[Evaluation of non-compliance of transfusion requests of packed red blood cells]

Background: Despite legislative acts develloped, many deficiencies were identified in blood requests at the National Blood Transfusion Center impedding board and blood safety

Aim:to evaluate the conformity of the different topics of packed red blood cells requests to the legislation

Methods: 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

Results: 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

Conclusions: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

[Monocentric study of Willebrand's disease in Tunisia: assets and difficulties]

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

[Quality of life during menstruation in women with an inherited bleeding disorder: a 31 cases study]

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 A and 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 in adult acute myeloid leukemia: which prognostic value?]

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 [registered sign]. 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

[Neuroacanthocytosis: a diagnosis that should be considered]

Neuroacanthocythosis regroup heterogeneous neurodegenerative diseases. These conditions share neurological, hematological and even systemic features. In spite of the genetic progress, their pathogenesis is still unknown. To report a new case of neuroacanthocythosis. A 37-year-old woman was admitted for orofacial choreatic movement disorder. These movements were associated to dysarthria, lip and tongue mutilation, areflexia and raised plasma creatine kinase level. Examination of blood smear reveled 10% of acanthocytosis. Neuro-acanthocytosis diagnosis, precisely chorea-acanthocytosis, was done. Neuro-acanthocytosis should be considered in any movement disorder in order to attempt a genetic counseling

[Iron overload in sickle cell anemia: a study of 94 patients]

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. To assess the state of iron overload in patients with sickle cell anemia according to their number of transfusions. The medical records of 94 patients with sickle cell anemia [46 had homozygous sickle cell disease, 41 had sickle-beta 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. 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. 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

[Beta thalassemia intermedia: about 36 cases]

Thalassemia intermedia empasses a mild clinical and biological spectrum. The aim is to report the clinical and biological features and treatment of this disease. It is a retrospective study about 36 thalassemia intermedia patients [17 males, 19 females]. Epidemiological, haematological aspects and treatment were reported. 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° 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]. Thalassemia intermedia is well tolerated. Transfusions and splenectomy were indicated in case of hypersplenious

[Splenectomy in hereditary hemolytic anemia: 82 Tunisian cases]

Splenectomy is frequently advised in hereditary hemolytic anemia.Severe complications could occur after splenectomy. To provide the indication and benefit of splenectomy clinical and biological patterns were performed in a retrospective study of 82 patients: 17 homozygous beta thalassemia, 17 thalassemia intermedia, 33 heterozygote HbS I beta thalassemia and 15 hereditary spherocytosis. 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. Splenectomy should be performed in hereditary hemolytic anemia to reduce and avoid transfusion

[Cytology and immunophenotype features of hairy cell leukeamia: about 6 cases]

Hairy cell leukemia is a rare lymphoproliferative disorder. With cytological and immunophenotypic features. We report 6 cases of hairy cell leukemia diagnosed in the Biological Department of Hematology at the Aziza Othmana Hospital of Tunis. Hairy cells was observed in blood smears of 5 cases. Flow cytometry analysis shown a monoclonal population B while gatinting on the expression of the CD19 and SSC signal. The positivity of the CD 103 is noted in 5 cases and the CD11c signal is intense in all the cases. Immunophenotype is of great interest in the diagnosis of hairy cell leukemia

[Hemoglobin O Arab: about 20 cases]

Hemoglobin O Arab is a rare abnormal hemoglobin. We report the Clinical and biological features of this disease 20 patients: 16 were compound heterozygous Hb O Arab/Beta thalassemia and 4 homozygous Hemoglobin O Arab. Patients are 7 men and 13 women. 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. Haemoglobin O Arab is an abnormal hemoglobin well tolerated except for heterozygous category which requires iterative transfusions. Spelenectomy is indicated in case of hypersplenism. The evolution is generally good with a long survival

Cytology and immunophenotype feautures of 80 acute myeloid leukemia

Acute myeloid leukemia [AML]'s diagnosis is clinical and biological. We report here 80 AML with cytology and immunophenotype features to establish correlations. 21 AML1, 23 AML2, 12 AML3, 2 AML4, 18AML5 and 3 AML6 were diagnosed by cytology. Only one case of AMLO was diagnosed by immunophenotype. Myelogysplasia is present in 29,8% cases. CD 19 and CD56 expression was significantly associated to AML +t[8;21]. Additionally, concomitant negativity of CD34 and HLA-DR was dis-crimininatif to AML3 diagnosis. Prognostic value to expression some CD needs time backwards

[Alpha interferon in children with philadelphia chromosome-positive chronic myeloid-leukaemia]

The present work focuses on the therapeutic efficacy and the toxicity of alpha interferon in patients younger than age 18 years. 5 patients younger than 18 years were treated and followed up between 1990 and 1999 at the department of haematology [Aziza Othmana Hospital] Hydroxyurea was given as initial treatment to all patients. After a median period of 8 months. these patients received alpha interferon [5 millions units/m 2 once]. Six months after the beginning of the alpha interferon a complete hematologic response was obtained in all patients. The median overall survival was of 66 months: 3 patients are still alive [2 patients in an advanced stage and one patient in chronic phase] and 2 patients died after transformation. The most common reported side effects of alpha interferon were asthenia, weight loss, fever, myalgia, chills and headaches - these toxic manifestations were mild and were noticed in all our patients. MyeIosuppression was noted in two patients. Interferon is well tolerated in patients younger than age years 18 old, with CML.It may offer an alternative to bone marrow transplantation in children in the chronic phase of CML without histocompatible donor. The role of new agents such as STI 571 needs to be evaluated as well

[Long term survival and prognostic factors in multiple myeloma treated with conventional chemotherapy. About 109 cases]

Our study is retrospective. We report the results of conventional chemotherapy in previosly untreated patients with myeloma. Survival and progonstic factors were analysed in 109 patients diagnosed from 1983 to 1992. The median age was 65 years, 87 patients [80%] were including in the stage III according the Durie Salmon staging system. The median survival time was 27 months and 10 years survival rate is 3,66%. In the univariate analysis, two prognostic variables were retained namely the hemoglobin and creatinine level. The study suggest that conventional therapy is agood treatment for old patients. However, patients younger than 55 years, must benefit from intensive chemotherapy supported by autologus bone marrow, pheripherol blood stem cells, or allogenic bone marrow transplantation. A considerable encrace in duration of remission and survival is possible