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Biomedical subjects

A Cosson

Publications and source records attributed to A Cosson.

At least 19 recordsLinked to original sources
PubMed1991

[Hemostasis anomalies and prognosis during severe infectious purpura in children. Retrospective study in 69 cases].

In 69 children with severe infectious purpura (SIP), anomalies of hemostasis on admission were studied retrospectively. Forty-four children presented with disseminated intravascular coagulation (DIC) and 18 with factor VII deficiency +/- thrombocytopenia +/- antithrombin III deficiency. Seven patients were free of hemostasis anomalies. In 5 children, purpura was necrotic on admission, whereas in 9 additional patients skin necrosis occurred subsequently (5 patients with and 4 without DIC). Among the 18 children (26%) who died, 16 were in the DIC group. Factors II and V, fibrinogen and platelet counts were lower in children who died and were correlated with the prognostic score. In the DIC group, however, factor VII + X level was not correlated with the prognostic score and was equally low in fatal and non-fatal cases. Antithrombin III level was markedly decreased in patients who subsequently developed necrosis. These results indicate that factor VII level decreases early in the course of SIP and that consumption of physiologic coagulation inhibitors probably plays a central part in the development of necrotic purpura.

Adolescent↗
PubMed1990

Translocations (5;17) and (7;17) in patients with de novo or therapy-related myelodysplastic syndromes or acute nonlymphocytic leukemia. A possible association with acquired pseudo-Pelger-Huët anomaly and small vacuolated granulocytes.

Twelve patients [two with de novo myelodysplastic syndrome (MDS), four with secondary MDS, five with de novo acute nonlymphocytic leukemia (ANLL), one with secondary ANLL] showed a 17p deletion resulting from translocations involving 17p: t(5;17)(p11;p11) in four cases, t(7;17)(p11;p11) in six cases, complex (5;17)(q23;p12) translocation with dicentric chromosome in one case, and t(17;?)(p11-12;?) in the remaining patient. All these structural anomalies were observed in hypodiploid clones associated with total or partial monosomy of chromosomes 5 and 7 (12 cases), monosomy 12 (five cases), monosomy 3 (four cases), and monosomy 4 (three cases). Median survival was only 3.3 months (range 3 days to 8 months). Striking features were observed in bone marrow mature granulocytes: all but one case had a pseudo-Pelger-Huët anomaly in a significant number of granulocytes, and eight patients had granulocytes with reduced size and clear cytoplasmic vacuoles. Careful cytological review of 51 patients with MDS or ANLL and various cytogenetic anomalies was performed for comparison: vacuolated granulocytes were a very uncommon finding. On the other hand, eight patients had a pseudo-Pelger-Huët anomaly, which correlated significantly with total monosomy 17 in these patients. A possible correlation between cytological anomalies and cytogenetic data is discussed, and the role of 17p in the nuclear segmentation of granulocytes is stressed.

Adult↗
PubMed1990

Prognostic value of dysmyelopoietic features in de novo acute myeloid leukaemia: a report on 132 patients.

The prognostic value of cytological features was assessed in 132 patients with de novo acute myeloid leukaemia (AML) treated by anthracycline-cytosine-arabinoside combination chemotherapy. Of these patients, 98 (75%) achieved complete remission (CR). A significantly lower CR rate was seen in patients with trilineage dysmyelopoiesis (TDMP) (P = 0.003), but not in patients with dyserythropoiesis and/or dysgranulopoiesis without abnormal megakaryocytes. Other unfavourable factors were age greater than 50 years (P = 0.042), leucocyte count greater than 100 x 10(9)/l (P = 0.006), M5 FAB subtype (P = 0.013), presence of complex cytogenetic rearrangement or abnormalities of chromosome 5 and/or 7 (P = 0.001). Bone marrow eosinophilia greater than 3% was significantly associated with a higher CR rate (P = 0.04). In a multivariate analysis, a low CR rate was best predicted by the presence of a complex karyotype or abnormalities of chromosome 5 and/or 7 (P = 0.0001) and by the TDMP (P = 0.0036). Median actuarial disease-free survival (DFS) was 24 months. Actuarial DFS was significantly shorter in patients with TDMP (P = 0.0001) and an elevated leucocyte count (P = 0.02). Age, FAB subtype and karyotype had no significant incidence on DFS. Presence of TDMP appears to be an important prognostic factor in de novo AML. This could be used as one of the guidelines to therapy.

Adolescent↗
PubMed1989

Distinctive features of procoagulant response of monocytes from diabetic patients.

The procoagulant activity (PCA) of disrupted monocytes was examined in 32 diabetic patients (26 with insulin-dependent and 6 with non-insulin-dependent diabetes) versus 30 control subjects. Diabetes monocytes exhibited a weak PCA before any incubation, associated in 10 cases with a significant amount of factor VII activity. Incubation led to a significant rise in PCA in diabetes cells, when stimulated with lipopolysaccharide or not, and in control cells only after stimulation. In incubated diabetes cells, PCA was prothrombinase-like when factor VII was associated with the freshly isolated cells, and tissue factor-like (as in the controls) when no factor VII was associated with the cells. The characteristics of PCA were not correlated with clinical features or with the type of diabetes. Our study suggests that diabetes monocytes exhibit a higher level of PCA than control ones, possibly corresponding to an in vivo stimulation, or at least a higher responsiveness to stimuli occurring in vitro.

Adult↗
PubMed1989

Cytological and ultrastructural assessment of free crystals or precipitates associated with pseudoleukocytosis and pseudothrombocytosis in cryoglobulinemia.

In 3 patients with cryoglobulinemia (case 1: type I: cases 2 and 3: type II) spurious leukocyte and platelet counts were seen using automatic particle counters, with up to 2.5X and 3X counts for leukocytes and platelets respectively, with peculiar volume histograms. All these anomalies were absent with manual counting and were still evident in warmed blood samples from 1 of the 3 patients. The corresponding blood smears showed numerous deposits, rectangular in the type I cryoglobulinemia and flake-like in the other two (type II) patients, responsible for the erroneous automatic counting. Ultrastructural study showed in cases 2 and 3 they consisted of dense amorphous protein clusters; and in case 1, a periodic disposition of hollow rods (each with 21-24 nm external diameter). Comparison with ultrastructure descriptions of isolated cryoglobulins from the literature showed that crystals made of hollow rods might be preferentially associated with monoclonal GK immunoglobulin. Close scrutiny of histograms from automatic cell counters and stained blood films is necessary to detect falsely elevated counts in patients with cryoglobulinemia, and in the management of suppressive therapy.

Adult↗
PubMed1988

Lupus anticoagulant: a clinical and laboratory study of 100 cases.

The clinical and laboratory features of 100 patients with lupus anticoagulant (LA) are reviewed. Subjects were divided into three groups according to their age (1-5, 15-35, 45-89 years). Female prevalence was observed in each group and overall F/M ratio was 3/1. An underlying autoimmune disease (principally lupus erythematosus) was found in 47 cases (10% of the children, 80% of the 15-35-year-old patients and 37% of the elderly patients). Biological criteria for the LA diagnosis were prolonged activated partial thromboplastin time and diluted thromboplastin time (1.3 x control), not corrected after addition of control to patient's plasma. Thromboplastin time was normal in 77 patients. Other types of coagulation inhibitors were eliminated by specific factor assays (with a 10-fold increase of cephalin concentration when necessary). Twenty-three thrombotic episodes were observed. No significant difference was found in the incidence of thrombosis between the autoimmune and non-autoimmune disease group, but the age when first thrombosis occurred was clearly lower in the former. Fourteen obstetrical accidents were noted in eight women but 13 pregnancies terminated without accident. Four patients experienced haemorrhagic complications; they all presented with a severe thrombocytopenia associated with the LA. In our experience, LA is a frequent coagulation abnormality, associated in about half of the cases with a clearly defined autoimmune disease. Clinical presentation appears as notably different according to the patient's age; it is particularly noteworthy that in nine out of 10 children, LA disappeared spontaneously within 6 months.

Adolescent↗
PubMed1988

Refractory anaemia according to the FAB classification: a report on 69 cases.

Between 1980 and 1986, we diagnosed refractory anaemia (RA), according to the FAB classification, in 69 patients, who constituted 22% of the 312 cases of myelodysplastic syndromes (MDS) seen over that period. The haematological features were variable, with pancytopenia in 14 cases (20%), bicytopenia in 24 (36%) and mono-cytopenia in the remaining patients, including 21 (30%) cases of anemia alone, 8 (12%) cases of refractory neutropenia and 2 (3%) cases of refractory thrombocytopenia. Myelodysplastic features were also quite variable, involving one, two or all three lineages. In patients with a single cytopenia or only one dysplastic lineage, FAB criteria appeared insufficient for adequate inclusion among RA and we suggest more precise diagnostic criteria, resulting from the utilization of cytogenetics, ferrokinetics, progenitor cultures and perhaps molecular biology, in such cases. Median survival was 42 months. 12 patients (17%) progressed to RAEB (of whom 7 finally developed ANLL) and 4 patients (6%) to CMML. In spite of the heterogeneity of haematological features, only two factors were associated with poor prognosis, namely age greater than 70 yr at diagnosis and haemoglobin less than 10 g/dl, whereas, to a lesser extent, neutropenia was associated with progression to RAEB.

Adult↗
PubMed1988

Congenital acute monoblastic leukemia with double translocation (8;16) (p11;p13) and (16;20) (q13;p13).

A new case of congenital acute monoblastic leukemia (AML5) with a rare (8;16) translocation is described: this translocation (or a variant) was previously reported only in 4 infants and 10 older children or adults, practically always in association with AML5 or acute myelomonocytic leukemia. The authors discuss the possible causative implication of band 8p11 with some cytological particularities, i.e. erythrophagocytosis by some blasts and strong peroxidase and esterase activities, observable here and in most of the cases with the t(8;16). The t(8;16) may be added to the other chromosomal abnormalities of congenital AML5.

Chromosome Banding↗
PubMed1987

Three cases of translocation (8;16)(p11;p13) observed in acute myelomonocytic leukemia: a new specific subgroup?

In three cases of acute nonlymphocytic leukemia we observed a translocation (8;16)(p11;p13); in one case it was the sole karyotypic change and in the other two cases it was associated with other structural anomalies. All three cases were nonhyperleukocytic myelomonocytic leukemias with erythrophagocytosis by some blast cells and cytochemistry results consistent with leukemic proliferation of a common monocytic-granulocytic precursor. The importance of this translocation is discussed, and the implication of band 16p13 in myelomonocytic leukemia is stressed.

Adolescent↗
PubMed1987

Total peroxidase deficiency in eosinophils: a report on twin sisters, one with a refractory anaemia.

A total lack of EPO was fortuitously discovered in a 63-yr-old woman from the north of France who had primary refractory anaemia, but without dysgranulopoiesis; MPO activity was normal. Her twin sister, whose blood count was normal, also had EPO deficiency. This familial disorder was first described in the Israeli Jewish population and is very rare in Caucasians; it seems to have no pathological consequences. Microscopic studies showed no anomaly except a negativity of Sudan Black B staining which we consider to be a sign of peroxidase deficiency. Ultrastructural studies of the granules revealed normal cristalloid, but the matrix, which contains EPO in normal eosinophils, was very thin; the nature of the relation between functional and morphological anomalies has still to be elucidated.

Anemia↗
PubMed1987

[Cytological diagnosis of myelodysplastic syndromes. Apropos of 200 cases].

The authors present a cytological study of 200 cases of myelodysplastic syndromes (MDS) [corrected], classified according to the FAB cooperative group. This analysis involves the conventional parameters of the peripheral blood and bone marrow differential counts, the systematic recording of signs of dysmyelopoiesis on the erythroblastic [corrected], granulocytic and megacaryocytic lineages and the assessment of blood granulocytes myeloperoxidase (MPO). On the outside of the typical acquired idiopathic sideroblastic anaemia (severe, isolated anaemia associated with intense dyserythropoiesis), the diagnosis of MDS requires very astute cytological interpretation: indeed, when the dysgranulopoiesis is pronounced, determination of peripheral blood and bone marrow differential count is especially difficult. Conversely when the dysmyelopoiesis is slight, a systematic search of morphology signs [corrected] of its presence must be done and associated with functional studies such as MPO activity technically easy to realize.

Adult↗
PubMed1986

[Inhibitors of factor VIII in non-hemophilic patients. Biological and therapeutic aspects. Apropos of 3 cases].

A coagulation inhibitor of the anti-factor VIII: C type was detected in three non-haemophilic male patients aged 75, 70 and 52 respectively. In all three patients antibody titres were low (less than 12.5 Bethesda units initially, less than 20 units subsequently), and a low but detectable level of factor VIII: C persisted (7 to 12 p. 100 in two patients who had severe haemorrhages and 2.100 in the third one). The 3 inhibitors inactivated factor VIII: C with a complex, type II kinetics (Biggs et al.). Strong doses of anti-haemophilic A fractions were biologically effective in one patient but could not stop severe bleeding. Activated plasma fractions were used successfully on several occasions. Once, moderate and repeated doses of anti-haemophilic A fractions resulted in satisfactory correction of factor VIII: C level, and a minor surgical operation could be performed. An immunosuppressive treatment was administered for 3 weeks to one patient and for 3 months to the other two patients. In all three cases the inhibitor disappeared after 5 to 8 months. In non-haemophilic patients with factor VII: C inhibitor the treatment of haemorrhagic episodes must take into account the severity of bleeding, then the usually complex kinetics of the inhibitor; thus it cannot be a direct copy of the treatment used in haemophiliacs with type I inhibitors.

Aged↗
PubMed1986

[Successes and failures of the activated partial thromboplastin time in the preoperative evaluation].

In a prospective study assessing haemostatic functions, the activated partial thromboplastin time was prolonged in 134 out of 10,229 patients studied, without an increase in the prothrombin or thrombin times; this abnormality persisted in only 37 of them on a new blood sample. A retrospective analysis was made of 265 patients who had such an isolated prolongation of the activated partial thromboplastin time on two successive blood samples: the causal abnormality remained unexplained in 135 patients; a well defined coagulation disorder without abnormal bleeding tendency was present in 110 patients (1 severe factor XII deficiency, 58 partial factor XI or XII deficiencies and 51 lupus anticoagulants); a bleeding disorder was diagnosed in 20 patients (8 haemophilias, 8 Von Willebrand's diseases, 4 factor VIII inhibitors). The well-iron efficacy of the activated partial thromboplastin time for detecting coagulation abnormalities is counter-balanced by some disadvantages such as the delay for biologic conclusions. In the preoperative assessment of haemostatic functions, rather than taking a routine approach, it would seem better to determine for each patient the need and the extent of biological testing according to the type of planned surgery, the clinical status of the patient and possible bleeding symptoms.

Blood Coagulation Tests↗
PubMed1985

[Hematologic changes induced by extracorporeal circulation].

The plasma and cellular changes seen during the use of extracorporeal circulatory circuits define the system's degree of haemocompatibility. Heparin is still very much used to prevent activation of the blood clotting mechanisms and to reduce their effects. The fall in concentration of the clotting factors and their inhibitors is usually moderate; it is due to haemodilution, particularly important in cardiac surgery and during plasma exchanges. Fibrinolysis is often activated. In cardiac surgery, it is seen in nearly 20% of cases straight after the end of the ECC, and in nearly 80% of cases during the ECC. In all cases of resin haemoperfusion, there is an early transitory fibrinolytic burst, seen only rarely during haemodialysis and plasma exchanges. This phenomenon is usually well controlled by the natural inhibitors; it can be prevented by antifibrinolytic drugs. Cellular changes show the same trends during cardiac surgery, haemoperfusion and haemodialysis. Thrombopaenia is seen within a few minutes starting of ECC. It is caused by platelet activation, with aggregate formation; these are then trapped by the microcirculation. Leukopaenia occurs at the same time, later followed by rebound; complement activation could be the main cause by forming aggregates of polymorphonuclear cells and monocytes. Intravascular mechanical haemolysis reaches significant levels only in a few cardiac surgical procedures. The great speed of activation of the plasma and platelet enzyme systems by the ECC circuits explains these early changes. They are not only due to direct effects of the physiological circulatory characteristics and to contact with artificial surfaces, but also to plasma-cell interactions and to the patients' reaction to these first alterations.

Blood Cell Count↗