[Dysfibrinogenemia and thrombosis. A case report]

Congenital dysfibrinogenemia is a functional disorder of the fibrinogen that represents a rare cause of thrombophilia. To report a Tunisian case of the association dysfibrinogenemia and thrombosis. A woman with ingerited dysfibrinogenemia associated with mild tendency to bleeding experienced a deep vein thrombosis of the lower-extremity at 26 years of age and a fatal pulmonary embolism a few years later. Paradoxically coagulation function of fibrinogen was markedly altered in vitro with a significally prolonged prothrombin time, activated partial thromboplastin time and thrombin time, a functional fibrinogen level that was undetected and a severely impaired fibrin polymerization. The thromboembolic events in the patient could be related to dysfibrinogenemia since the main causes of thrombophilia were excluded. Although it is rare, this cause of thrombophilia must be misdiagnosed, systematic measuring of prothrombin time, activated partial thromboplasting time and functional fibrinogen might be helpful

Sweet syndrome in my myeloid malignancy

Sweet's syndrome is an acute febrile neutrophilic dermatosis. This condition is characterized by the sudden onset of fever and tender, erythematous, well-demarcated papules which show dense neutrophilic infiltrates on histological examination. Sweet's syndrome is often associated with hematologic disease including myeloid leukemia. Aim: Report of news cases of Sweet's syndrome. We report two cases of Sweet's syndrome occurring respectively after the use of Granulocyte-Colony Stimulating Factor [G-CSF], and the All-Trans-Retinoic-Acid [ATRA] in two females aged respectively 46 and 35 years. The outcome was favourable in the two cases

Bacterial flora and antibiotic resistance in a clinical haematology unit

Infections are among the most serious complications in neutropenic patients and are associated with an increased morbidity and mortality. Ongoing surveillance of infection in neutropenic patients is essential to detect changes in epidemiology and to guide better empirical antibiotic regimens and infection control policies. The aim of this study is to analyze the bacterial flora and the antibiotic resistance of isolates in a clinical haematology unit during three years period. From 1 January 2003 to 31 December 2005, 437 strains were isolated from different specimens. Antimicrobial susceptibility testing has been carried out by disk diffusion method as referred to the French Society of Microbiology. All susceptibility data were stored in a laboratory data base using Whonet software. Duplicate isolates defined as the same bacterial species for the same patient with the same profile of susceptibility were excluded. Gram negative bacilli [GNB] rate was 47.1% and Gram positive cocci [GPC] rate 52.9%.The most frequently identified species were coagulase negative staphylococci [CNS]: 29.3%, Escherichia coli:14%, Staphylococcus aureus: 10.7%, Klebsiella pneumoniae: 9.1% and Pseudomonas aeruginosa:7.5%. The global rate of methicillin resistant staphylococci was 27.7% for S. aureus and 61.4% for CNS, no GISA [glycopeptide intermediate S.aureus] was detected during the study period. For E. coli, the frequencies of resistance to ceftazidime, ciprofloxacin and amikacin were respectively: 45%, 26. 3% and 21.3%.Concerning K. pneumoniae, 84, 8% of strains were resistant to ceftazidime and were producing extended spectrum,-lactamase [BLSE]. The trends of resistance showed an increasing rate of K. pneumoniae BLSE: 57.1% in 2003 versus 95.5% in 2005. However; all isolates remained susceptibles to imipenem and colistin. Concerning P. aeruginosa, 50% were resistant to ceftazidime, 50% to imipenem, 51.6% to ciprofloxacin and 54.5% to amikacin. An increasing rate of imipenem resistance in P. aeruginosa was observed from 2003 to 2005[28. 6% in 2003 versus 45. 5% in 2005]. Following this study, a restriction use of ceftazidime [substituted by piperacillin-tazobatam] was instaured in the unit. A further study should be conducted to evaluate the impact of piperacillin-tazobactam as a first line treatment in neutropenic patients

Cytology and immunophenotype feautures of 80 acute myeloid leukemia

Acute myeloid leukemia [AML]'s diagnosis is clinical and biological. We report here 80 AML with cytology and immunophenotype features to establish correlations. 21 AML1, 23 AML2, 12 AML3, 2 AML4, 18AML5 and 3 AML6 were diagnosed by cytology. Only one case of AMLO was diagnosed by immunophenotype. Myelogysplasia is present in 29,8% cases. CD 19 and CD56 expression was significantly associated to AML +t[8;21]. Additionally, concomitant negativity of CD34 and HLA-DR was dis-crimininatif to AML3 diagnosis. Prognostic value to expression some CD needs time backwards

[Alpha interferon in children with philadelphia chromosome-positive chronic myeloid-leukaemia]

The present work focuses on the therapeutic efficacy and the toxicity of alpha interferon in patients younger than age 18 years. 5 patients younger than 18 years were treated and followed up between 1990 and 1999 at the department of haematology [Aziza Othmana Hospital] Hydroxyurea was given as initial treatment to all patients. After a median period of 8 months. these patients received alpha interferon [5 millions units/m 2 once]. Six months after the beginning of the alpha interferon a complete hematologic response was obtained in all patients. The median overall survival was of 66 months: 3 patients are still alive [2 patients in an advanced stage and one patient in chronic phase] and 2 patients died after transformation. The most common reported side effects of alpha interferon were asthenia, weight loss, fever, myalgia, chills and headaches - these toxic manifestations were mild and were noticed in all our patients. MyeIosuppression was noted in two patients. Interferon is well tolerated in patients younger than age years 18 old, with CML.It may offer an alternative to bone marrow transplantation in children in the chronic phase of CML without histocompatible donor. The role of new agents such as STI 571 needs to be evaluated as well

[Long term survival and prognostic factors in multiple myeloma treated with conventional chemotherapy. About 109 cases]

Our study is retrospective. We report the results of conventional chemotherapy in previosly untreated patients with myeloma. Survival and progonstic factors were analysed in 109 patients diagnosed from 1983 to 1992. The median age was 65 years, 87 patients [80%] were including in the stage III according the Durie Salmon staging system. The median survival time was 27 months and 10 years survival rate is 3,66%. In the univariate analysis, two prognostic variables were retained namely the hemoglobin and creatinine level. The study suggest that conventional therapy is agood treatment for old patients. However, patients younger than 55 years, must benefit from intensive chemotherapy supported by autologus bone marrow, pheripherol blood stem cells, or allogenic bone marrow transplantation. A considerable encrace in duration of remission and survival is possible