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Biomedical subjects

B Jude

Publications and source records attributed to B Jude.

At least 73 records · Page 4Linked to original sources
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

Acquired type II von Willebrand's disease: demonstration of a complexed inhibitor of the von Willebrand factor-platelet interaction and response to treatment.

An acquired von Willebrand's disease developed in two patients in association with a monoclonal gammopathy plus a Sjögren's syndrome and a chronic lymphocytic leukaemia (CLL). In both cases a plasma inhibitor to von Willebrand factor (vWf) was suspected and characterized after plasma gel filtration. The inhibitor was shown to be entirely complexed with vWf and was only demonstrated after complex dissociation by heating. The inhibitor was able to inhibit the binding of 125I-vWf to platelets in the presence of ristocetin in both cases and to thrombin-stimulated platelets in one case. In the two patients, the highest molecular weight multimers (HMWM) of vWf were absent when assessed by sodium dodecyl-sulphate agarose plasma electrophoresis. Intravenous infusion of 1-deamino-(8-D-arginine) vasopressin (DDAVP) resulted in the appearance of the HMWM in both cases and of the satellite bands of each multimer subunit which were lacking prior to the infusion in one patient. After transfusion of a VIII/vWf concentrate containing a significant amount of HMWM, there was a rapid plasma clearance of the vWf-related activities and of the HMWM when compared to that seen in a patient with type III constitutional vWD. We conclude that in the two patients studied the coagulation defect was related to the presence of a circulating inhibitor to vWf which could be responsible for the disappearance of the HMWM from plasma.

Aged↗
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↗
PubMed1987

[Severe pulmonary embolism disclosing a deficiency in protein C].

A constitutional deficit in protein C is a rare disorder. Our report concerns a 35 year old non-smoking male, presenting with a past history of uncomplicated phlebitis of the lower limbs. In the course of a new episode of phlebitis, the patient presented with a severe pulmonary embolus treated by embolectomy under extracorporal circulation, with interruption of the inferior vena cava. The plasma level of protein C (Elisa technique) measured before treatment with antivitamin K was 40% (normal 70-140%). The absence of associated hepatic or haematological anomalies confirmed the constitutional character of the deficit. The immediate and subsequent progress turned out favourably on treatment with heparin, then anti-vitamin K.

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↗
PubMed1982

["Lymphoid" blastic transformation in chronic myelogenous leukemia. Report of three cases (author's transl)].

Three cases of chronic myelogenous leukemia (CML) were studied, occurring in 22, 43, and 30-year-old-men. Two observations (nos. 1 and 3) concerned typical CML, treated by discontinuous busulfan; in the last patient (no. 2), also presenting in addition with a constitutional deficiency from Hageman factor, diagnosis (Ph 1 chromosome) was based on a moderate leukocytosis with myelemia, spontaneously regressive for more than one year. Chronic phase duration was 17, 16 and 8 months respectively. During the first blast crisis, abnormal cells were rather of granular type in one case (no 1), undifferentiated in the other two. In the three observations, complete remission was easily obtained with prednisolone - vincristine but revealed very brief; 2, 2 and 4 months. Among the three patients a second blast crisis was preceded, in two cases (nos. 1 et 3), by a new CML phase during 3 and 1 months respectively. Treatment was then purely palliative by 6-mercaptopurine and hydroxyurea.

Adult↗
PubMedDate not supplied

[Constitutional thrombophilias: indications of the biological profile and therapeutic consequences].

In laboratory screening in patients with clinical thrombophilia (early thromboembolism episode < 50 years, spontaneous thrombosis, recurrent thrombosis, unusual site of thrombosis, thrombotic family history or coumarin-induced skin necrosis complication), an isolated or combined inherited thrombophilia can be observed: antithrombin (0.5 to 4.9 per cent), protein C (1.4 to 8.6 per cent) and protein S (1.4 to 7.5 per cent) deficiencies or factor V Leiden (20 to 30 per cent). Special attention is mandatory in prescribing biological exploration because of the many physiological or pharmacological interferences which can modify the results. Identification of a genetic defect may induce specific management and individuals should receive counselling regarding the implications of this diagnosis. Further prospective studies should help to determine the thrombotic risk in symptomatic and non-symptomatic patients with inherited thrombophilia and the risk/benefit ratio of laboratory screening for hereditary thrombophilia and therapeutic intervention.

3' Untranslated Regions↗
PubMedDate not supplied

[Therapy of bacterial infections occuring during aplastic phasis of acute leukemias (author's transl)].

On a three years' period, 122 patients with AL (45 children, 76 adults) received 158 treatments of induction, involving a severe medullary aplasia. In 48 cases (30.4%) none infectious complication was recorded. In the other 110 observations, infections syndrome (septicemia due to gram-negative rods above all) was treated by an early and intensive combination antibiotherapy (three successive protocols are employed, after failure of the precedent), in association with a careful medical reanimation and trasfusions of granulocytes in 16 cases. Regression was obtained in 94 cases (59,5%) but infection was lethal in 16 patients (10.1%). More than cytological variety of AL, principal pronostic element is age (15 deaths among 88 infections episodes in adults, one among 22 in children). Granulocytes rate at the time of aplasia (lower than 500 /mm3) also represents a factor of gravity. Among 94 infections episodes, antibiotherapy was successful alone in 81 cases (86.2%), illustrating the fundamental place of this treatment.

Acute Disease↗
PubMedDate not supplied

[Protein C deficiency and vascular thromboses. Apropos of 2 cases and a review of the literature].

Protein C, a physiological inhibitor of coagulation, acts by inactivating coagulation factors V and VIII. It was identified 20 years ago and purified 10 years later. Its anticoagulant properties have been confirmed by the demonstration of thromboembolic diseases associated with constitutional protein C deficiency. Deficiency is defined as a less than 65 p. 100 level of the protein. There is no correlation between protein C level and clinical severity. Constitutional protein C deficiency is transmitted as an autosomal dominant trait. The protein C level observed in homozygous deficiency is about 50 p. 100, more often quantitative (type I) than qualitative (type II), the other coagulation factors being present at normal levels. Protein C deficiency is responsible for recurrent and familial thromboembolic necrosis and for cutaneous necrosis during treatment with antivitamin K drugs. Protein C assays must now be part of the aetiological evaluation of thromboembolic disease. Physiological variations in protein C levels have been encountered in neonates and pregnant women as well as in some pathological conditions, after surgery or under certain treatments. Familial inquiries are essential to detect asymptomatic protein C deficient subjects. Treatment rests on anticoagulants: antivitamin K drugs after effective heparinization in thromboembolic accidents, prevention of accidents by heparin in protein C deficient subjects and when a risk of thromboembolic disease is present. We report here one case of venous thrombosis and one case of arterial thrombosis, both being characterized by the finding of protein C deficiency during full evaluation of haemostasis factors.

Adult↗
PubMedDate not supplied

[Contribution of ABVD chemotherapy in refractory Hodgkin's disease. Study of 21 observations (author's transl)].

On a two years period, 21 patients with advanced Hodgkin's disease who failed usual therapeutic proceedings were treated by a sequential combination chemotherapy with adriamycin, bleomycin, vinblastine and dacarbazine (ABVD, first described by Bonadonna). Initially 16 patients among 21 had a disseminated disease with stage III B (9 obs.) or IV B (7 obs.). Previously all had been submitted, during a four years mean period, to multiple therapeutic trials always involving MOPP plus lymphoid irradiation and/or other combination chemotherapy. In 19 patients among 21, ABVD was decided because of persistent abdominal lymphoid and/or visceral localizations (liver: 6 obs.; lung: 4 9bs; épidural space: 1 obs.). In 12 patients, abdominal localizations were proved after delayed staging laparotomy. Immediate results with ABVD were: CR: 9 obs. (43%); PR: 5 obs. (24%); failure: 7 obs. (33%). With vinblastine interrupted by intermittent ABVD, 4 CR are persisting with a 8 to 23 months' follow up; among 5 patients who relapsed (3 to 24 months), 4 are still alive. After irradiation of résidual lesions, 4 PR among 5 are persisting with a 6 to 12 months' follow up. For the 21 patients, median survival has not been reached at 30 months.

Adolescent↗