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N Ben Romdhane

Publications and source records attributed to N Ben Romdhane.

16 recordsLinked to original sources
PubMed2006

FIP1L1-PDGFRA positive chronic eosinophilic leukemia in Tunisian patients.

Hypereosinophilic syndromes (HES) are a heterogenous group of rare disorders characterized by sustained and otherwise unexplained overproduction of eosinophils with organ involvement and consecutive dysfunction. Detection of the FIP1L1-PDGFRA fusion gene or the corresponding cryptic 4q12 deletion in HES supports the diagnosis of chronic eosinophilic leukemia (CEL) and provides a molecular explanation for the pathogenesis of this disorder. We screened seven Tunisian patients fulfilling the WHO criteria of HES for the presence of the FIP1L1-PDGFRA fusion gene using nested reverse transcription polymerase chain reaction on peripheral blood samples. Four of the seven patients were positive for this fusion gene. Sequence analysis revealed a substantial heterogeneity of the fusion transcripts due to the involvement of several FIP1L1 exons. All patients were male. The median age at diagnosis was 24 years (range, 18-50); one patient had a history of hypereosinophilia of more than 10 years. Two patients had clinically important and symptomatic eosinophilic endomyocardial disease with thrombotic events. Splenomegaly was constant in FIP1L1-PDGFRA positive CEL but not in the other HES patients (only 1/3).

Adolescent↗
PubMed2005

First-line thalidomide-dexamethasone therapy in preparation for autologous stem cell transplantation in young patients (<61 years) with symptomatic multiple myeloma.

Thalidomide-dexamethasone therapy was given in patients (<61 years) with previously untreated symptomatic multiple myeloma. The aim of this study was to assess the efficacy and toxicity of this combination as first-line therapy, and to determine its effect on stem cell collection and engraftment. During first-line therapy, thalidomide and dexamethasone were administered for 75 days (200 mg/day) and 3 months, respectively. The monthly dose of dexamethasone was 20 mg/m2/day for 4 days, with cycles repeated on days 9 to 12 and 17 to 20 on the first and the third month of therapy. After first-line therapy, a collection of peripheral blood stem cells (PBSC) was performed. Between May 2003 and September 2004, 60 patients were included. On an intent-to-treat basis, the overall response (> or =partial response) rate was 74%, including 24% of patients who obtained a complete remission. Grade 3-4 toxicities consisted of infections (12%), deep-vein thrombosis (3%), constipation (5%), and neuropathy (5%). A total of 58 patients (96%) proceeded to PBSC mobilisation and yielded a median number of 8 x 10(6) CD34+ cells/kg. First-line thalidomide-dexamethasone therapy is effective and relatively well tolerated in young patients with symptomatic multiple myeloma. This combination does not affect PBSC mobilisation.

Adult↗
PubMed2005

Prevalence of inherited prothrombotic abnormalities and central venous catheter-related thrombosis in haematopoietic stem cell transplants recipients.

In this prospective study, we assessed the incidence of central venous catheter (CVC)-related thrombosis in haematopoietic stem cell transplant (HSCT) recipients. We determined the contribution of inherited prothrombotic abnormalities in blood coagulation to CVC-related thrombosis in these patients. The study was conducted between May 2002 and September 2004. CVCs were externalized, nontunneled, polyurethane double lumen catheters. Before catheter insertion, laboratory prothrombotic markers included factor V Leiden, the prothrombin gene Gly20210A mutation, plasma antithrombin levels, and protein C and S activity. All patients were systematically examined by ultrasonography just before, or <24 h after, catheter removal, and in case of clinical signs of thrombosis. A total of 171 patients were included during the 28-month study period. Five (2.9%) and three (1.7%) patients had evidence of protein C and protein S deficiency, respectively. Only one patient had an antithrombin deficiency (0.6%). In total, 10 patients (5.8%) were heterozygous for the factor V Leiden mutation, and one patient had heterozygous prothrombin G20210A mutation (0.6%). We observed a CVC-related thrombosis in 13 patients (7.6%). Thrombosis was diagnosed in four out of 20 patients (20%) with a inherited prothrombotic abnormality compared to nine of 151 patients (6%) who did not have a thrombophilic marker (relative risk 3.3 CI 95% 1.1-9.9). Our results suggest that inherited prothrombotic abnormalities contribute substantially to CVC-related thrombosis in HSCT recipients. In view of physicians' reluctance to prescribe prophylactic anticoagulant treatment in these patients, a priori determination of inherited prothrombotic abnormalities may form a basis to guide these treatment decisions.

Blood Coagulation Factors↗
PubMed2000

[Costal hydatid cyst (report of 2 cases)].

Hydatidosis is an endemic affection in our country. Hepatic and pulmonary localisation is the most frequent. The other localisation especially in the bone, are exceptional. We report 2 cases of costal hydatid diagnosed on 17 and 33 years old male patients admitted for parietal tumor respectively localized in cervix and thorax. The diagnosis, suspected on chest tomography is confirmed by histologic patterns of resected rib.

Adolescent↗
PubMed1999

[Arterial aneurysms and Behçet's disease: apropos of 3 cases].

PURPOSE: Results that were obtained from three patients and a literature review allow the authors to better define the place of medical and surgical treatments according to the different locations of arterial aneurysm occurring in the course of Behcet's disease. METHODS: Retrospective report of five cases of arterial aneurysm that occurred during Behcet's disease in three patients. RESULTS: Due to the unusual symptoms, diagnosis can be hampered. Though lesions may be observed in various areas, they were mostly aortic (two patients). Surgical revascularization was done in all three patients. It mainly involved prosthetic grafts. Two patients were subsequently treated by postoperative immunosuppressive drugs. The mean follow-up was 22 months. Relapsing aneurysm was observed in one patient, while in another patient aneurysm developed in a new location. CONCLUSION: These types of arterial lesions respond poorly to medical treatment when a combination of colchicine and intermittent intravenous bolus of cyclophosphamide is used. Surgical indications should be given great yet selected weight, using prosthetic rather than venous autologous grafts (due to the risk of spontaneous venous involvement). Since either complication or recurrence is possible, prolonged monitoring is required.

Adult↗
PubMed1990

[Systemic mastocytosis. A case report].

The authors report a case of systemic mastocytosis in a 65 year-old woman. The clinical picture was generalized abdominal and extra abdominal lymph nodes, ascites, splenomegaly, and hepatomegaly. As gastro-intestinal symptoms led to suspect a digestive malignant lymphoma; the patient underwent an exploratory laparotomy. The diagnosis of systemic mastocytosis was established by demonstrating an excessive number of mast cells in the cutaneous and extracutaneous tissues (liver, mesenteric lymph, nodes, spleen and bone marrow). Bone marrow biopsy revealed extensive myelofibrosis.

Aged↗