New treatment modalities are currently being investigated to broaden the options available for TI management.

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BACKGROUND: 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. AIM: We report the clinico-biological characteristics of VWD's patients and analyze diagnosis difficulties. METHODS: 33 cases were diagnosed in the laboratoryfrom February to May, 2011. Screening hemostasis included the measuring of FVIII: C, VWF: Ag and VWF: RCo. Blood cell count and blood group were performed in all cases. RESULTS: 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: Ag <0,7 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.

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BACKGROUND: Immunophenotyping is an essential step in the diagnosis of acute myeloid leukemia. Its prognostic value remains controversial with contradictory results. AIM: To assess prognostic impact of the immunophenotyping in AML. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

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In Tunisia, the ATRA era began in 1998 with the use, consecutively, of two regimens combining ATRA and an anthracycline with cytarabine (APL93), and without cytarabine (LPA99). From 2004, 51 patients with confirmed APL either by t(15;17) or PML/RARA were treated according to the PETHEMA LPA 99 trial. Forty three patients achieved CR (86%). The remaining seven patients had early death (one died before treatment onset): four caused by differentiation syndrome (DS) and three died from central nervous system hemorrhage. Multivariate analysis revealed that female gender (P=0.045), baseline WBC> 10 G/L (P=0.041) and serum creatinine > 1.4mg/dl (P=0.021) were predictive of mortality during induction. DS was observed in 16 patients (32%) after a median onset time of 15 days from treatment onset (range, 2-29). Body mass index ≥ 30 (P=0.01) remained independent predictor of DS. Occurrence of hypertensive peaks significantly predicted occurrence of DS (P=0.011) and was significantly associated with high BMI (p=0.003). With a median follow-up of 50 months, 5 year cumulative incidence of relapse, event free and overall survival were 4.7%, 74% and 78%, respectively.

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BACKGROUND: Sickle cell disease is an autosomal, recessive hemoglobinopathy characterized by hemolytic anemia. Red blood cell transfusions are uncommon therapeutic mainstay in sickle cell disease and repeated transfusions can result in iron overload. The predicted risks of iron overload and organ failure increase with both the duration of disease requiring transfusion therapy and the number of transfusions. AIM: To assess the state of iron overload in patients with sickle ce anemia according to their number of transfusions. METHODS: The medical records of 94 patients with sickle cell anemia (46 had homozygous sickle cell disease, 41 had sickle-ß thalassemia, 7 had compound heterozygous hemoglobin: 4 SC and 3 SOArab) were retrospectively reviewed for the following: clinical exam, serum ferritin level, liver function tests, abdominal ultrasound exam and heart Doppler. RESULTS: 61% of our patients are from the Northern- west of the country. The average age is 18.29 years (2 to 62 years) and the sexratio is 0.62. In addition to parental consanguinity which is found in 28.72% of the cases. The average level of ferritin is 660.35 ng/ml. 41.5% of the patients have a high status of ferritin witch ranged from 521.4 to 3360 ng/ml. There is not a significant difference of ferritin level according to age, sex and a phenotype of sickle cell anemia. However, it is higher among the transfused patients with a same phenotype (p<0.05). We found a correlation between serum ferritin levels and the number of transfusions (r =+0.74). Splenectomy has a preventive role because it allowed stopping the transfusion in 65% of the cases. The evaluation of organ dysfunction has found a hepatomegaly in 29% of the cases, half of witch were have a high status of serumferritin (> 1000 ng/ml). Left ventricular hypertrophy associated to valvulopathy was classified in 10 % of the cases. CONCLUSION: Iron overload in sickle cell anemia, though relying on transfusion, remains moderate. The repetitive assessment of serum ferritin level is considered as the best test though it does not evaluate an organic dysfunction. To evaluate them better, other tests are requiring: magnetic resonance imaging and Tc-Squid biosusceptometers.

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BACKGROUND: Thalassemia intermedia empasses a mild clinical and biological spectrum. The aim is to report the clinical and biological features and treatment of this disease. METHODS: It is a retrospective study about 36 thalassemia intermedia patients (17 males, 19 females). Epidemiological, haematological aspects and treatment were reported RESULTS: The diagnosis was carried out at a relatively old age 15 years (1-72).The thalassemia intermedia was characterized by mild facial deformities, splenomegaly and moderate anemia ( Hb = 9.1 g/dl). The mean serum ferritin was 518 ng/ml (25-1800). Three phenotypes are caracterised: heterozygosis beta thalassemia, beta degrees thalassemia and beta + thalassemia. Clinical complications were hypersplenism, extra medullary hematopoiesis, leg ulcers, thrombosis and pulmonary hypertension. Treatment was based on occasionally transfusion and splenectomy on event of hypersplenious (47%). Evolution of this disease was generally good with a long lifespan at 31 years (6-83). CONCLUSION: Thalassemia intermedia is well tolerated. Transfusions and splenectomy were indicated in case of hypersplenious.

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BACKGROUND: Splenectomy is frequently advised in hereditary hemolytic anemia. Severe complications could occur after splenectomy. AIM: To provide the indication and benefit of splenectomy METHODS: clinical and biological patterns were performed in a retrospective study of 82 patients: 17 homozygous beta thalassemia, 17 thalassemia intermedia, 33 heterozygote Hb/S beta thalassemia and 15 hereditary spherocytosis. RESULTS: Splenectomy was performed for: Hypertransfusion in homozygous thalassemia, hereditary spherocytosis; hypersplenism in Thalassemia intermedia and splenic sequestration in heterozygote HbS/beta thalassemia. The benefit of splenectomy was proved in hereditary spherocytosis (100%), heterozygote HbS/beta thalassemia (90%) and thalassemia intermedia (75%); nevertheless in homozygous beta thalassemia. Post splenectomical complication are often thrombocytosis, thrombosis and infections. CONCLUSION: Splenectomy should be performed in hereditary hemolytic anemia to reduce and avoid transfusion.

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BACKGROUND: Hairy cell leukemia is a rare lymphoproliferative disorder. AIM: with cytological and immunophenotypic features. METHODS: We report 6 cases of hairy cell leukemia diagnosed in the Biological Department of Hematology at the Aziza Othmana Hospital of Tunis. RESULTS: Hairy cells was observed in blood smears of 5 cases. Flow cytometry analysis shown monoclonal a monoclonal population B while gatinting on the expression of the CD19 and SSC signal. The positivity of the CD103 is noted in 5 cases and the CD11c signal is intense in all the cases. CONCLUSION: Immunophenotype is of great interest in the diagnosis of hairy cell leukemia.

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AIM: was to provide the clinical and biological patterns hemoglobine disease in Tunisia. METHODS: This retrospective study collected to 16 cases of hemoglobin C disease : 6 homozygotic Hb C and 10 heterozygotic Hb C/beta-thalassemia. RESULTS: The clinical profile is characterized by mild hemolytic anemia (Hb = 11.7 g/dl) associated with splenomegaly and hypersplenism. Contrary to homozygous state, the Hb C/beta-thalassemia is associated with microcytosis and pseudopolycythemia. The diagnosis is based on target cells, specific intraerythrocytic Hb C crystals in blood smear and Hb C level at 100%. CONCLUSION: The Hb C disease must be considered as a benign hemoglobinopathy which is associated with a long survival without major complications.

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BACKGROUND: Hemoglobin O Arab is a rare abnormal hemoglobin. AIM: We report the Clinical and biological features of this disease METHODS: 20 patients.:16 were compound hétérozygous Hb O Arab/Béta thalassemia and 4 homozygous Hemoglobin O Arab. Patients are 7 men and 13 women. RESULTS: Most of them are originated from the North West of Tunisia with a age average of 39.7 years. Diagnosis was carried out at a relatively old age (26.9 years old). The homozygous form was not very symptomatic. The compound heterozygous form was more severe and characterized by a mild form of thalassemia with a moderate microcytic hypochromic anaemia (Hb = 8.8 g/dl). It was often complicated of thrombopenia due to hypersplenism in 40% of the cases. Treatment was based on occasionally transfusion and splenectomy on event of hypersplenism. Evolution of this disease was generally good with a long lifespan of patients. CONCLUSION: Haemoglobin O Arab is an abnormal hemoglobin well tolerated except for heterozygous category which requires iterative transfusions. Spelenectomy is indicated in case of hypersplenious. The evolution is generally good with a long survival.

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