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Biomedical subjects

E Liozon

Publications and source records attributed to E Liozon.

At least 37 records · Page 2Linked to original sources
PubMed2000

Anticardiolipin antibody levels predict flares and relapses in patients with giant-cell (temporal) arteritis. A longitudinal study of 58 biopsy-proven cases.

OBJECTIVE: To evaluate the usefulness of anticardiolipin antibodies (aCL) in identifying flares and relapses in giant-cell arteritis. METHODS: We studied 58 consecutive patients with biopsy-proven temporal giant-cell arteritis. C-reactive protein and aCL serum levels were measured simultaneously at the time of diagnosis and at each out-patient visit until recovery. All observed episodes of a rise in C-reactive protein attributable to a precise cause, for which the simultaneous measurement of aCL was available, were analysed. RESULTS: The mean duration of clinical observation and serum aCL assessment was 34+/-18 and 24+/-11 months, respectively. Anticardiolipin antibody positivity (IgG or total antibodies > or =20 U) before treatment was found before treatment in 27 cases (46.6%) (mean 45.6+/-26 U/l, range 20-110 U). Levels of aCL decreased below 10 U with appropriate treatment in all patients except one, after a variable delay. No rise in aCL levels was recorded subsequently in any patient whose disease was controlled permanently. A significant rise in aCL was recorded in 20 of 27 (74%) of the flares or relapses of giant-cell arteritis, including seven of 12 flares in seven patients whose initial aCL level was <20 U vs none of the 28 inflammatory episodes unrelated to giant-cell arteritis (P<0.0000001). IgM aCL, infrequently found at diagnosis, was not associated with signs of disease activity. CONCLUSION: Serum aCL levels are useful in the detection of flares and relapses in giant-cell arteritis, with fairly good sensitivity (74%) and a specificity of 100%, and can be of value in distinguishing subclinical flares from infection.

Aged↗
PubMed2000

[Horton's disease and hypereosinophilic syndrome: a non-fortuitous association].

Giant cell arteritis is a T-cell dependent auto-immune vasculitis that involves a T-helper l (Th1) cell response. We report the unusual case of a woman who developed successively a biopsy-proven temporal arteritis without tissue eosinophilia at age 65, an isolated blood eosinophilia at age 69 and an hypereosinophilic syndrome at age 71, while still taking corticosteroids at the dose of 3mg/day. Considering this temporal relationship, some characteristics of her hypereosinophilic syndrome pointing to a Th2-dependent disease (absence of definite signs of myeloproliferative syndrome, elevated IgE levels and quick normalisation of eosinophil counts under corticosteroid therapy) and the existence of an unexplained blood inflammatory response at the time of HES onset, we postulate that an unbalanced T helper response led in this exceptional case to both diseases.

Aged↗
PubMed1999

[Ocular complications of Horton's disease].

Ocular manifestations of giant cell arteritis, of which permanent amaurosis overshadows all others, occur still frequently. They are usually preceeded by the classical signs of temporal arteritis, which should allow an earlier diagnosis and prompt corticosteroid treatment. Indeed, corticosteroids have proved very effective in preventing ocular complications. Once permanent amauposis is present, the treatment is still urgent, in order to avoid the involvement of the other eye and to restore some vision of the affected eye. However, once the eye is damaged, there is little hope that corticosteroid treatment will improve the vision, depending essentially upon which delay it has been started. These fearsome ocular complications argue for a better knowledge of the suggestive signs of temporal arteritis and highlight the urgency of corticosteroid treatment.

Adrenal Cortex Hormones↗
PubMed1999

Temporal artery biopsy: a diagnostic tool for systemic necrotizing vasculitis. French Vasculitis Study Group.

OBJECTIVE: To describe the clinical, biologic, and histologic features of temporal artery biopsy (TAB)-localized systemic necrotizing vasculitides (SNV), and to assess their frequency among elderly patients undergoing TAB for suspected giant cell (temporal) arteritis (GCA). METHODS: The frequency of a TAB localization of SNV was prospectively assessed in a multicenter study of elderly patients undergoing TAB for suspected GCA. All patients with SNV fulfilling the American College of Rheumatology criteria for a specific vasculitic syndrome and with evidence of vasculitis on TAB were included in a retrospective, descriptive study. RESULTS: SNV was diagnosed based on the TAB in 1.4% of the patients with suspected GCA and in 4.5% of the positive (inflamed) TAB specimens. We retrospectively selected 27 patients (18 female, 9 male; mean +/- SD age 62+/-15 years, range 22-79 years) with SNV and TAB-localized vasculitis. Only 2 of these patients were known to have SNV before TAB localization. Twenty-two patients (81%) had cephalic symptoms, including jaw claudication in 33%, clinically abnormal temporal arteries in 33%, and neuro-ophthalmologic symptoms in 11%. All patients had systemic symptoms suggestive of SNV and histologically proven NV in the TAB specimens (70%) or elsewhere in other biopsy sites (74%). Abnormal biologic results suggestive of SNV were present in 17 patients (63%). For 4 patients, the TAB-documented involvement led to initial misdiagnoses of GCA, and systemic manifestations that developed under steroid therapy revealed the correct diagnosis. The final diagnoses of the patients were polyarteritis nodosa (PAN) (n = 11), Churg-Strauss syndrome (n = 6), micropoly-angiitis (n = 3), Wegener's granulomatosis (n = 3), hepatitis B virus-related PAN (n = 2), hepatitis C virus-related cryoglobulinemic vasculitis (n = 1), and rheumatoid vasculitis (n = 1). CONCLUSION: TAB-localized SNV presents a major diagnostic dilemma because it can mimic GCA. Careful analysis of clinical, biologic, and histologic data should lead to the correct diagnosis and help guide the clinician's choice of appropriate therapy.

Adrenal Cortex Hormones↗
PubMed1998

Thymoma-associated pancytopenia: effectiveness of cyclosporine A.

Aplastic anemia is a rare complication of thymoma and is properly documented in only few cases. Here, we report the case of a previously healthy, 65-year-old patient who was found simultaneously to have a spindle-cell thymoma and severe hypoplastic anemia with a mild infiltration of the bone marrow by CD4+ and CD8+ T lymphocytes, CD16+ natural killer cells, and a decrease in blood CD4/CD8 ratio. Cultures of marrow erythroid progenitors demonstrated serum inhibitor. While steroids, cyclophosphamide and antilymphocyte globulin failed to improve hematopoiesis, cyclosporine A (Cy-A) led to a partial, stable remission that was sustained for 4 years. Since Cy-A has been associated with good responses in three cases of thymoma-associated red cell aplasia, we recommend its use in cases of thymoma-associated cytopenias refractory to steroids and cyclophosphamide.

Adrenal Cortex Hormones↗
PubMed1998

Acquired hemophilia due to factor VIII inhibitors in 34 patients.

BACKGROUND: Acquired hemophilia is a rare disease caused by the development of auto-antibodies against factor VIII. SUBJECTS AND METHODS: We studied the characteristics and outcomes of 34 patients (19 women and 15 men) with acquired hemophilia from 1980 to 1997. RESULTS: The mean age of the patients was 61 years (range, 22-93 years). An underlying disease was observed in 18 (53%) patients: 5 patients had cancer, 4 an autoimmune disorder, 2 a dermatologic disorder, 3 asthma, 3 were postpartum, and 1 had an adverse reaction to ampicillin. Factor VIII level was <5% in 30 (90%) patients; factor VIII antibodies were elevated (>10 Bethesda units) in 23 (69%) patients. Bleeding requiring transfusions was reported in 25 (75%) patients. Human factor VIII was given to 14 patients and porcine factor VIII to 5. Six patients received prothrombin complex concentrates and one desmopressin. Several immunosuppressive treatments were used, mainly corticosteroids, cyclophosphamide, and intravenous immunoglobulin. Bleeding stopped in all but one patient within 2 weeks. Most patients achieved complete remission, although two relapses were observed subsequently. CONCLUSION: This large study helps to clarify the presentation and clinical course of acquired hemophilia. Prospective studies are needed to determine the efficacy of treatment.

Adult↗
PubMed1998

[Diabetes insipidus revealing primary malignant non-Hodgkin's lymphoma of bone].

INTRODUCTION: Diabetes insipidus is a very rare, occasionally misleading, revealing symptom of non Hodgkin's malignant lymphoma. We report a case of this uncommon association and a review of the literature is done. EXEGESIS: Soon after a transient attack of diplopia due to a 6th cranial nerve palsy, idiopathic cerebral diabetes insipidus was diagnosed in a 32-year-old immunocompetent man. As anterior hypopituitarism was absent, the patient was discharged and treated with desmopressin acetate. Six months later, recurrence of diploplia together with raised erythrocyte sedimentation rate was observed and B cell lymphoma was observed in the iliac bone and skull, with extension into the right cavernous sinus. Both tumoral lesions led to important uptake of gallium-67. After a 26-month follow-up following intensive chemotherapy including autologous peripheral stem cell transplantation and local radiotherapy, lymphoma symptoms had disappeared, but the patient's condition had only partially improved with regard to diabetes insipidus. The association of lymphoma and diabetes insipidus which has only been reported in 14 cases is discussed. CONCLUSION: Since endocrine symptoms are often preceding hematological diagnosis, malignant lymphomas should systematically be viewed as potential etiology of diabetes insipidus. Magnetic resonance imaging which is more sensitive than computerized tomography in revealing brain lymphomas is mandatory. Gallium scan imaging might help guide the diagnosis in some instances.

Abducens Nerve↗
PubMed1998

Absence of anti-beta2 glycoprotein I antibodies in giant cell arteritis: a study of 45 biopsy-proven cases.

OBJECTIVE: To search for a relationship between serum anti-beta2 glycoprotein I (anti-beta2GPI) antibodies and the occurrence of ischaemic complications in giant cell arteritis (GCA), since the latter do not correlate with anti-cardiolipin antibodies (ACL), which are frequently observed in GCA. METHODS: IgG and IgM anti-beta2GPI antibodies and ACL were measured by enzyme-linked immunosorbent assays in sera, collected before treatment, from 45 unselected patients with biopsy-proven GCA, including 15 patients with ischaemic events. RESULTS: IgG and IgM anti-beta2GPI antibodies were not detected in any of the patients, contrasting with the presence of ACL in 51%, of them, without correlation with ischaemia. CONCLUSION: Anti-beta2GPI antibodies are not detectable in GCA, contrasting with the occurrence of ACL, and ischaemic complications are apparently unrelated to the most frequent anti-phospholipid antibodies.

Aged↗
PubMed1998

[Leukemic risk in thrombocythemia].

OBJECTIVES: To define pathogenesis, incidence, clinical presentation and prognosis of leukemic transformation (LT) in patients with essential thrombocytemia (ET); to compare the incidence of LT in previously treated versus untreated patients; to search for risk factors of LT, with special reference to the iatrogenic risk of myelosuppressive drugs. DATA SOURCES AND EXTRACTION: Most significant French and English-language papers published between 1981 and 1997 dealing with either ET, LT, clonality, cytogenetics, alkylating agents or hydroxyurea were reviewed. Patient characteristics, clinical presentation, bone marrow and cytogenetic findings, and survival data were extracted from each case report and review, and possible risk factors were analyzed. DATA SYNTHESIS: Despite the existence of clonal hematopoiesis in up to 60-80% of patients with ET, suggesting an intrinsic potential for subsequent leukemia, spontaneous LT is poorly documented and the common presumption that TL occurs in excess in treated patients with ET is still questionable. The incidence of LT, probably underestimated in the past, varies from 0.7% to 5.3% in retrospective series, and from 8% to 12% in prospective studies. Recurrent cytogenetic abnormalities are significantly more frequent and complex in marrow karyotypes performed at the time of blastic crisis, as compared with those performed at diagnosis. A review of 62 reports of LT highlights some important characteristics: a) LT can occur anytime during the chronic phase of ET, especially within the first eight years, b) all subtypes of leukemia are described, notably unclassifiable cases and megakary oblastic acute leukemias, c) bone marrow fibrosis is frequent, as is a progressive onset of a myelodysplastic syndrome preceeding the blastic crisis, d) the prognosis of LT is very poor, two-third of all patients die within 6 months. Apart from the onset of a myelodysplastic syndrome, which carries a poor outcome, there is no well-established risk factor of LT. The leukemogenic risk of myelosuppressive therapies, clearly demonstrated for years in polycythemia vera, cannot be directly ascertained in patients with ET, but is suggested by various indirect observations. This putative risk of inducing secondary leukemia affects not only alkylating agents but also hydroxyurea. CONCLUSION: LT is a highly serious and largely unpredictable complication of ET. The current debate on the specific risk of myelosuppressive treatments deserves large-scale prospective randomized studies. Until this crucial point is elucidated, any cytotoxic therapy should be reserved to elderly and/or symptomatic patients and to those having other vascular risk factors.

Antineoplastic Agents↗
PubMed1997

Is treatment with hydroxyurea leukemogenic in patients with essential thrombocythemia? An analysis of three new cases of leukaemic transformation and review of the literature.

From 1981 to 1995, we diagnosed, followed and treated at our institution fifty-eight cases of essential thrombocythemia (ET), using hydroxyurea (HU) as first-line therapy in these patients. Three patients who were continuously receiving HU had a leukemic transformation after a chronic phase of respectively 47, 81 and 90 months. One patient developed an acute leukemia with minimal myeloid differentiation (AML MO) and soon died of refractory disease; the second developed a refractory anemia with excess blasts in transformation (t-RAEB) and survived one year; the third patient developed a chronic myelomonocytic leukemia (CMML) and is alive at 21 months. The two former patients had complex nonrandom bone marrow karyotypic abnormalities, suggestive of therapy-related leukemia, whereas the latter one had a normal karyotype throughout the chronic and leukemic phase. These findings, together with recently published results on myeloproliferative disorders (MPD) treated with HU, suggest that this drug might be as leukemogenic as other myelosuppressive therapies in patients with ET. Longterm HU therapy should be reserved for patients in whom the treatment benefits obviously outweigh the risk of inducing leukemia.

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PubMed1997

[Raynaud syndrome complicated by digital gangrene during treatment with interferon-alpha].

In a 43-year old male suffering from idiopathic hypereosinophilic syndrome (HES) since 1984, successfully treated with alpha interferon (alpha IFN) for 32 months, a severe Raynaud's phenomenon of the four extremities occurred and eventually evolved into digital necrosis within a few weeks. The arterial echography/doppler and plethysmography patterns were suggestive of isolated small-to medium-size digital artery occlusions. An extensive search for an aetiology of digital necrosis, including complete tests of autoimmunity, remained negative. Two months later, despite alpha IFN withdrawal and intravenous infusions of ilomedin, the digital ischemia evolved to extensive necrosis that necessitated several amputations and a definitive spinal chord stimulation. Pathologic examination of arteries showed no vasculitis but diffuse arterial occlusions by thrombi.

Adult↗