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

[Frequency of autoimmune diseases in 218 patients with autoimmune thyroid pathologies].

PURPOSE: The aim of our study was to investigate the frequency of auto-immune diseases in patients suffering from autoimmune thyroid diseases. METHOD: We realised a retrospective study from 1981 to 1993 including 218 patients suffering from thyroiditis who were followed in the same hospital service. There were 202 women and 16 men with a mean age 49 at the moment their thyroid pathology was discovered. RESULTS: Thirty patients had one or more autoimmune disease associated to their thyroid disorders, representing 13.7% of total patients. The two most frequent autoimmune diseases were lupus and Sjögren's syndrome. In 17 cases the diagnosis of the associated autoimmune disease was made simultaneously. The systemic disease preceded with an 8-year delay the thyroid disease in five cases, and the thyroid disease was annunciatory in eight cases with a delay of 5 years. The frequency of autoimmune diseases seems to be higher in patients suffering from thyroid disorders than in the general population. They are probably common physiopathological mechanisms. CONCLUSION: The frequency of these associations suggests the need for a long-lasting survey of those patients having thyroid disorders. Initial evaluation and a regular checking in patients suffering from an autoimmune disease is recommenced.

Comorbidity↗

PubMed1998

[Vitiligo in multiple autoimmune syndrome: a retrospective study of 11 cases and a review of the literature].

PURPOSE: The occurrence in the same patient of three or more autoimmune diseases defines multiple autoimmune disease. Multiple autoimmune disease is an unusual condition in which dermatological autoimmune diseases and especially vitiligo have an important place. METHODS: We examined retrospectively 11 cases of multiple autoimmune diseases associating vitiligo. We studied the clinical characteristics of vitiligo and those of the associated autoimmune disorders. RESULTS: Type III multiple autoimmune disease was diagnosed in all the 11 cases observed. Autoimmune vitiligo was the first autoimmune disorder observed in seven cases and was bilateral, symmetrical and acrofacial in eight cases. Autoimmune thyroid disorder was associated in ten cases. Our data confirms the important association between vitiligo and thyroid autoimmune disorders. CONCLUSION: The predominant female ratio and the acrofacial topography of skin lesions could predict association with others autoimmune disorders in patients with vitiligo.

Adolescent↗

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

[A feminist poem: searching for the author].

Explore the source record for details and available documents.

Acquired Hyperostosis Syndrome↗

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↗

PubMed1997

[Are the anatomopathological classifications of primary Gougerot-Sjögren syndrome correlated with each-other and with clinical symptoms?].

Seventy-two cases of primary Sjögren's syndrome, according to the European classification criteria, were studied looking for a correlation between anatomic criteria and clinico-biological signs in this disease. Labial salivary gland biopsy was performed in all patients and anatomic criteria were evaluated according to both Chisholm and Chomette scales. Work-up included recording of functional and clinical signs. Our study shows no clinico-histological correlation in Sjögren's syndrome. Moreover, we did not find any correlation between the two histological scales. We think, therefore, that labial salivary gland biopsy appears to be an important step in Sjögren's syndrome diagnosis according to European criteria. However, it does not present any benefit in patient treatment.

Adult↗

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↗

PubMed1997

Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides.

Twenty-five patients with good-prognosis polyarteritis nodosa or Churg-Strauss syndrome entered a prospective, randomized, multicentre study comparing two treatments: either oral corticosteroids and oral cyclophosphamide (CY; 2 mg/kg/day) for 1 yr (group A), or oral corticosteroids and monthly i.v. CY pulses (0.6 g/m2) (group B) for 1 yr. The objective was to determine the optimal CY regimen. Judgement criteria were the efficacy of the treatment in controlling the disease and the development of side-effects. Among the 25 patients who could be analysed, complete recovery was achieved with the experimental treatment in 9/12 patients in group A and 10/13 patients in group B. Two patients in each group relapsed after the end of therapy and were well controlled by corticosteroids or other drugs. One failure occurred in each group. The mean follow-up was 60.8 +/- 14.5 months after the beginning of the treatment. Side-effects associated with the administration of CY and steroids were noted 27 times in group A vs 14 times in group B (not significant). The oldest patient in these series (group B) died of pneumonia. No superiority in terms of efficacy could be established between the two regimens; however, the number of patients included was too small to conclude definitively. Toxic side-effects were significantly more frequent in women (P < 0.02). The high number of adverse effects leads us to recommend pulse over oral CY and an overall lowering of the doses of immunosuppression.

Adult↗

PubMed1997

[Acquired hemophilia caused by autoantibodies against factor VIII coagulation activity. Clinical, biological study and therapeutic indications. Experience based on a study of 9 cases].

STUDY DESIGNS: To describe retrospectively the experience of the Internal Medicine and Clinical Hematology Departments of a University Hospital on adult acquired hemophilia (AH) caused by autoantibody against factor VIII coagulant (f.VIII:C) activity. Diagnosis, clinical datas, associated diseases, treatment and final outcome are described and compared to the published literature. MATERIAL AND METHODS: All cases admitted in both departments since 1989 were enrolled in the study. Clotting analyses comprised clotting times (activated partial thromboplastin time, prothrombin and thrombine times), measurements of f.VIII:C level, antifactor VIII detection and measurement by the Bethesda method assay, Search for an etiologic factor could not be standardized. All patients were followed until cure, sustained improvement, or death. RESULTS: From 1989 to 1996, AH was diagnosed in nine adult patients. Mean age was 76 +/- 24.6 years (range : 65-89) and sex ratio male to female was 2. Eight bleeding episodes occurred in seven patients, resulting consistently in severe hemorrhagic anemia and leading to hemodynamic failure in two, while two others remained asymptomatic for prolonged periods. The initial levels of f.VIII:C ranged from less than 1% to 20%, and the titers of inhibitors ranged from 0.5 to 100 Bethesda units. An underlying disease, to which the appearance of their inhibitor could be related, either concomitantly or up to 1 year later, was found in four cases including (one case each): rheumatoid arthritis, lupus erythematosus with antiphospholipid syndrome, followed by non-Hodgkin malignant lymphoma, relapsing carcinoma and, biliary tract surgery. Six acute bleeding episodes necessitated symptomatic measures, based on activated prothrombin complex concentrates in four instances, with a good response in all cases. Preparation to minor surgical operations was achieved in two asymptomatic subjects by either highly purified factor VIII concentrations infusion or intravenous 1-desamino-8-D-arginine vasopressin, with a good control of local hemostasis in each case. Three received intravenous immunoglobulins, which resulted in success in one, failure in one and, questionable response in the latter. Immunosuppression, mainly with corticosteroids, cyclophosphamid, or both, was given to seven, resulting in disappearance of inhibitor in five (delay to cure ranged from 2 weeks to 10 months), improvement in one, and failure in one (in this latter case, cure was eventually achieved with the anti-Hodgkin disease MOPP chemotherapy). After a 27-month mean follow-up, six patients experienced a sustained complete response and one a sustained partial response to immuno-suppression, two untreated patients remained asymptomatic, two died later from malignancy (carcinoma and myelodysplastic syndrome). CONCLUSION: AH usually presents as a severe or even a life-threatening disease, necessitating prompt and thorough symptomatic measures directed at the cessation of bleedings and prevention of their relapse. In our experience, no death was attributable to AH or its treatment. Immunosuppression is useful in selected cases, but must be carefully discussed, since it can be highly toxic, especially in the elderly. Given the possibility of a delayed onset of some etiologic factors, a prolonged surveillance of each case of idiopathic AH is mandatory.

Aged↗

PubMed1996

[Immunohistochemical study of lesions in Horton's temporal arteritis before and during corticotherapy].

The immunohistochemical study was performed on temporal artery biopsies from eight patients with giant cell (temporal) arteritis: three before treatment, four after a short period of corticosteroid therapy (from 1 day to 7 days) and one during relapse occurring after a treatment of 9 years; from four subjects with clinical symptoms but without histological features of giant cell arteritis and from five negative controls. Before treatment, biopsies of patients with temporal arteritis showed an inflammatory infiltrate with macrophages and T cells, essentially CD4+ and memory T cells (CD45 RO+), expressing the markers of activation IL2 receptor and HLA DR. Few B and NK cells were also detected. Adhesion molecules, LFA1 and I-CAM1, were strongly expressed by T cells and macrophages. In contrast, the ligand to the CD2, the CD58 marker, was rarely detected. These immunohistochemical features were also observed after a short corticosteroid treatment (by intravenous methylprednisolone or oral prednisone), with presence of activated T cells, memory T cells, macrophages and I-CAM1 and LFA1 expressing cells in the infiltrate. A temporal biopsy, performed after a long time of corticosteroid therapy, showed activated T cells, macrophages and memory cells in o,ne arteriole. In controls, this study showed some mononuclear cells dispersed in intima and adventia, but without activated or memory T cells. Our results support the presence of immune local response in temporal arteritis, incompletely improved by a short corticosteroid treatment.

Adrenal Cortex Hormones↗

PubMed1995

Large-scale study suggests no direct link between human herpesvirus-6 and primary Sjögren's syndrome.

The aim of this study was to evaluate the role of human herpesvirus-6 (HHV-6) in the development of primary Sjögren's syndrome (PSS). Serum HHV-6 antibody levels, as measured by immunofluorescence assay (IFA) and the prevalence of HHV-6 DNA in peripheral blood mononuclear cells (PBMCs) determined by polymerase chain reaction (PCR), were studied in 49 PSS patients and 50 control subjects, all in-patients in the University Hospital Internal Medicine ward, Limoges, France. In addition, portions of labial salivary gland were obtained from 34 patients and 15 controls, the presence of viral DNA being detected by the same PCR technique. The results were then compared with clinical observations of systemic disease manifestations in patients and a histological study of salivary gland involvement. No significant difference in HHV-6 seroprevalence was found between control subjects (50.0%) and patients (63.3%) nor was there any statistically significant difference between patient and control groups for total viral DNA in PBMCs (22.4%, 12.0%) and salivary glands (8.8%, 6.6%). Analysis of clinical and histological data revealed no detectable correlation between disease severity and viral involvement. Tests for HHV-6A and HHV-6B proved positive in patient and control groups, HHV-6B being the most frequently encountered type in both groups. In conclusion, the results of this large-scale trial does not confirm the suspected direct role of HHV-6 in the etiology of PSS.

Antibodies, Viral↗

PubMed1995

No direct relationship between HIV1/2 infection and HHV-6 antibody response in a large-scale European and African trial.

BACKGROUND: Tropism of both human herpesvirus-6 (HHV-6) and human immunodeficiency virus (HIV) for CD4+ lymphocytes, and several studies on HIV enhancer transactivation by HHV-6 have suggested that HHV-6 could be implicated as a cofactor in the progression of HIV infection to AIDS. OBJECTIVES: To determine if there is any relationship between HHV-6 infection and the course of HIV infection. STUDY DESIGN: 527 French and 558 African sera (210 from Senegal, 348 from Ivory Coast) were tested for HHV-6 titers by immunofluorescence assay (IFA). For each country, patients studied were divided into several groups; those with AIDS, those who were HIV-seropositive but symptomless, and HIV-seronegative patients. RESULTS: No statistical difference could be found between HHV-6 prevalences or HHV-6 geometric mean titers obtained for each geographical area in the different HIV-1- and/or HIV-2-positive and HIV-negative groups. HIV patients with clinical manifestations of AIDS did not differ in percent seropositivity or distribution of titers from the HIV-asymptomatic patients or HIV-seronegative patients. CONCLUSION: These findings suggest that there is no correlation between HHV-6 antibodies and HIV seropositivity or with the stage of HIV infection.

Journal Article↗