Imagerie des fistules arterio-veineuses durales a drainage veineux peri -medullaire

Les fistules durales rachidiennes à drainage veineux périmédullaire constituent une pathologie d’individualisation récente. C’est les plus fréquentes des malformations vasculaires vertébromédullaires,elles se présentent sous la forme d’une myélopathie spastique progressive chez un sujet de plus de 40 ans. A partir d’une étude rétrospective des données cliniques et explorations radiologiques d’une série de 11 cas en IRM et artériographie médullaire, nous avons recensé l’ensemble des signes radiologiques qui orientent le diagnostic. L’IRM a montré un hypersignal T2 centromédullaire de façon constante ainsi que des vaisseaux périmédullaires pathologiques. L’angiographie médullaire reste l’examen clé pour le diagnostic. Elle a permit de montrer dans dix cas le shunt artério-veineux ainsi que ses afférences artériels et son retour veineux, permettant de planifier le traitement. Huit patients ont été pris en charge chirurgicalement avec exclusion de la fistule suivie d’une amélioration clinique. L’imagerie joue aussi un rôle important dans le suivi postthérapeutique. L’IRM permet de mettre en évidence la disparition progressive des anomalies initiales, signede l’efficacité du traitement. Les fistules artério-veineuses à drainage veineux périmédullaire sont une pathologie qui reste encore méconnue. La connaissance de tous ces éléments d’imagerie est fondamentalepour un diagnostic et une prise en charge précoces, seuls garants d’une bonne évolution après traitement

Case Report of Cutaneous Large B-cell leg-type Lymphoma in Young Woman

Cutaneous diffuse B-cell lymphoma leg type is a rare entity, it represents approximately 5% of primary cutaneous lymphomas. It has a poor prognosis. We report case of t 58 years old patient with a cutaneous B cell lymphoma leg type with dual location at the upper and lower limbs and bone lysis. Histological examination of the skin biopsy showed diffuse lymphomatous proliferation dissecting collagen, made of large cells with abundant cytoplasm moderately ill-defined, ovoid nuclei and with a discreet nuclear irregularity. The immunohistochemical study revealed lymphoma cells phenotype B with a homogeneous expression of CD 20, a homogeneous expression of CD 3, CD 43 positive. Bcl 2 was positive, asthe Bcl 6 and Mum 1. CD 5, CD10, MPO, CD 34, and Tdt were negative. The Ki 67 was positivein90%. The staging showed extensive osteolysis of the lower end of the humerus and the ulna. The patient received 2 courses RCHOP, with faillure of treatment, and then she received one course of RDHAP before dying. The particularity of this observation consists in the age; our patient was younger than the average, and the double localization of lymphoma in both arm and leg, with osteolysis. We discuss also the difficulties of treatment of this disease

Hashimoto’s thyroiditis and acute chest syndrome revealing sickle cell anemia in a 32 years female patient

Sickle cell anemia results from a single amino acid substitution in the gene encoding the 4-globin subunit. Polymerization of deoxygenated sickle hemoglobin leads to decreased deformability of red blood cells. Hashimoto's thyroiditis is a common thyroid disease now recognized as an autoimmune thyroid disorder, it is usually thought to be haemolytic autoimmune anemia. We report the case of a 32 years old women admitted for chest pain and haemolysis anemia in which Hashimoto's thyroiditis and sickle cell anemia were found. In our observation the patient is a young woman whose examination did not show signs of goitre but the analysis of thyroid function tests performed before an uto-immune hemolytic anemia (confirmed by a high level of unconjugated bilirubin and a Coombs test positive for IgG) has found thyroid stimulating hormone (TSH) and positive thyroid antibody at rates in excess of 4.5 times their normal value. In the same period, as the hemolytic anemia, and before the atypical chest pain and anguish they generated in the patient, the search for hemoglobinopathies was made despite the absence of a family history of haematological disease or painful attacks in childhood. Patient electrophoresis's led to research similar cases in the family. The mother was the first to be analyzed with ultimately diagnosed with sickle cell trait have previously been ignored. This case would be a form with few symptoms because the patient does not describe painful crises in childhood or adolescence

The immunophenotype of adult t acute lymphoblastic leukemia in Morocco

Background: There is paucity of detailed studies of adult T cell acute lymphoblastic leukemia (T-ALL) in developing countries reflecting the condition of these patients including clinical and biological features. Objective: This study was carried out to analyze the immunophenotypic characteristics of 40 Moroccan patients with T-ALL and its association with biological and clinical features. Patients and Methods: Between 2006 and 2009, 130 adult patients diagnosed with acute lymphoblastic leukemia (ALL) were immunophenotyped by 3-color flow cytometry using a panel of monoclonal antibodies. Cases presenting features of a T-lineage phenotype were subjected to detailed analysis including immunophenotypic, clinical and biological parameters. Results: Proportion of T-ALL among ALL Moroccan patients was 31.0%. Median age of patients was 28 years. Twenty-nine patients were females and 11 were males. 45.0% of patients (18/40) had features of immature T-ALL stages (pro-T and pre-T ALL), 30.0% (12/40) of CD1a+ cortical T-ALL stage and 25.0% (10/40) had a characteristic phenotype of medullary T-ALL. The frequencies of progenitor cell markers CD10, CD34 and TdT expression were 14.0; 57.5% and 50.0% respectively. The aberrant expression of B li­neage associated antigen CD79a were positive in 20.5% of the cases and the aberrant expression of myeloid antigens CD13 and/or CD33 was found in 22 (55.0%) cases. No significant association was encountered between TdT, CD34 or myeloid antigens positivity and high risk features at presentation as age, sex, and white blood cells. However, myeloid antigens (CD13 and/or CD33) was significantly associated with T-cell maturation stages (p = 0.009). Conclusion: To the best of our knowledge, this is the first report from North Africa of immunophenotypic study on adult T-ALL. Our findings indicate that the proportion of T-ALL among ALL in Morocco is similar to that reported in others Mediterranean countries like France and Italy and that myeloid-associated antigens expression is frequently associated with immature immunophenotype. Key Words: adult T cell acute lymphoblastic leukemia, immunophenotypic analysis, immunological subtypes, myeloid and T-cell antigens

An autosomal recessive leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa maps to chromosome 17q24.2-25.3

Background Single-gene disorders related to ischemic stroke seem to be an important cause of stroke in young patients without known risk factors. To identify new genes responsible of such diseases, we studied a consanguineous Moroccan family with three affected individuals displaying hereditary leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa that appears to segregate in autosomal recessive pattern. Methods All family members underwent neurological and radiological examinations. A genome wide search was conducted in this family using the ABI PRISM linkage mapping set version 2.5 from Applied Biosystems. Six candidate genes within the region linked to the disease were screened for mutations by direct sequencing. Results Evidence of linkage was obtained on chromosome 17q24.2-25.3. Analysis of recombination events and LOD score calculation suggests linkage of the responsible gene in a genetic interval of 11 Mb located between D17S789 and D17S1806 with a maximal multipoint LOD score of 2.90. Sequencing of seven candidate genes in this locus, ATP5H, FDXR, SLC25A19, MCT8, CYGB, KCNJ16 and GRIN2C, identified three missense mutations in the FDXR gene which were also found in a homozygous state in three healthy controls, suggesting that these variants are not disease-causing mutations in the family. Conclusion A novel locus for leucoencephalopathy with ischemic stroke, dysmorphic syndrome and retinitis pigmentosa has been mapped to chromosome 17q24.2-25.3 in a consanguineous Moroccan family

Narrowing the gap for hematopoietic stem cell transplantation in the East-Mediterranean/African region: comparison with global HSCT indications and trends.

Hematopoietic Stem Cell Transplantation (HSCT) activity was evaluated in the African (AFR)/EMRO region and compared to the global activity for the years 2006-2013. Data were obtained from 1570 teams in the 6 WHO continental regions. Of these, 29 (1.85%) of all teams were active in 12 of the 68 AFR/EMRO countries. They reported 2.331 (3.3%) of the worldwide 71.036 HSCT, and a transplant rate of 32.8 (TR; HSCT/10 million inhabitants; worldwide 128.5). This reflects still the lowest regional TR despite an increase of 90% since 2006. HSCT activity in AFR/EMRO countries was characterized by a higher use of allogeneic compared to autologous HSCT, an almost exclusive use of family donors, including haploidentical family donors. These findings contrast with the prevalence of autologous over allogeneic HSCT, and a higher frequency of unrelated HSCT in other parts of the world. Of note, the increase by 200% in HSCT for hemoglobinopathies from 2006 to 2013 (72 per year) in the AFR/EMRO region. This reflects the specific role of HSCT for these disease categories with high prevalence and incidence in the AFR/EMRO region. This report provides information for the competent authorities to foster adequate infrastructure. It urges transplant organization to optimize their cooperation

Ptosis unilatéral induit par la vincristine chez un adulte traité pour Burkitt

Nous rapportons le cas d’un patient de 40 ans suivi pour un lymphome de Burkitt classé groupe C par l’atteinte neurologique et médullaire initiales, traité selon le protocole LMB A02, qui a présenté un ptosis aigue unilatéral de l’œil droit associé à une paralysie du nerf III homolatéral  au 39ème  jours de la chimiothérapie. Le bilan étiologique était négatif, une origine iatrogénique médicamenteuse a été  ainsi évoquée. La dose cumulée de vincristine reçu était de 4 mg, le ptosis diminuait  progressivement après l’arrêt de vincristine. Un traitement neuroprotecteur  adjuvant a été proposé avec une bonne évolution.

THE IMMUNOPHENOTYPE OF ADULT T ACUTE LYMPHOBLASTIC LEUKEMIA IN MOROCCO

Background: There is paucity of detailed studies of adult T cell acute lymphoblastic leukemia (T-ALL) in developing countries reflecting the condition of these patients including clinical and biological features. Objective: This study was carried out to analyze the immunophenotypic characteristics of 40 Moroccan patients with T-ALL and its association with biological and clinical features. Patients and Methods: Between 2006 and 2009, 130 adult patients diagnosed with acute lymphoblastic leukemia (ALL) were immunophenotyped by 3-color flow cytometry using a panel of monoclonal antibodies. Cases presenting features of a T-lineage phenotype were subjected to detailed analysis including immunophenotypic, clinical and biological parameters. Results: Proportion of T-ALL among ALL Moroccan patients was 31.0%. Median age of patients was 28 years. Twenty-nine patients were females and 11 were males. 45.0% of patients (18/40) had features of immature T-ALL stages (pro-T and pre-T ALL), 30.0% (12/40) of CD1a+ cortical T-ALL stage and 25.0% (10/40) had a characteristic phenotype of medullary T-ALL. The frequencies of progenitor cell markers CD10, CD34 and TdT expression were 14.0; 57.5% and 50.0% respectively. The aberrant expression of B li­neage associated antigen CD79a were positive in 20.5% of the cases and the aberrant expression of myeloid antigens CD13 and/or CD33 was found in 22 (55.0%) cases. No significant association was encountered between TdT, CD34 or myeloid antigens positivity and high risk features at presentation as age, sex, and white blood cells. However, myeloid antigens (CD13 and/or CD33) was significantly associated with T-cell maturation stages (p = 0.009). Conclusion: To the best of our knowledge, this is the first report from North Africa of immunophenotypic study on adult T-ALL. Our findings indicate that the proportion of T-ALL among ALL in Morocco is similar to that reported in others Mediterranean countries like France and Italy and that myeloid-associated antigens expression is frequently associated with immature immunophenotype. Key Words: adult T cell acute lymphoblastic leukemia, immunophenotypic analysis, immunological subtypes, myeloid and T-cell antigens

A rare association of Castleman′s disease and nephrotic syndrome

Castleman′s Disease (CD) is an uncommon and poorly understood disorder of lymph node hyperplasia of unknown etiology. This entity belongs to the atypical lymphoproliferative disorders, a heterogeneous group of diseases characterized by a hyperplastic reactive process involving the immune system. The association of the nephrotic syndrome and CD is extremely rare and their interrelation remains enigmatic. We report a case of CD of the hyaline-vascular type with unicentric localization complicated by nephrotic syndrome