[Haematological malignancies (HM) in Basse-Normandie (BN): incidence and clinical and biological characteristics over the period 1997-2005 in patients less than 25 years old]. / Épidémiologie des hémopathies malignes en Basse-Normandie : incidence et caractéristiques cliniques et biologiques chez l'enfant et l'adulte jeune de moins de 25ans (1997-2005).

BACKGROUND: The study was designed to present the incidence of all the haematological malignancies (HM) in Basse-Normandie (BN) over the period 1997 to 2005 in patients less than 25 years old. BN is an administrative region in the North-West of France, composed of three departments: Calvados, Manche and Orne. We extracted data from the Registre régional des hémopathies malignes de Basse-Normandie, a French registry which belongs to the Association of the French Cancer Registries (Francim). METHODS: All the HM were coded using the third edition of the International classification for oncologic diseases (ICD-O-3). We compared the clinical and biological descriptive data in patients less than 15 years old to those of young adults (15-24 years old). RESULTS: A total of 305 new cases of HM were recorded over the period 1997 to 2005. HM were more frequent in men (168 cases) than in women (137 cases). Patients less than 25 years old accounted for 4.1% of all HM cases, whereas patients less than 15 years old and young adults (15-24 years old) represented 2.2% and 1.9% of all cases, respectively. In patients less than 25 years old, the overall world-standardized incidence rates (WSR) were 7.67/100,000 (95% CI: 6.31-9.04) in BN (8.08 [6.15-10.02] for men and 7.24 [5.31-9.17] for women). In patients less than 15years, the overall WSR was 7.38/100,000 (6.23-8.52), with no difference between boys (7.57) and girls (7.17). Acute lymphoblastic leukaemia (ALL) was the most frequent HM, WSR=4.02/100,000 (3.16-4.88) (4.30 [3.08-5.53] in men, 3.73 [2.52-4.93] in women), with similar clinical and biological criteria between patients less than 15 years and young adults. In young adults, the overall WSR was 8.21/100,000 (7.47-8.96), similar between men and women (9.02 [7.93-10.12] and 7.37 [6.35-8.38], respectively). Their highest WSR was obtained for Hodgkin lymphomas (HL): 3.37/100,000 (2.89-3.85), similar between men (3.49 [2.8-4.17]) and women (3.25 [2.58-3.93]). The study did not show any significant difference between the Calvados, Manche and Orne departments (except for HL, which seems more frequent in Manche department for 15-24 years old cases). There was no evidence of an increased risk for ALL in the subdistricts Beaumont-Hague and Les Pieux, which respectively have a nuclear waste reprocessing plant and a nuclear power plant on their territory. The subtype of HM was dependent on age whereas clinical and biological data were the same, whatever the age. CONCLUSION: These results contribute to HM monitoring in an area where the nuclear industry is present and to improve the organization and follow-up of medical care.

Performance evaluation of the body fluid mode on the platform Sysmex XE-5000 series automated hematology analyzer.

We evaluated the performance of the automated body fluid mode of the Sysmex XE-5000 series automated haematology analyzer and compared the performance of the automated method for obtaining white blood cell (WBC), red blood cell (RBC) counts and WBC differential counts with microscopic method. One hundred and seventy-four samples were analysed: 81 ascitic fluid, 32 cerebrospinal fluid (CSF), 26 pleural fluid (PF), 18 synovial fluid (SF), 13 peritoneal fluid (PeF) and 4 other types. The agreement between the automated method and the manual reference showed high correlation, with Pearson correlation coefficients greater than 0.9 for all types of body fluids. We also demonstrate that the automated body fluid analysis on the XE-5000 is an acceptable alternative to the microscopic reference method as far as ascitic fluid, peritoneal dialysis fluid, SF or PF are concerned. Conversely, results for body fluid samples from oncology patients with leukaemia or tumours showed significant differences between both methods, as XE-5000 counted blast cells and neoplastic cells in mononuclear cell count. XE-5000 could represent an attractive method for the automated analysis of WBC, RBC, mononuclear cell count (MNC) and polymorphonuclear (PMN) cells of most body fluids. However, CSFs from patients with leukaemia or lymphoma should be processed with the microscopic reference method in order to detect abnormal leukaemic cells.

[Haematological malignancies: incidence in Basse-Normandie, France, for 1997-2004]. / Epidémiologie des hémopathies malignes en Basse-Normandie.

BACKGROUND: The study was designed to present the incidence of all the haematological malignancies in Basse-Normandie over the period 1997-2004. We extracted the data from the "Registre régional des hémopathies malignes de Basse-Normandie (RRHMBN)", a French registry which belongs to the Association of the French Cancer Registries (FRANCIM). All the malignant haematological diseases were coded using the third edition of the International Classification for Oncologic Diseases (ICD-O-3) and the ADICAP classification, a special version adapted in 2001 for haematology. Five thousand five hundred and ten new cases of malignant haematological disorders were registered over the period 1997-2004. Whatever the department constituting the Basse-Normandie (Calvados, Manche and Orne), no significant difference of incidence was detected. In men, the more frequent malignant disorders were non-Hodgkin's malignant lymphomas (NHML) followed by chronic lymphocytic leukemia and other mature neoplasms, myelodysplastic syndromes (MDS), multiple myeloma (MM), myeloproliferative disorders (MPD), acute myeloid leukemias (AML), Hodgkin's lymphomas (HL), Waldenström's macroglobulinemia (WM) and acute lymphoblastic leukemia (ALL). In women, MM is the third more frequent haematological disorders after NHML and lymphocytic leukaemia and other mature neoplasms, MPD, MDS, AML, Hodgkin's lymphomas, WM and ALL. The other haematological disorders are very rare. We provide the incidence for the main haematological disorders and for the first time we also present the incidence of the different subtypes of the Hodgkin's and non-Hodgkin's malignant lymphomas, mature lymphoid neoplasms, MPD and also MDS. These results are useful for the organization and follow-up of medical care. The development of specialized haematology and active protocols can optimize the management of the older patients. A high quality of the collected data remains necessary for a continuous watching and research on patients with malignant haematological diseases.

Cyclin D1 expression in patients with multiple myeloma.

INTRODUCTION: Chromosomal abnormalities are detected in 50 to 70% of patients with multiple myeloma (MM). By conventional cytogenetic analysis, a t(11;14)(q13;q32) is observed at a frequency of 3 to 14%. MATERIALS AND METHODS: To demonstrate a cyclin D1 expression in MM patients or MM cell lines, 14 patients with multiple myeloma (MM) and nine human multiple myeloma cell lines (HMCL) were screened by a competitive RT-PCR and/or Northern blot analysis for cyclin D1 expression. Furthermore, we screened 10 MM patients with FISH to demonstrate a relationship between the cyclin D1 expression and the presence of the t(11;14). RESULTS: Five HMCL had a cyclin D1 overexpression: three of them had a t(11;14)(q13;q32) and two had extra copies of chromosome 11. A cyclin D1 expression was found at diagnosis in seven out of 14 untreated MM patients (50%). Out of 14 MM patients, FISH studies were performed in 10 patients. A t(11;14) was detected in three out of 10 patients and extra copies of chromosome 11 were found in two additional patients. CONCLUSION: Cyclin D1 expression is a common event in MM patients (50%) and is associated either with a t(11;14)(q13;q32) or extra copies of chromosome 11. The prognostic role of the cyclin D1 expression and the level of this expression, as compared to other B-cell chronic lymphoproliferative disorders such as mantle cell lymphoma or hairy cell leukemia, remains to be determined in the pathogenesis of multiple myeloma.

[Improvement of prescriptions for serum tumor markers in a general hospital]. / Amélioration de la prescription de marqueurs tumoraux sériques dans un hôpital général.

OBJECTIVES: Using a standardized prescription sheet we attempted to improve requests for serum tumor markers in a general hospital. METHODS: Over two 35-day periods before and one year after defining a local prescription consensus and introducing a new prescription sheet, we counted the number of orders for five tumor markers (CEA, CA 19-9, CA 15-3, CA 125, alpha FP) and determined their compliance to the defined prescription protocol. RESULTS: Between the two study periods, the number of prescriptions for the designated tumor markers fell by 24%, from 153 requests in 94 patients to 123 requests in 99 patients, despite a 6% increase in the number of admissions. There was a significant reduction in the number of serum markers orders per prescription (from 1.6 to 1.2) although the distribution by tumor marker remained unchanged. Compliance to the prescription protocol improved, rising from 65 to 87% in units where the pre-protocol compliance rate was below 80%. The rate of compliance was not correlated with correct completion of the new prescription sheet (91% vs 86% respectively). The 6-month cost-savings was estimated at 31,104 FF using the general French nomenclature for laboratory tests. Direct cost reduction was estimated at 5,688 FF. CONCLUSION: Long-lasting improvement of serum tumor marker prescriptions can be achieved in a general hospital. Obtaining a local consensus implicating all prescribing units seems more important than a change in the presentation of the prescription sheet.

Myelodysplastic syndromes in childhood: is the FAB classification relevant? Report of 81 children from a French multicentre study. French Group of Cellular Hematology.

We reviewed the peripheral blood and bone marrow smears of 81 children with myelodysplastic syndrome (MDS). The morphological FAB classification was applicable in 59 children (72.8%): RAEB and RAEBt were the most frequent, 32 cases (39.5%). CMML was observed in 15 cases (18.5%) and in 25% of them, serological evidence for a recent EBV infection was demonstrated. In 22 cases (27.2%), the FAB classification was not convenient. In some of these children, dysmyelopoiesis was associated with constitutional disorders. Among these various inherited conditions, Down syndrome in which myelodysplasia is the expression of an abnormal clonal hematopoiesis, and mitochondrial cytopathies in which MDS is the hematological expression of a polyclonal multi-organ disease. The FAB classification does not appear to be satisfactory for all the disorders included in the group of childhood MDS and should be modified for specific use in children.

Childhood leukemia incidence in the vicinity of La Hague nuclear-waste reprocessing facility (France).

The incidence of leukemia is examined in young people (aged under 25 years) living within a 35 km radius of the French nuclear-waste reprocessing plant operating in La Hague, Normandy. During the period 1978-90, a total of 23 cases was diagnosed, giving an incidence rate of 2.99 per 100,000 which is close to the expected rate. In the 'canton' in which the nuclear plant is located, three cases of leukemia were observed compared with 1.2 expected, giving a standardized incidence ratio of 2.5. This nonsignificant finding is compatible with no increased risk and also with a sevenfold excess risk. Therefore, it reinforces the necessity of conducting a case-control survey, which is now planned, to assess the part played by occupational radiation exposure in leukemia incidence for this French area.

[Treatment of elderly cancer patients]. / Traitement des cancéreux âgés.

Management of cancer in the elderly differs from that in other age groups only as far as concerns practical issues. The gradual deterioration of somatic and psychologic tolerance capacities is often associated with multiple pathological conditions which may delay diagnosis and hinder the implementation of therapy. Major therapeutic risks are mainly ascribable to chemotherapy which requires strict application of contraindications and close monitoring during treatment. Under these conditions, carefully determined by a thorough workup prior to treatment, the elderly patient, although fragile, can nevertheless benefit from adequate and efficient therapy. Although seemingly aggressive in some instances, this active outlook is preferable to systematic palliative therapy chosen only because of age, often constraining and not always useful or safe. Age in itself is not a reason for systematic abandon when significant results can be obtained without unacceptable risks until an advanced age through well-adjusted measures and continuous attention.

[Therapeutic advances in geriatry. A report on 47 cases (author's transl)]. / Apport thérapeutique en milieu gériatrique. A propos de 47 cas.

A group of 47 elderly patients with various ailments and aged between 66 and 99 years were treated with tiapride: very good results were obtained in 7 cases of severe buccofacial dyskinesia, though the dosage had to go as high as 800 mg/day; results were excellent in 2 cases, good in 9, moderate in 5, and nil in 3 out of 19 patients with agitated states; overall results were good (1 failure) in 8 cases of alcoholism (4 acute and 4 chronic); 8 good results and 4 failures were noted in 12 patients with either cancer pain, or pain following zoster or due to arthrosis or migraine. The product was very well-tolerated by these elderly patients.