Nouvelle revue francaise d'hematologie最新文献

Erythrocyte aggregation is a physiological phenomenon and constitutes one of the most important factors accounting for the non-Newtonian properties of normal human blood. Pathological aspects have also been described and therapy aimed at reducing hyperaggregability has been proposed. The object of this study was to establish normal values of erythrocyte aggregation parameters as measured by laser light backscattering and to study the influence of various physiological factors. Normal values were determined from a reference population. Sex and age induce variations in erythrocyte aggregation which are neither fibrinogen nor haematocrit dependent and there is a general trend towards stronger aggregation in women, although neither hormonal state nor oestroprogestative treatment appear to influence the female aggregation parameters. In elderly people stronger aggregation is also observed but this effect is of lower magnitude. In vivo, the plasma fibrinogen level is the most important factor influencing erythrocyte aggregation, while variations in haematocrit play a lesser role and mean corpuscular volume, red cell distribution width and white blood cell and platelet counts have no effect. Finally, no difference is noted in cigarette smokers.

Cerebral aspergillosis has a very poor prognosis. When this complication occurs in the immunocompromised host, evolution is virtually fatal in all cases despite surgical and medical treatment. We describe in this report the case of a child with acute lymphoblastic leukaemia who developed pulmonary aspergillosis, and subsequent cerebral dissemination during therapeutic induction. Due to multifocal cerebral lesions, surgery was impossible. The patient was administered long term treatment including amphotericin B, flucytosine and itraconazole for 9 months, during which time a neutropenic period occurred with reactivation of cerebral mycotic lesions, in spite of modification of antileukaemic therapy. Seven years later, he nevertheless remains in complete remission without any neurological sequelae. Thus cerebral aspergillosis requires early diagnosis and can be treated using a strong combination of antimycotic drugs (amphotericin B, flucytosine and itraconazole) on a long term basis, even when aspergillomas cannot be removed surgically. Antileukaemic therapy must be concomitantly adapted to avoid or limit neutropenia.

In the present study, the response to last salvage chemotherapy was analysed in a series of 30 patients with poor prognosis Hodgkin's disease having received high dose chemotherapy followed by autologous bone marrow transplantation. The probability of survival was 43% at 152 months for the 21 chemosensitive patients as compared to 11% at 36 months for the 9 chemoresistent patients. Two toxic deaths occurred, both in the group of chemoresistant subjects, while the probability of absence of disease progression was 65% at 152 months in the 21 chemosensitive cases. According to these results, the response to the last conventional therapy before grafting is an important prognostic factor for survival and absence of disease progression after transplantation. Patients with chemoresistant Hodgkin's disease should benefit from new therapeutic approaches in the context of phase I or II clinical trials.

Although clotting factor concentrates prepared from large pools of plasma are considered to be safe for enveloped viruses (HIV, HBV and HCV), these products are likely to remain at risk for non lipid enveloped viruses. The prevalence of positive IgG serology for human parvovirus B19 (HPV-B19) was determined in 53 children suffering from haemophilia A, who had been treated from birth with only one brand of a highly purified factor VIII concentrate prepared by ion exchange chromatography with a solvent-detergent step (HPSD-VIII). HPV-B19 seropositivity ranged from 20 to 43% in 198 age matched controls. In contrast, the incidence of positive HPV-B19 IgG serology varied from 75 to 100% in haemophiliacs and all 10 severe haemophilia A patients under 3 years of age were seropositive. The presence of HPV-B19 in this clotting factor concentrate demonstrates that at least a proportion of such products continue to transmit non lipid enveloped viruses.

Haematological features of 64 patients suffering from non operable cyanotic congenital heart disease (CCHD) treated with hydroxyurea (HU) were compared with those of 43 patients suffering from the same disorder who had not yet received this drug. Patients with subclinical renal dysfunction were excluded by measuring plasma creatinine levels. MCV and HbF were higher among patients receiving HU, the increase in MCV being cumulative with HU dosage but the rise in HbF dose independent. HbF response to HU was found to be due to the coordinated increase in F-cell and F-reticulocyte production rather than to a selective survival of F-cells. Absence of a relationship between plasma erythropoietin and HbF levels excluded a dominant role of the former in increasing F-cell production and results determined after doubling the HU dosage or immediately after initiating therapy suggested genetic differences to be responsible for the individual variations in Hb F response. No irreversible toxic effects or malignancies were noted in this series of patients. HU was administered for a relatively long period of time, the mean duration of treatment exceeding 5 years, while the study also included patients below the age of 10 years.

Two new cases of central nervous system (CNS) large cell lymphoma without evidence of systemic lymphoma in patients with chronic lymphocytic leukaemia (CLL) are reported. This unusual presentation of Richter's syndrome emphasizes the necessity to evoke this diagnosis in the case of neurologic symptoms in CLL.

From 1980 to 1993, 39 splenectomies were performed in the Department of Visceral Surgery of Saint-Louis Hospital, in patients referred for myelofibrosis associated with myeloid splenomegaly. The short term morbidity was considerable: 33 serious haemorrhagic, infectious or thrombotic complications including 5 fatal accidents were observed in 18 patients. Severe thrombotic or infectious complications leading to 6 further deaths occurred in 8 patients over the two years following splenectomy, while six cases of acute leukaemia appeared between 6 months and 3 years after splenectomy. In 40% of cases with regular follow-up, the operation did not provide any haematological improvement and all these patients died. Only patients with minimally progressive or stable myelofibrosis and residual marrow activity in isotope studies showed an amelioration of general status with relief of pain and reduction of transfusional requirements. The indication for splenectomy should therefore probably be limited to such cases.

Quantitative data provided by flow cytometers are as yet not fully exploited due to the lack of standardization. However, fluorescence standardization systems are now available which allow the measurement of antigen density on a routine basis and the present review focuses on the interest of such quantitative techniques for the monitoring of haemopoietic malignancies. Antigen quantitation: (i) permits a more objective characterization of positivity, especially in the case of weakly expressed antigens; (ii) facilitates the analysis of complex populations, since certain antigens are expressed at different levels on different cell subsets; (iii) provides new data contributing to a more precise definition of cell differentiation; (iv) is of value to ascertain malignancy from the detection of aberrant antigen densities on putative neoplastic cells; (v) provides additional parameters suitable for the evaluation of residual disease and for the monitoring of immunological therapeutic regimens; (vi) contributes to prognosis. Quantitation of antigen densities should therefore be included in the routine study of haemopoietic malignancies.

In the present paper, we report two cases of normal pregnancy after high dose chemotherapy and autologous stem cell transplantation (ASCT) in two of the 72 women belonging to a group of 188 patients transplanted in our unit for advanced malignant lymphoma. These pregnancies occurred 19 and 33 months after high dose therapy in two women aged 27 and 28, neither of whom had received previous total body irradiation or pelvic radiotherapy. There are several reasons for the relatively low frequency of pregnancies after ASCT. In particular, the median age of the patients is 35 years, most of these women are transplanted in relapse and have received previous pretreatment with alkylating agents and the disease free survival rate does not exceed 40%. The preservation of long term fertility should be taken into account when deciding therapeutic options for young women with curable disease.

An evaluation of the WBC differential given by the analyzer Coulter MAXM was performed in a children's hospital, in order to establish optimal interpretation of the automated MAXM report in pediatrics. The patient group comprised 419 children of age up to 15 years. Assessment was carried out by comparison of the MAXM results with microscopic examination of blood smears, good correlation being observed between the two methods in non flagged samples and those flagged only with an isolated "Imm Grans/Bands 1" message indicating the presence of band cells. Comparison of the MAXM and optical differentials in normal and abnormal samples was performed according to a standard protocol taking into account both suspect flags and quantitative abnormalities defined in relation to age. In this group of 419 children, there were 20 (4.8%) false positive and 8 (1.9%) false negative reports, the 8 false negative results corresponding to either minor morphological (4) or borderline quantitative abnormalities (4). Overall, the instrument was capable of detecting significant abnormalities in blood samples with a sensitivity of 95.5% and a specificity of 91.7%. Although the review rate was 45.3%, the high efficiency (93.3%) suggested that most blood smear reviews led to the discovery of a morphological and/or quantitative abnormality. Furthermore, the review rate was greater in neonates (< 7 days) and infants (7 days-1 year) but closer to those of adult populations in older children. The Coulter MAXM was thus shown to be appropriate for use in pediatrics, despite a higher frequency of blood smear review than in adults, particularly among patients less than one year old.