Formulation development of the deleterious rhizobacterium Pseudomonas trivialis X33d for biocontrol of brome (Bromus diandrus) in durum wheat.

AIM: To develop an appropriate formulation of the deleterious rhizobacterium Pseudomonas trivialis X33d and to evaluate its effectiveness to reduce brome growth. METHODS AND RESULTS: Two formulations of Ps. trivialis X33d, a semolina-kaolin granular formulation (Pesta) and talc-kaolin powder, were prepared and their effectiveness in reducing brome growth was evaluated. Both brome suppression and cell viability of X33d were higher in Pesta granular formulation than in talc-kaolin powder one. The impact of storage temperature and the addition of adjuvants (sucrose and oil) to the granular formulation of X33d were assessed in order to improve the shelf life of the formulation. The longest viability was found in formulated product supplemented with adjuvants and stored at 4°C. The effect of Pesta granules supplemented with adjuvants and stored for 6 months at 4°C on brome and wheat growth under controlled and greenhouse conditions was evaluated. The X33d formulation in Pesta increased the growth of wheat and reduced brome growth. CONCLUSION: Our results indicate that Ps. trivialis X33d formulated in Pesta has potential as a bioherbicide to control brome. SIGNIFICANCE AND IMPACT OF THE STUDY: Because of the impracticality of applying bacterial cell suspension on a large scale, the use of Pesta granules of X33d against brome could help in achieving a sustainable agriculture application of a bioherbicide.

[Molecular analysis of crossing-over in the CMH in two Tunisian families with aplastic bone marrow]. / Analyses moléculaires de recombinaisons localisées au niveau du CMH dans deux familles tunisiennes.

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.

First Report of Cercospora tripolitana Causing Leaf Spot of Emex spinosa in Tunisia.

Emex spinosa (L.) is a common weed in cereal crops and pastures in northern Tunisia. The build up of the seed bank from a cropping-grazing farming system makes chemical and cultural controls inefficient. Biological control as part of integrated weed management may improve weed control. Diseased seedlings were collected from several locations in northern Tunisia during field surveys. Symptoms were small, circular, light brown leaf spots varying in size (1.75 to 3.5 mm in diameter) with a definite dark brown border on both sides of leaves that wilted and died. Microscopic observations showed conidiophores and conidia within and around the spots. Pure cultures from single conidia were obtained on carrot leaf extract agar. The fungus was identified as Cercospora tripolitana on the basis of identification keys described by Chupp (1). Conidia, borne on unbranched, fasciculated conidiophores, were elongate, hyaline, multiseptate, 110 to 150 × 1.8 to 3.7 µm (average 130 to 2.5 µm), and had truncate bases. For pathogenicity testing, six plants were sprayed with a spore suspension of 6 × 105 conidia/ml. Controls were sprayed with sterile distilled water. Plants were placed in a growth chamber at 22°C, 95% relative humidity, and 18/6 h of light/dark and monitored for symptoms. Ten days after inoculation, symptoms identical to those observed in the field were observed on inoculated plants. Control plants did not develop any symptoms. Four weeks later, diseased leaves turned yellow and died. The fungus was reisolated from symptomatic plants according to Koch's postulates. Although C. tripolitana has been previously reported in North and South Africa, to our knowledge, this is the first report of the fungus as a pathogen on E. spinosa under Tunisian agroecological conditions, making it a promising candidate for weed control. Reference: (1) C. Chupp. A Monograph of the Fungus Genus Cercospora. C. Chupp, Ithaca, New York, 1953.

First Report of Leaf Spot Caused by Cercospora bizzozeriana on Lepidium draba in the United States.

The herbaceous perennial Lepidium draba L. is an invasive weed of rangelands and riparian areas in North America and Australia. As of 2002, it had infested 40,500 ha of rangeland in Oregon and large areas in Wyoming and Utah. Little is known of plant pathogens occurring on L. draba, especially in the United States, that could be useful for biological control of the weed. Leaf spots were first noted on a stand of L. draba near Shepherd, MT in 1997. The spots were mostly circular but sometimes irregularly shaped and whitish to pale yellow. The pathogen was erroneously assumed to be Cercospora beticola since its morphological traits closely resembled that species and the area had large fields of sugar beet with heavy Cercospora leaf spot incidence. Diseased leaves of L. draba were collected in 1997 and 2007. Conidia, borne singly on dark gray, unbranched conidiophores produced on dark stromata late in the season, were elongate, hyaline, multiseptate, 38 to 120 × 2 to 6 µm (mostly 38 to 50 × 2 to 5 µm) and had bluntly rounded tips and wider, truncate bases. These characteristics were consistent with the description of C. bizzozeriana Saccardo & Berlese (2). To isolate the fungus, spores were picked from fascicles of conidiophores with a fine-tipped glass rod, suspended in sterile water, and spread on plates of water agar. Germinated spores were transferred to potato dextrose agar (PDA). The ITS1, 5.8S, and ITS2 sequences of this fungus (GenBank Accession No. EU887131) were identical to sequences of an isolate of C. bizzozeriana from Tunisia (GenBank Accession No. DQ370428). However, these sequences were also identical to those of a number of Cercospora spp. in GenBank, including C. beticola. We also compared the actin gene sequences of the Montana isolate of C. bizzozeriana (GenBank Accession No. FJ205397) and an isolate of C. beticola from Montana (GenBank Accession No. AF443281); the sequences were 94.6% similar, an appreciable difference. For pathogenicity tests, cultures were grown on carrot leaf decoction agar. Aqueous suspensions of 104 spores per ml from cultures were sprayed on 6-week-old L. draba plants. Plants were covered with plastic bags and placed on the greenhouse bench at 20 to 25°C for 96 h. Koch's postulates were completed by reisolating the fungus from the circular leaf spots that appeared within 10 days, usually on lower leaves. Spores of C. bizzozeriana were also sprayed on seedlings of sugar beet, collard, mustard, radish, cabbage, and kale under conditions identical to those above. No symptoms occurred. After the discovery of the disease in 1997, plants of L. draba in eastern Montana, Wyoming, and Utah were surveyed from 1998 to 2003 for similar symptoms and signs, but none were found. This, to our knowledge, is the first report of C. bizzozeriana in the United States. The initial report of the fungus in North America was from Manitoba in 1938 (1). It has recently been reported as occurring on L. draba in Tunisia (4) and Russia (3) and is reported as common in Europe (2). A voucher specimen has been deposited with the U.S. National Fungus Collections (BPI No. 878750A). References: (1) G. R. Bisby. The Fungi of Manitoba and Saskatchewan. Natl. Res. Council of Canada, Ottawa, 1938. (2) C. Chupp. A Monograph of the Fungus Genus Cercospora. C. Chupp, Ithaca, NY, 1953. (3) Z. Mukhina et al. Plant Dis. 92:316, 2008. (4) T. Souissi et al. Plant Dis. 89:206, 2005.

First Report of Rust Caused by Puccinia carduorum on Italian thistle in Tunisia.

Italian thistle (Carduus pycnocephalus L.) is a common and increasingly important weed in Tunisia. It is also problematic in the western United States and a target of biological control. In surveys conducted in northern Tunisia from 2003 to 2005, Italian thistle plants in many locations were found diseased by rust. Eighty-five isolates of rust were collected from Italian thistle during these surveys. Each isolate was collected from a single plant and stored individually as mixtures of urediniospores and teliospores at 4°C or in liquid nitrogen. Urediniospores and teliospores of all isolates were similar in morphology and matched the description of Puccinia carduorum Jacky (3). Isolate B1003 (BPI No. 878207), collected from Béja, Tunisia, was arbitrarily selected for further study. Comparison of internal transcribed spacer (ITS) regions of B1003 (GenBank Accession No. EF050059) with other ITS sequences indicated a 97% similarity to P. carduorum (GenBank Accession No. PCU57351) from Carduus nutans subsp. leiophyllus. Eight Italian thistle plants, grown from seeds collected in Béja, were inoculated in the 3- to 5-leaf stage with urediniospores of B1003 by spraying plants with an aqueous suspension of urediniospores at 106 spores per ml with approximately 0.03 ml of surfactant until they were thoroughly wet. Four plants were sprayed with water plus surfactant only. All plants were covered with plastic bags and placed in a growth chamber at 18/20°C night/day temperatures. Bags were removed 24 h after inoculation and plants were monitored daily for symptoms. Plants sprayed with water plus surfactant only did not develop symptoms. Six inoculated plants developed disease symptoms similar to those observed on samples collected during the surveys. White flecks appeared within 7 days of inoculation and developed into brown pustules 10 days after inoculation. Pustules enlarged and produced urediniospores until they covered both sides of diseased leaves. One month after appearance of symptoms, diseased leaves turned yellow and died. Urediniospores from these plants were used to inoculate six plants each at the 2- to 5-, 6- to 8-, and >8-leaf stages. All plants became diseased and produced uredinia. Plants in the 2- to 5-leaf stage were more severely diseased than other plants. P. carduorum was introduced to the United States in a field test for control of C. nutans subsp. leiophyllus (musk thistle) and has become established in a number of states (1). An isolate of this fungus has also been found from C. tenuiflorus (slender-flower thistle) in California (4). However, neither isolate causes substantial disease on Italian thistle from California (2,4). Isolates of P. carduorum from C. pycnocephalus in Greece, Italy, and Turkey caused little disease on most Italian thistle collections from California (2), indicating variability in susceptibility among plants as well as in virulence among rust isolates from different geographical locations. To our knowledge, this is the first report of P. carduorum parasitizing Italian thistle in Tunisia. Tests will be conducted to determine the host range of this isolate among C. pycnocephalus collections and other species. References: (1) A. B. A. M. Baudoin and W. L. Bruckart. Plant Dis. 80:1193, 1996. (2) W. L. Bruckart and G. L. Peterson. Phytopathology 81:192, 1991. (3) D. B. O. Savile. Can. J. Bot. 48:1553, 1970. (4) A. K. Watson and K. Brunetti. Plant Dis. 68:1003, 1984.

[Evaluation of a prospective therapeutic protocol in adult patients with nonsevere aplastic anemia in South Tunisia]. / Evaluation d'un protocole thérapeutique prospectif des aplasies médullaires acquises non sévères de l'adulte dans le Sud tunisien.

INTRODUCTION: Immunosuppressive drugs are usually used in the treatment of acquired aplastic anemia (AAA). The aim of this study was to evaluate the efficiency of a prospective therapeutic protocol using cyclosporine and androgens in the treatment of adult patients with nonsevere AAA. METHODS: Twenty-nine patients diagnosed and treated at the University Hospital of Sfax (Tunisia), during a 10-year period (1991-2000) were included. In addition to symptomatic treatment (transfusion, antibiotics), all the patients received a specific treatment including two drugs: cyclosporine 5mg/kg per day and androgens 0,5mg/kg per day. RESULTS: The response rate at three, six and 12 months were 48, 75, and 87%, respectively. Survival rate was 52% at one year, and 37% at five and 10 years. The main toxicities were hepatic, renal, and hypertension, observed in 53, 16 and 15%, respectively. These toxicities were reversible in 65, 87 and 100% of the cases, respectively. CONCLUSION: The response and survival rates in our series are quite satisfactory when compared to those obtained with other immunosuppressive drugs (cyclosporine and antilymphocyte serum) in the literature. The addition of androgens in our patients seemed to potentiate the immunosuppression induced by ciclosporin, but secondary toxic effects were more common.

[Clinical spectrum of cobalamin deficiency in Tunisia]. / Le spectre clinique des déficits en cobalamines en Tunisie.

PURPOSE: the aim of this study was to determine the prevalence of cobalamin (vitamin B12) deficiency in different populations of patients with clinical manifestations associated or secondary to cobalamin or folates deficiency and to analyse the demographic, clinical, paraclinical investigations in cobalamin deficient patients in Tunisia. METHODS: it was a prospective (1999-2001) multicenter study of 604 patients divided into four groups. The first group is composed of 478 consecutive patients with anaemia and/or macrocytosis with megaloblastic haemopoiesis on bone marrow examination without myelodyslasic or malignancy signs. The second group is made up of 34 patients with unexplained neurological symptoms without the presence of anaemia. The third group was composed of 82 invidious with isolated psychiatric disorders and the 10 patients with Hashimoto thyroïditis constituted the last group. RESULTS: serum cobalamin level was low in 98 %, 23%, 14% of cases, respectively, in the first three groups. Only one case of patients with Hashimoto thyroiditis has serum cobalamin deficiency. Pernicious anaemia (Biermer's disease) was established by dual isotope schilling examination in 103 patients among a sample of 120 serum cobalamin deficient patients (86%). The median age at presentation was 45.5 years. Severe chronic atrophic gastritis was diagnosed in 97.5% of patients with Biermer's disease. Serum antibodies against intrinsic factor and gastric parietal cells were detected in (42.5%) and (60.6%) patients, respectively; (25.5%) patients had the both types of antibodies. 23.4% patients were positive for antithyroid antibodies. Anti-nuclear antibodies were detected in 3% patients. CONCLUSION: an interesting finding of our study was the high frequency of cobalamin deficiency in Tunisia, particularly in relative young patients. Our patients had classic features of florid cobalamin deficiency (severe haematological manifestations and neuro-psychiatric disorders). The main underlying causes of such deficiencies were Biermer's disease. Subtle clinical manifestations should be recognized and investigated even in young patients at risk.

[Nosologic discussion between Fanconi disease and congenital dyskeratosis: 1 case of congenital bone marrow aplasia]. / Discussion nosologique entre dyskératose congénitale et maladie de Fanconi: à propos de 1 cas d'aplasie médullaire constitutionnelle.

UNLABELLED: Based on a case report of aplastic anemia associated with malformation, we discuss the diagnostic criteria and the nosologic problem between the 2 principal aplastic anemia accompanied with malformation: Fanconi disease and dyskeratosis congenita. CASE REPORT: A 19-year-old girl, issued from a third degree consanguineous marriage, was admitted because of anemic and hemorrhagic syndrome. Physical examination showed several malformations: microphtalmia, brownish spots, generalized hyperpigmentation and ungueal dystrophy without mucosal leucoplasia. Statural and ponderal retardation were noted. On the hemogram there was a pancytopenia and on biopsy, the bone marrow was desertic. The caryotype performed on peripheral blood lymphocytes after sensibilisation with mitomycin C revealed chromosomal instability aspects. Based on these clinical and biological features, the diagnosis of hereditary aplastic anaemia was retained. The patient was given norethandrolone. She died 3 months later by septic shock. DISCUSSION: Coexistence of aplastic anemia with a malformative syndrome suggests most probably an hereditary form of aplastic anemia. Fanconi anemia is the most frequent. It associates characteristic anomalies of the face, with microphtalmia, brownish spots, statural and ponderal retardation, and thumb anomalies. Ungueal dystrophy, mucosal leucoplasia are almost pathognomonic of congenital dyskeratosis. When the malformative syndrome is not characteristic, the cytogenetic study may also fail to make the differential diagnosis, as was the situation in our case.

First Report of Smut Caused by Microbotryum silybum on Ivory Thistle.

Silybum eburneum Coss. & Durieu. (ivory thistle) and S. marianum (L.) Gaertn. (milk thistle) are dominant, invasive weeds in northern Tunisia (1). S. marianum is also invasive in the United States and targeted for biological control. The smut fungus Microbotryum silybum Vánky & Berner is a naturally occurring pathogen of S. marianum in Greece (2) but not in Tunisia or the United States. To assess the safety of the fungus for biological control in the United States, plants related to S. marianum were evaluated for susceptibility to M. silybum in the quarantine facility of the Foreign Disease-Weed Science Research Unit (FDWSRU), USDA/ARS, Fort Detrick, MD. Because of the close genetic relationship of S. eburneum to S. marianum, both were tested for susceptibility under greenhouse conditions at the FDWSRU. All inoculations were done by placing 5 mg of teliospores of M. silybum in the central whorl of rosettes with three to five true leaves. Individual plants in soil-filled pots were placed in a controlled chamber at 16°C with 10 h of light daily. Photon flux density in the chamber was 34 µmol·m-2·s-1 supplied by three 1.8-m long 115W fluorescent tubes and three 52W incandescent bulbs. The central whorl was misted with distilled water twice daily for 2 weeks and the temperature was then lowered to 8°C for 6 weeks. The plants were transferred to a greenhouse bench at 22 to 25°C with 14 h of light daily. Photon flux density on the bench was 620 µmol·m-2·s-1 provided by two 500W sodium vapor lamps, one 1,000W metal halide lamp, and incidental sunlight. After approximately 7 weeks, plants of each species had fully developed capitula that flowered normally, produced no flowers, or formed abnormal flowers. Abnormal capitula contained powdery masses of teliospores in the ovaries of the florets. In contrast to systemic infections that were observed in the field (2), different branches of bolted plants bore both diseased and normal capitula. In turn, diseased capitula of both species were either completely diseased (all florets filled with teliospores) or partially diseased. Four of ten S. marianum plants and six of nine S. eburneum plants were diseased. Pathogenicity tests were repeated four times with similar results. In Greece, field inoculation of S. marianum with 5 mg of teliospores produced an average of 89% diseased plants with an average of 250 g of teliospores produced per plant. A similar level of disease is possible for S. eburneum under field conditions. Teliospores from smutted ovaries of both plant species conformed to the description for M. silybum (2). Both species are annual plants that reproduce solely by seeds. Since M. silybum prevents seed production, this fungus has great potential as a biological control agent in the United States and Tunisia. A voucher specimen has been deposited with the U.S. National Fungus Collections (BPI 863477). Nucleotide sequences for the internal transcribed spacer region are available in GenBank (Accession No. AY285774). To our knowledge, this is the first report of M. silybum parasitizing S. eburneum. References: (1) G. Pottier-AlaPetite. Flore de la Tunisie: Angiospermes-Dicotylédones, Gamopétales, Tunis, 1981. (2) K. Vánky and D. Berner. Mycotaxon 85:307, 2003.

First Report of a Leaf Spot Caused by Cercospora bizzozeriana on Lepidium draba subsp. draba in Tunisia.

Lepidium draba (L.) subsp. draba (synonym = Cardaria draba (L.) Desv.), commonly known as white-top or hoary-cress (1), family Brassicaceae, is a common weed and emerging problem in wheat in Tunisia. It is also a problematic invasive weed in the northwestern United States and a target of biological control efforts. During the summer of 2002, dying L. draba plants were found around Tunis, Tunisia. Plants had grayish white leaf spots on most of the leaves. In some cases, the leaf spots dropped out of the leaves producing "shot-holes". In most cases, the leaf spots coalesced, and the leaves wilted and died. Diseased leaves were collected, air-dried, and sent to the quarantine facility of the Foreign Disease-Weed Science Research Unit (FDWSRU), USDA/ARS, Fort Detrick, MD. The air-dried leaves were observed microscopically, and numerous conidiophores and conidia were observed on both sides of the leaves within and around the lesions. The fungus isolated (DB03-009) conformed to the description of Cercospora bizzozeriana Saccardo & Berlese (2). Conidiophores were unbranched, pale olive-brown, 1 to 5 geniculate, and uniform in color and width. Conidia were hyaline, straight to slightly curved, multiseptate, and 57 to 171 × 3.8 to 6.7 µm (average 103 to 4.6 µm). Stems and leaves of 12 rosettes (10 to 15 cm in diameter) of 6-week-old L. draba plants were spray inoculated with an aqueous suspension of conidia (1 × 105/ml) harvested from 6- to 8-day-old cultures grown on carrot leaf decoction agar. Six of the plants and two noninoculated plants were placed in a dew chamber at 22°C in darkness and continuous dew. The other half of the plants and two noninoculated plants were placed on a greenhouse bench at approximately 25°C and covered with clear polyethylene bags. After 72 h, plants from the dew chamber were moved to a greenhouse bench, and the bagged plants were uncovered. All plants were watered twice daily. After 9 days, symptoms were observed on the plants that had been bagged but not on the plants from the dew chamber. Symptoms were identical to those observed in the field in Tunisia and included "shot holes". No symptoms were observed on noninoculated plants. C. bizzozeriana was reisolated from the leaves of all symptomatic plants. Completion of Koch's postulates was repeated with an additional five plants. This isolate of C. bizzozeriana is a destructive pathogen on L. draba subsp. draba, and severe disease can be produced by inoculation of foliage with an aqueous suspension of conidia. This isolate is a good candidate for mycoherbicide development in Tunisia where the weed and pathogen are indigenous. However, some commercially grown Brassica species were found susceptible to this isolate, which will preclude its use as a classical biological control agent in the United States. To our knowledge, this is the first report of C. bizzozeriana on L. draba subsp. draba in Tunisia. A voucher specimen has been deposited at the U.S. National Fungus Collections (BPI 843753). Live cultures are being maintained at FDWSRU and the Institut National Agronomique de Tunisie, Tunis, Tunisia. References: (1) I. A. Al-Shehbaz and K. Mummenhoff. Novon 12:5, 2002. (2) C. Chupp. A Monograph of the Fungus Genus Cercospora. C. Chupp, Ithaca, New York, 1953.

[Myelodysplastic syndrome / acute myeloid leukemia with t(8; 21) and bundle of Auer rods in neutrophils: an unusual hemopathy]. / Syndrome myélodysplasique / leucémie aiguë avec t(8;21) et polynucléaires à corps d'Auer en fagot: une hémopathie rare de présentation inhabituelle.

We report the case of a 8-year-old girl diagnosed with myelodysplastic syndrome. This case was morphologically characterized by the presence of bundle of Auer rods in the neutrophils. The evolution of the disease was marked by a quick transformation in a acute myeloid leukaemia with t(8;21) refractory to treatment. We reviewed the literature for clinical, biological and therapeutic features of this rare childhood hemopathy.

[Low dose cytarabine in the treatment of acute myeloid leukemia. Apropos of 41 cases]. / Cytarabine à faible dose dans le traitement des leucémies aiguës myéloïdes. A propos de 41 cas.

This is a retrospective study on the use of cytarabine at low doses in acute myeloid leukemias in 41 patients. Four groups of AML are included: group A: 19 cases of de novo AML in elderly patients; group B: ten cases of AML in relapse; group C: five cases of AML refractory to previous treatment and group D: seven cases of secondary AML. Cytarabine was given subcutaneously at the dose of 10 mg/m2 of body surface, for 21 days per month for the first course; then 15 days per month for the following courses. The response rates were 42, 20, 0, and 43% respectively for the A, B, C, and D groups. A complete remission was attained in only 15% (six patients). Extra haematological tolerance was excellent. Infection complications were noted in 66%, whereas a severe neutropenia was observed in 34% of patients. Hemorrhagic complications were more rare (20% of patients). The mean duration of complete remission was 10 months. The median survival was 10.5 months (2 to 31 months) for the responder patients, and 2.4 months (1 to 7 months) for the non-responders. Cytarabine at low doses seems to be a good indication for first intention treatment of AML in elderly patients. It does not give a bone marrow aplasia, the infection and hemorrhagic episodes are less numerous than with conventional dose chemotherapy, the life quality is improved, and treatment at home is often possible.

[Evaluation of tumoral and lymph node response to neoadjuvant chemotherapy in undifferentiated nasopharyngeal carcinoma]. / Evaluation de la réponse tumorale et ganglionnaire à la chimiothérapie néoadjuvante dans les carcinomes indifférenciés du nasopharynx.

Between January 1994 and June 1995, 19 cases of nasopharyngeal carcinoma, classified N2-N3 (AJC/UICC 1987) were treated by neoadjuvant chemotherapy including cisplatin 100 mg/m2 on day 1 and epirubicin 80 mg/m2 on day 1. Following course started on day 21. A clinical and scannographic evaluation was made after 3 courses of chemotherapy. Fifty-eight percent of the patients were N3. Seventy-four percent were T3-T4. Tolerance to chemotherapy was good in 100% of the cases. A functional improvement was obtained in 14 among 16 patients who were initially symptomatic. For lymph nodes, an objective response (OR) was observed in all patients. The response was complete (CR) in 53%. A regression of primary tumor was obtained in 68% of the patients, but it was complete in 16% only. Sixty-nine percent of the patients has have a tumoral OR have a lymph node CR. Ninety percent of the patients with a complete lymph node response have a tumoral regression more than 50%. A correlation seems likely between the importance of the tumoral and the lymph node responses.

[Hemoglobin beta S haplotype in the Kebili region (southern Tunisia)]. / Etude de l'haplotype beta S de l'hémoglobine dans la région de Kebili (Sud tunisien).

Sickle cell anemia is a monogenic hereditary disease characterized by a mutation in the beta globin gene. Five major haplotypes associated with the beta S mutation have been defined: Benin, Bantu, Senegalian, Camerounian, and Arabo-Indian. Previous studies in northern Tunisia showed that sickle cell anemia was of Benin origin in this region. Patients from the south of Tunisia, mainly from the Kebili region, were not previously concerned. In this study, we have determined the beta S haplotype and evaluated phenotypical expression of the disease in 14 patients from this latter region. The use of four restriction endonucleases having polymorphic sites in the beta globin gene showed that all patients had the Benin haplotype, confirming the Benin origin of sickle cell anemia in Tunisia. This haplotype is associated with an heterogeneous expression of fetal hemoglobin (HbF) with extremes varying from 2.4 to 16.3% and a mean expression rate of 8.16%, which is in accordance with literature data. In spite of the haplotype homogeneity in our patients, clinical heterogeneity was noted. A unique case of alpha-thalassemia could not explain this heterogeneity. In contrast, we found a certain correlation between fetal hemoglobin expression and clinical severity.

[Prevalence of hemoglobin abnormalities in Kebili (Tunisian South)]. / Prévalence des anomalies de l'hémoglobine à Kebili (sud Tunisien).

BACKGROUND: Hemoglobin abnormalities constitute a public health problem in many countries in the world. In Tunisia, these disorders were thought to affect only the North-western population. However, the existence of hemoglobinosis concentration in Kebily in south Tunisia has been suggested by previous work. In order to estimate their frequencies, we performed a screening of hemoglobin abnormalities in the North-Kebili region, to establish a prevention program of the homozygous forms. METHODS: This screening concerned all 1st and 2nd grade primary school pupils in North Kebily. After a questionnaire, a blood sample was drawn from every child. Hemogram, sickling test, and hemoglobin electrophoresis at alkaline pH were performed for all children. Hemoglobin electrophoresis at acid pH and a specific hemoglobin A2 titration were performed for some children. RESULTS: The study concerned 1,400 children, aged between 5 and 12 years, the mean age was 7 years and 7 months +/- 10 months. Consanguinity rate and coefficient were respectively 44% and 2249 x 10(5). Endogamy was very high. The global rate of hemoglobin abnormalities was 9.4%. Drepanocytosis with a rate of 4.9% was the most frequent, followed by beta thalassemia (3.1%) and C hemoglobinosis (1.6%). These abnormalities were unequally distributed; very frequent in some localities, they were quite absent in others. CONCLUSIONS: This study revealed a hemoglobinosis concentration in Tunisia, which can be classified second after that of Beja in North-western Tunisia. The heterogeneous distribution of the hemoglobin abnormalities in North-Kebili region and the high consanguinity and endogamy rates constitute factors that promote homozygous and double heterozygous forms to arise and justify the elaboration of a preventive strategy.

[Treatment of aplastic anemia with cyclosporine, prednisolone and androgens (primary results apropos of 10 cases]. / Traitement de l'aplasie médullaire par ciclosporine, prednisolone et androgènes (résultats préliminaires à propos de 10 cas).

Aplastic anaemia is a potentially fatal haematopoietic disorder whose aetiology is not yet clarified. In our preliminary study we have introduced cyclosporin in the aplastic anaemia treatment to evaluate its effect on the disease evolution. Ten aplastic anaemia patients, mean age 33.33 +/- 20.01 years, were treated with cyclosporine (9 +/- 2.35 mg/kg/d), prednisolone (0.5 mg/kg/d) and androgens (1 mg/kg/d). The prednisolone was always combined with cyclosporine. The androgens were administered concomitantly with the cyclosporine or alternately. Seven patients responded to the treatment after a median remission delay of 6 weeks (2-12 weeks). They became independent of blood requirements at a median of 36 weeks (8-108 weeks); the three other patients died during the first trimester without showing any improvement. Among the seven responders, two relapsed early and transiently. The rate of actuarial survival was 70 per cent. The median duration of survival was 10.5 months. The side effects observed included one case of malignant lymphoma, six cases of liver toxicity and five cases of kidney toxicity. This toxicity was reversible after dose adjustment of the cyclosporine. In our study, the introduction of cyclosporin in the aplastic anaemia treatment resulted in improved therapeutic response. Androgens should be used to maintain the haematologic response. This therapeutic protocol associated with drug monitoring seems promising and the side effects should not limit its use because of the severity of the underlying disease.

[Fanconi disease: study of 43 cases in southern Tunisia]. / La maladie de Fanconi: étude de 43 cas dans le sud tunisien.

BACKGROUND: To report the epidemiologic, clinical, biological features and course of Fanconi's anemia in southern Tunisia. PATIENTS AND METHODS: During a period of 12 years we observed 43 cases. For each patient, careful clinical, biological (hemogram, myelogram, bone marrow biopsy, hemoglobin electrophoresis, karyotype) and radiological (skeleton X-rays, abdominal echography and intravenous urography) examinations were performed. All the patients who were at a pancytopenia stage were given androgens. None had a bone marrow allograft. RESULTS: There were 24 girls and 19 boys. The mean age at diagnosis was 10 years and 9 months. The familial character was present in 53% of the cases. The most frequent initial complaint was anemic syndrome (69%). In ten cases (24%), the diagnosis has been established during a familial investigation. Malformations were present in all cases (abnormal pigmentation: 86%; skeletal maturation retardation: 83%; facial dysmorphy: 76%; statural hypotrophy: 65%; bone abnormalities: 53%; renal malformations: 44%). Anemia was present in 88% of the cases, thrombocytopenia and neutropenia in all cases. Bone marrow was hypoplastic or aplastic in all cases on biopsies. Spontaneous chromosomal breaks were found in 79% of the studied cases. Fetal hemoglobin was increased in 80% of the studied cases with a mean level of 20.5%. Actuarial survival rate at 5 years was 48%, but long survival durations were rare (eight out of 43 patients). DISCUSSION: This disease, rare in the world, seems to be frequent in southern Tunisia. A normal karyotype (with classical techniques), found in five patients, could not discard the diagnosis; for this reason, the use of sensitizing agents should improve the sensitivity of the test. Besides, an increased level of fetal hemoglobin enabled us to suggest the diagnosis in some cases. Androgenotherapy increased the survival duration to more than 5 years in eight patients. However, bone marrow allograft remains the only possibility of cure.

[Plasmocytic pleural effusion disclosing multiple myeloma]. / Epanchement pleural plasmocytaire révélateur d'un myélome multiple.

Pleural effusion caused by plasma cell involvement in multiple myeloma has been reported unfrequently, and has been described at a frequency below 1% of multiple myeloma. In this study, we report an observation with pleural effusion as first symptom of multiple myeloma. The analysis of the pleural liquid showed plasma cells with a monoclonal IgG Kappa immunoglobulin. In addition, there was a bone marrow infiltration by plasma cells, a serum monoclonal immuno-globulin of the same type and osteolytic lesions. Our patient has received one course of chemotherapy with: vincristine, melphalan, cyclophosphamide and prednisone. The patient did not respond to the therapy and died one month later. Pleural effusion seems to be an expression of aggressive myeloma. Survival exceeds rarely 4 months.

[Regional portal hypertension revealing malignant adrenal cortical carcinoma]. / Une hypertension portale segmentaire révélatrice d'un cortico surrénalome malin.

The authors report a rare case of regional portal hypertension secondary to splenic vein obstruction by left adrenal carcinoma metastasis. They review the principal literature data concerning regional portal hypertension and adrenal cortical carcinoma.

[Diagnostic and therapeutic aspects of lymphatic tuberculosis: apropos of 42 cases]. / Aspects diagnostique et therapeutique de la tuberculose ganglionnaire: à propos de 42 cas.

We report a retrospective study of 42 cases of lymph node tuberculosis. We noted symptoms of tuberculosis impregnation in 92%, cervical localization in 71%, positive tuberculin intra-dermo-reaction in 77%, and accelerated erythrocyte sedimentation rate in 73% of the cases. Koch bacillus was detected in expectoration, urine or gastric liquid at the rate of 11% of the cases. Lymph node function was suggestive in 4 out of 12 patients, showing giant cells with or without caseum. Lymph node biopsy, performed in 32 patients, was contributive in 94% of them. Another tuberculous localization was found in 14 cases mainly pulmonary (8 cases).