A. Braham

Granulocytic sarcoma of the rectum: Report of one case that presented with rectal bleeding

Granulocytic sarcoma is an uncommon and localized extramedullary tumor composed of immature granulocytic cells. It may present in association with acute myeloid leukaemia, myelodysplastic syndrome and chronic myelogenous leukaemia. Granulocytic sarcoma may occur in any anatomical site but involvement of the gastrointestinal tract is rare, especially in the rectum. We report on the case of a 17 year old female who presented with rectal bleeding, abdominal pain and weight loss one mo prior to admission. Rectosigmoidoscopy revealed a rectal polypoid and ulcerated mass. The histological examination of the mass showed granulocytic sarcoma. Bone marrow examination was compatible with acute promyelocytic leukaemia (FAB type M3). This case report is a reminder of this peculiar sign of tumoral syndrome in acute myeloid leukaemia. We also discuss diagnostic methods and analyze the disease course.

La maladie de Takayasu dans la région centre de la Tunisie. À propos de 27 cas

BACKGROUND Takayasus arteritis is a rare inflammatory disease and few data are available in Tunisia. The aim of this study is to evaluate clinical and radiological features of the disease in the centre of Tunisia. METHODS We retrospectively studied medical records of patients treated in departments of internal medicine or cardiology from three university hospitals in Sousse and Monastir over the period 1985-2005. The criteria for inclusion were those proposed by the American College of Rheumatology. RESULTS Twenty-seven patients were identified. The mean age at presentation was 33.2 years (range 16-68 years) and 88.9% were female. The mean delay from the onset of the symptoms to the time of diagnosis was 4.2 years. Intermittent claudication was the most common presentation (81.5%) and hypertension was noted in 40.7% of cases. Arterial localization most frequently involved was subclavian artery. The aorta was involved in 52.3% and renal arteries in 36.3% of cases. Stenosis or occlusions was constant but aneurysms were noted in 7.4%. Functional difficulty was the main complaint in the follow-up, death related to Takayasus disease was noted in 3.7%. The mean follow-up time was 75.8 months (6.3 years). CONCLUSION There is no epidemiologic particularity of Takayasus disease in Tunisia, however involvement of the subclavian artery was more frequent than the aortic localization.

Noninvasive Fibrosis Markers for the Prediction of Significant Fibrosis in Patients With Chronic Hepatitis C Virus Infection in Tunisia

Summary:Optimized clinical management of chronic hepatitis C requires precise definition of the stage of liver fibrosis. Liver biopsy is viewed as the criterion standard for staging fibrosis in chronic hepatitis, but simple, noninvasive tests for the detection of significant fibrosis would be beneficial. Aim:To evaluate and compare the diagnostic accuracies of the simple fibrosis tests such as aspartate aminotransferase-to-alanine aminotransferase ratio (AAR), platelet count, and aspartate aminotransferase-to-platelet ratio index (APRI) for the prediction of significant fibrosis in patients with chronic hepatitis C. Methods:We studied 38 patients with chronic hepatitis C who underwent a liver biopsy. To evaluate diagnostic accuracies of the simple fibrosis tests, sensitivity, specificity, and the area under the receiver operating characteristic curve were calculated. Results:The AAR increased and platelet count decreased as liver fibrosis worsened, but there was no significant correlation with the fibrosis score. The receiver operating characteristic curves of AAR, platelet count, and APRI were 0.68, 0.38, and 0.91, respectively. The APRI identified patients with significant fibrosis with a positive predictive value of 86.6%. Conclusions:Our study confirms that the APRI can predict significant fibrosis in chronic hepatitis C patients with a high degree of accuracy.

A case of primary biliary cirrhosis associated with pernicious anemia: a case report

Primary biliary cirrhosis is often associated with autoimmune diseases. However, its association with pernicious anemia has rarely been reported.We report a case of a 68-year-old woman who presented jaundice and pruritus. Mildly elevated serum levels of alkaline phosphatase and γ-glutamyl transpeptidase were detected. The titer of anti-mitochondrial M2 anti-body was elevated. Histology of liver biopsy showed features of primary biliary cirrhosis. In addition, aregenerative macrocytic anemia was found in the full blood count. The diagnosis of pernicious anemia was established by megaloblastosis in bone marrow, atrophic gastritis without Helicobacter pylori, low level of vitamin B12 and good response to treatment regimen of vitamin B12. The association of primary biliary cirrhosis and pernicious anemia is unlikely to be casual and may be explained by autoimmune mechanism commonly shared by the diseases.

Takayasu's disease in the center of Tunisia: 27 cases.

BACKGROUND Takayasus arteritis is a rare inflammatory disease and few data are available in Tunisia. The aim of this study is to evaluate clinical and radiological features of the disease in the centre of Tunisia. METHODS We retrospectively studied medical records of patients treated in departments of internal medicine or cardiology from three university hospitals in Sousse and Monastir over the period 1985-2005. The criteria for inclusion were those proposed by the American College of Rheumatology. RESULTS Twenty-seven patients were identified. The mean age at presentation was 33.2 years (range 16-68 years) and 88.9% were female. The mean delay from the onset of the symptoms to the time of diagnosis was 4.2 years. Intermittent claudication was the most common presentation (81.5%) and hypertension was noted in 40.7% of cases. Arterial localization most frequently involved was subclavian artery. The aorta was involved in 52.3% and renal arteries in 36.3% of cases. Stenosis or occlusions was constant but aneurysms were noted in 7.4%. Functional difficulty was the main complaint in the follow-up, death related to Takayasus disease was noted in 3.7%. The mean follow-up time was 75.8 months (6.3 years). CONCLUSION There is no epidemiologic particularity of Takayasus disease in Tunisia, however involvement of the subclavian artery was more frequent than the aortic localization.

409 INDICATIONS FOR LIVER BIOPSY IN HBeAg-NEGATIVE CHRONIC HEPATITIS B VIRUS INFECTION WITH PERSISTENTLY NORMAL ALT. REAL LIFE STUDY

NA therapy in comparison to Peg-IFN is lacking and might provide useful information into viral immune control. Aims: To compare the kinetics of qHBsAg, IP-10 and HBVDNA levels before and during therapy with Peg-IFN and then in Peg-IFN nonresponders (NR) after at least 6 months break (median 15 months) during consequent NA therapy in the same cohort of CHB patients. Patients: 44 monoinfected CHB patients (68%HBeAg+, 20%cirrhotic, median age 37y) were treated with Peg-IFN for 48 weeks. 19 PegIFN non-responders were consequently treated with NA (9 entecavir and 10 tenofovir) for at least 12 months. Methods: Plasma levels of HBsAg were measured by Abbott ARCHITECT assay, HBVDNA by real-time PCR [both log10 IU/ml] and IP-10 levels by ELISA [pg/ml] at baseline, month 6 (M6), M12 and follow-up month 6 (FUM6) for Peg-IFN. All results are presented as medians. Results: During Peg-IFN therapy, baseline HBsAg, HBVDNA and IP10 were similar. HBsAg decline was steeper in responders (n = 17) vs. non-responders (n = 27) during therapy and follow up (M6: −1.03 vs. −0.26; M12: −1.47 vs. −0.39 and FUM6: −0.27 vs. −1.63, all p < 0.01), but IP-10 levels were similar. During NA therapy in 19 Peg-IFN NR, HBsAg decline was similar between Peg-IFN and NA therapy (M6: −0.23 vs. −0.13 and M12: −0.31 vs. −0.28), while IP-10 levels kinetic was different during Peg-IFN and NA therapy in same patients (baseline: 162 vs. 288; M6: 331 vs. 124 and M12: 279 vs. 129, both p < 0.01). Conclusions: Response to Peg-IFN was linked with sharper HBsAg levels decline on treatment. Plasma HBsAg kinetics were similar during Peg-IFN and NA therapy in Peg-IFN non-responders in contrast to differences in IP-10 levels kinetics during Peg-IFN and consecutive NA therapy likely due to disparity in viral control.

Atteinte pancréatique au cours de syndrome d’hyperéosinophilie idiopathique: à propos d’un cas

RésuméLe syndrome d’hyperéosinophilie idiopathique (SHI) est une affection rare, qui constitue un diagnostic d’exclusion. Les manifestations cliniques les plus fréquentes sont cardiaques, pulmonaires, neurologiques et vasculaires. L’atteinte pancréatique est exceptionnelle. Nous rapportons l’observation d’une patiente, âgée de 71 ans, sans antécédents pathologiques, qui se présente pour un amaigrissement, une diarrhée liquidienne, des épigastralgies et un prurit. La biologie objectivait un syndrome inflammatoire biologique, une hyperéosinophilie persistante à 1 900 éléments/mm3, une anémie à 8,6 g/dl et une lipasémie élevée. L’examen parasitologique des selles, les sérologies parasitaires ainsi que les ANCA étaient négatifs. La tomodensitométrie abdominale avait révélé un pancréas tuméfié de rehaussement homogène associé à une densification de la graisse péripancréatique et de l’arrière cavité des épiploons. La biopsie cutanée objectivait une infiltration par des éosinophiles sans stigmates de vascularite. Le myélogramme et la biopsie ostéomédullaire montraient une infiltration par des éosinophiles avec un caryotype médullaire normal. Une polyneuropathie sensitivomotrice a été notée à l’électromyogramme. Le diagnostic du SHI était alors retenu. La patiente a été traitée par corticothérapie (prednisone: 1 mg/kg par jour) avec une bonne évolution clinique, biologique et radiologique.AbstractIdiopathic hypereosinophilic syndrome is very rare. The most common symptoms are cardiac, pulmonary, neurological, and vascular. Pancreatic involvement is exceptional. We report a 71-year-old woman without significant past medical history. She presented for weight loss, osmotic diarrhea, epigastralgia, and pruritus. Laboratory tests highlighted an inflammatory syndrome, a persistent hypereosinophilia at 1,900/mm3, an anemia at 8.6 g/dl, and increased pancreatic enzymes. Stools examination, parasite serology, and serologic testing for ANCA were all negative. The main imaging finding was pancreas hypertrophy and fat infiltration around the pancreatic gland. Skin biopsy showed an infiltration by eosinophils without vasculitis. In bone marrow aspirate and biopsy, we also noted infiltration by eosinophils and a normal karyotype. In electromyography, a motor and sensory neuropathy was found. The diagnosis of idiopathic hypereosinophilic syndrome was made and prednisone was started, with good outcomes in clinical, biological, and imaging parameters.