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We aimed to evaluate therapeutic results of radiotherapy for gastric mucosa-associated lymphoid tissue (MALT) lymphomas.We reviewed retrospectively the records of 11 patients presenting with gastric MALT lymphoma treated between 1993 and 2014. Patients with low-grade lymphoma in failure after helicobacter eradication had exclusive gastric external radiotherapy. Chemotherapy followed by radiotherapy were indicated in case of high grade lymphoma. Radiotherapy doses range between 30 and 40Gy (2Gy per fraction, five fractions per week).All tumours were IE stage. Seven patients with low-grade lymphoma had radiotherapy. Four patients with high-grade lymphoma received chemotherapy then radiotherapy. Ten patients are in complete remission after treatment achievement. Five and 10 years disease-free survival are 100%. No severe toxicity was seen.Eradication of Helicobacter pylori is the mainstay of treatment of gastric MALT. External irradiation is an effective and well-tolerated treatment modality in case of resistance to helicobacter eradication.

Apres traitement d’un lymphome hodgkinien (LH), plusieurs types de tumeurs peuvent survenir dont les lymphomes non hodgkiniens. Nous rapportons le cas d’un lymphome gastrique B diffus a grandes cellules, diagnostique chez une patiente, sept ans apres sa guerison d’un LH.

Obesity is associated with reduced exercise maximal fat oxidation rate (FATmax), which is generally assessed by cardiopulmonary cycling test. The six-minute walking test (6MWT) presents an alternative method in patients.The aim of this study was to establish a practical reference equation facilitating the prediction of FATmax from the 6 MWT in obese children of both genders.This study is a cross-sectional study using mixed linear and multiple regression models.Anthropometric measurements were recorded and submaximal cycling test and 6 MWT conducted for 131 school-aged obese children, 68 boys and 63 girls. A multiple regression analysis for FATmax, including six-minute walking distance (6 MWD), anthropometric and cardiac parameters as the dependent variables, was performed for the two genders separately.Mean 6 MWD and FATmax were 564.9 ± 53.7 m and 126.5 ± 12.1 mg min(-1) for boys and 506.7 ± 55.0 m and 120.7 ± 10.0 mg min(-1) for girls, respectively. The 6MWD, body mass index, Z-score, fat-free mass, waist and hip circumferences (WC and HC), rest heart rate, and systolic and diastolic blood pressures were highly correlated with FATmax for both genders. There was a significant correlation between 6 MWD and FATmax in both boys and girls (r = 0.88 and r = 0.81, P0.001, respectively). Stepwise regression analyses revealed that the combinations of 6 MWD with HC for boys and 6MWD with WC for girls improved the predictability of the model (R(2) = 0.81 for boys and R(2) = 0.72 for girls; P0.001).In obese children, the 6MWT can be used to predict FATmax when formal test of exercise capacity and gas exchange analysis are unavailable or impractical. It is therefore possible to prescript targeted exercises at FATmax, without performing indirect calorimetry, just from a field test.

Summary Introduction Cryptococcus neoformans ( C. neoformans ) is a fungal opportunistic pathogen which infects immunocompromised patients, especially those who are seropositive for the human immunodeficiency virus (HIV) and those with lymphoproliferative malignancies. Lungs and meninx are the most frequently involved sites, but isolated lymph nodes are less infected. Case report We report the observation of an 18-year-old-patient, receiving chemotherapy for a lymphoblastic acute leukemia, who developed an isolated subclavicular lymphadenopathy. The diagnosis of cryptococcal lymphadenitis was confirmed by fine needle aspiration cytology and biopsy. Surgery and fluconazole allowed a favourable outcome. Conclusion Isolated cryptococcal lymphadenopathy is a rare complication that occurs in patients with hematological malignancies. The diagnosis is based on culture and/or fine needle aspiration cytology and/or biopsy. The prognosis is favourable with surgical treatment and antifungal therapy.

Invasive aspergillosis (IA) is a major opportunistic infection in haematology patients. Preventive measures are important to control IA because diagnosis is difficult and the outcome of treatment is poor. We prospectively examined the environmental contamination by Aspergillus and other fungal species and evaluated the prevalence of invasive aspergillosis in the protect unit of haematology. A three-year prospective study (December 2004-September 2007) was carried out in the department of haematology of Hedi Chaker Hospital. Suspected invasive aspergillosis cases were reviewed and classified as proven, probable and possible invasive aspergillosis using the EORTC criteria. During the study period, we collected weekly environmental samples (patient's rooms, tables and acclimatisers) and clinical samples from each patient (nasal, expectoration and auricular). Among 105 neutropenic patients, 16 had probable and 13 had possible IA. A total of 1680 clinical samples were collected and A. flavus was most frequently isolated (79.2%). Analysis of 690 environmental samples revealed that Penicillium (44%) was the most frequent followed by Cladosporium (20%), Aspergillus spp. (18%) and Alternaria (13%). The PCR-sequencing of 30 A. flavus isolates detected from clinical and environmental samples confirmed the mycological identification. Our findings underline the importance of environmental surveillance and strict application of preventive measures.

Introduction Le syndrome de la jonction pyelo-ureterale (SJPU) est la plus frequente des uropathies obstructives congenitales. Il est defini par un trouble urodynamique des voies excretrices hautes en amont d’un obstacle fonctionnel ou organique de la jonction pyelo-ureterale. Son traitement est chirurgical. Sa surveillance en imagerie repose essentiellement sur la scintigraphie dynamique pour definir le succes operatoire. Objectif Evaluer le rythme de surveillance isotopique de nos patients apres traitement et son interet. Materiel et methodes Etude retrospective portant sur 107 patients operes pour SJPU durant une periode de 11 ans. Tous ces patients ont beneficie d’au moins une scintigraphie renale dynamique 99mTc-MAG3 ou 99mTc-DTPA en postoperatoire. Resultats Le controle scintigraphique postoperatoire a ete fait chez 104 patients (97,2 %) a 3 mois, contre 49,5 % a 6 mois et 86 % a un an. Dix patients (9,3 %) ont presente une complication precoce, precisement une stase obstructive, lors du controle postoperatoire du 3e mois, parmi lesquels 3 seulement (2,8 %) ont ete repris chirurgicalement suite a un controle scintigraphique apres un mois. Une resolution spontanee de l’obstruction a ete observee chez les 7 patients restants. Par ailleurs, chez 8 autres patients, la scintigraphie a redresse le diagnostic de stenose de l’anastomose suspecte sur l’urographie intraveineuse. Le suivi isotopique ulterieur des patients a ete pratique a six mois, un an, deux ans et cinq ans. Lors du suivi au long cours aucune modification du nephrogramme n’a ete notee. Conclusion La surveillance des patients operes pour un syndrome de la jonction pyelo-ureterale doit faire obligatoirement appel a une scintigraphie dynamique renale qui represente l’examen de reference. Cet examen doit etre pratique a 3 mois en postoperatoire. S’il est sans anomalie, il n’est pas necessaire de continuer le suivi isotopique des patients operes.

In order to select compatible human leucocytes antigens (HLA) donors for bone marrow graft, all the members of 76 families were typed by serology for HLA class I (A and B locus) and class II (DR, DQ locus) by polymerase chain-reaction-sequence-specific primes (PCR-SSP). The HLA typing interpretation revealed the existence of crossing-over in major histocompatibility (CMH) regions for two families, AB and AT, with aplastic bone marrow. The study of crossing-over site has needed the genotyping of seven short tandem repeat (STR) markers located on the short arm of chromosome 6 (D6S291, D6S273, TNFa, C1.2.C, C3.2.11, D6S265, D6S276), using ABI Prism 310 sequencer. HLA and STR Haplotypic analysis enabled us to confirm the crossing-over between locus B and DR in AB family and between locus A and B in AT family. Based in this study, we recommend to be careful in the interpretation of the results of HLA typing between donors and recipients of bone marrow. Complementary investigations should be accomplished for studying genetic abnormalities, which would be involved in this pathology.

The occurrence of an isolated ovarian or pelvic relapse of acute lymphoblastic leukemia (ALL) in complete remission after chemotherapy has rarely been described. We report the case of a 12-year-old girl, treated for ALL, who developed an isolated left ovarian and fallopian tube localization without medullary or blood relapse 4 years after the end of the initial treatment. She presented with an isolated pelvic mass. The diagnosis was established by a CT-guided biopsy. The treatment consisted of a second course of chemotherapy and complementary surgery; a second complete remission was obtained.

Resume A propos d'une observation d'aplasie medullaire avec syndrome malformatif nous discutons les criteres diagnostiques et les cadres nosologiques des 2 principales aplasies medullaires hereditaires s'accompagnant d'un syndrome malformatif : la maladie de Fanconi et la dyskeratose congenitale. Observation Une jeune fille, âgee de 19 ans, issue d'un mariage consanguin au troisieme degre, a ete hospitalisee pour exploration d'un syndrome anemique et hemorragique. L'examen clinique a montre un syndrome malformatif associant une microphtalmie, des taches cafe au lait, une hyperpigmentation generalisee et une dystrophie ungueale des doigts et des orteils sans leucoplasie des muqueuses. Un retard staturoponderal a ete note. L'hemogramme a montre une pancytopenie et la biopsie medullaire une moelle hematopoietique desertique en faveur d'une aplasie medullaire. Le caryotype realise sur des lymphocytes sanguins apres sensibilisation par la mitomycine C, a objective un syndrome d'instabilite chromosomique franc. Le diagnostic d'aplasie medullaire hereditaire a ete retenu et la patiente a ete mise sous norethandrolone. La patiente est decedee3 mois plus tard par choc septique. Discussion Devant un tableau d'aplasie medullaire associe a un syndrome malformatif, le diagnostic d'aplasie medullaire hereditaire est le plus probable. La maladie de Fanconi est la plus frequente, evoquee devant une dysmorphie de la face caracteristique avec microphtalmie, des taches cafe au lait, un retard staturoponderal, des anomalies de la colonne du pouce. La dystrophie ungueale, la leucoplasie des muqueuses sont presque pathognomoniques du diagnostic de dyskeratose congenitale. Si le syndrome malformatif est ambigu, l'etude cytogenetique ne permet pas de lever le doute diagnostique dans toutes les situations, comme dans notre observation.

Factor XIII deficiency is a rare autosomal recessive congenital disorder of haemostasis characterised by a plasmatic factor XIII level less than 1% in homozygote and bleeding as of the youth. We report a study about ten patients with congenital factor XIII deficiency from seven south-Tunisian families, there are seven females. Umbilical bleeding was common and only two patients had intracranial bleeding. The standard screening tests are normal. Factor XIII activity was less than 1% in all patients. A sub-unit A deficit was detected for the ten patients. Out hemorrhagic context, five patients receive regular prophylactic transfusion with fresh frozen plasma.

From July 1992 to December 2000, 288 cases of monoclonal gammapathy (MG) were collected at the university hospital of Sfax. The middle age of the patients at the time of the diagnosis was 62 years and 7 months with extremes to 18 months and 99 years and median to 64 years. One hundred and eighty-two patients were men and 106 women. Among the 270 observations for which aetiology has been established, 73 were classified MG of undetermined significance (MGUS), 160 myeloma (or plasmocytoma) and 37 other malignant MG (Waldenstrom's macroglobulinemia: 13, lymphoma: 9, alpha heavy chains disease: 6, primary amyloidosis: 5, chronic lymphocytic leukaemia: 4). Rheumatological affections (19.2%), infections and renal failure (10% each), haematological and autoimmune diseases (9.6% each) were pathologies most often associated with MGUS. Agarose gel electrophoresis did not show a monoclonal peak in 16% of the cases. In the 242 patients with a peak on electrophoresis, the peak was in the beta zone in 22% of cases and in the gamma zone in 78% of cases. The IgG isotype represents more than the half of the cases of our set (51.7%). IgG is even more predominant in the MGUS group (65.8%). The IgA isotype counts for 20.8% of the cases in our set and the free light chains (kappa or lambda) for 13.6% of the cases whereas the IgM represents 8.7% only of the 288 cases of our set which involves three cases of IgD myeloma and six cases of biclonal gammapathy.

Resume La maladie de Gaucher est l’une des rares maladies lysosomiales qui pourrait beneficier d’un traitement enzymatique substitutif susceptible d’ameliorer considerablement le pronostic de certaines formes. Le but de ce travail est d’etudier l’epidemiologie de cette maladie en Tunisie, de degager les difficultes diagnostiques et therapeutiques et peut-etre de preciser nos besoins, ulterieurs en traitement substitutif. Patients et methodes. – Nous avons mene une etude retrospective en envoyant une fiche d’etude aux differents services susceptibles de prendre en charge des patients atteints de la maladie de Gaucher a savoir les services de pediatrie, de neonatologie, de medecine interne, d’hematologie, de neurologie et de cardiologie. Resultats. – Cette etude nous a permis de colliger 27 cas de maladie de Gaucher suspectee ou confirmee sur une periode de 18 ans allant de 1983 a 2001. L’âge de revelation de la maladie a varie de la naissance a 73 ans avec un âge moyen de 14,5 ans. Selon l’âge de revelation et la presentation clinique nous avons pu classer nos patients en 20 cas de type 1 (74 %) ; trois cas de type 2 (11 %) et trois cas de type 3 (12 %), et un cas de type non determine. 1. Maladie de Gaucher de type 1. L’âge de revelation de la maladie a varie de 10 mois a 73 ans avec une moyenne de 19 ans. Les principaux signes cliniques observes sont representes par la splenomegalie, l’hepatomegalie, la pâleur, les manifestations hemorragiques mais aussi l’osteoporose et les douleurs osseuses observees des 40 % des cas. Le diagnostic etait fonde sur l’histologie avec mise en evidence de cellules de Gaucher dans divers tissus alors que la confirmation du diagnostic par dosage de la glucocerebrosidase intraleucocytaire n’a eu lieu que dans 50 % des cas. Le traitement etait toujours symptomatique (antalgiques, transfusions). Une splenectomie a ete realisee dans 47 % des cas, alors qu’aucun patient n’a beneficie d’un traitement specifique (greffe de moelle osseuse ou traitement enzymatique specifique). Sur le plan evolutif, le recul a varie de un mois a 18 ans avec un recul moyen de quatre ans. Parmi les 12 patients ayant un recul superieur ou egal a un an, on a note une amelioration apres splenectomie dans trois cas, une stabilisation dans trois cas et une aggravation concernant surtout l’atteinte osseuse dans deux cas. Un seul patient âge de 73 ans est decede dans un tableau d’insuffisance respiratoire et seuls trois patients ont ete perdus de vue. 2. Maladie de Gaucher de type 2. Trois patients ont pu etre identifies, âges de 1 jour, 45 jours et 3 mois. L’atteinte viscerale etait severe et l’atteinte neurologique a comporte surtout des convulsions, une hypertonie, des troubles de l’oculomotricite et une regression psychomotrice. Les trois enfants sont decedes. 3. Maladie de Gaucher de type 3. Trois patients etaient probablement atteints de maladie de Gaucher de type 3 avec des signes visceraux apparus a des âges respectifs de 9 mois, 1 an et 3 ans, et des signes neurologiques apparus a des âges de 2,5 et 3 ans. Deux patients sont decedes et le troisieme a ete perdu de vue. Conclusion. – La maladie de Gaucher n’est pas exceptionnelle en Tunisie. Le type 1 est de loin le plus frequent. Nous avons constate une insuffisance diagnostique puisque le dosage enzymatique intraleucocytaire n’est pratique que dans 50 % des cas, et surtout des insuffisances therapeutiques avec la non prescription de traitement specifique.