E. Vidal

Thymoma-associated pancytopenia: effectiveness of cyclosporine A

Abstract 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.

Lymphomes malins et syndrome des antiphospholipides non simultanés : quatre observations

Objective. – We report four cases of non-synchronous antiphospholipid syndrome (APS) and malignant lymphoma, which highlight the complex relationship that seems to exist between these illnesses. Methods. – In a retrospective study conducted in two departments (internal medicine and clinical hematology) of a university hospital, we collected all observations of patients with both APS and malignant lymphoma diagnosed throughout the past decade. Results. – An association of APS with malignant lymphoma was recorded in three female and one male patient, median age 42.5 years at the time of diagnosis of the first disease. In each case, the primary APS was diagnosed, with arterial thrombotic events in three cases and venous thrombotic events in one case. One patient had isolated IgG anticardiolipin antibody, whereas the others had a combination of IgG anticardiolipin antibody and lupus anticoagulant with or without IgG anti-β2 glycoprotein I antibody. One patient also had an acquired inhibitor to factor VIII:C and a chronic C virus hepatitis. The mean time apparently separating the two illnesses ranged from 18 months to 9 years, but in two cases the diagnosis of APS was delayed due to a progressive, atypical, neurological onset. In two instances, the APS took place at a distance from a cured malignant lymphoma (Hodgkin’s disease and nodal large cell B-cell lymphoma), whilst in the others it preceded a B-cell lymphoma (nodal and cutaneous, small cells and primary hepatic, large cells). Treatment resulted in complete haematological response in both cases, with disappearance of anticardiolipin antibody and lupus anticoagulant in the latter following a double autologous peripheral blood stem cell transplantation. In addition, late carcinomas (breast, kidney, thyroid) were seen in two patients. Conclusions. – Our data indicate that the diagnosis of a malignant lymphoma should be considered in patients with a primary APS and peripheral lymph node enlargement or unexplained constitutional symptoms. Conversely, a late onset of arterial or venous thrombotic diathesis after a malignant lymphoma may indicate not only late relapse of malignant lymphoma but also a subsequent APS.

Gougerot-Sjögren, syndrome sec et hépatite C

The authors report their own experience about the possible role of hepatitis C virus (HCV) in Sjogrens syndrome, studying two population: 15 HCV chronic liver diseases and 26 primary Sjogrens syndrome.

Physiopathologie de l’artérite à cellules géantes

Giant cell arteritis is a large-vessel vasculitis affecting all three layers of the arterial wall. Histopathology of this vasculitis consists of an inflammatory infiltrate with CD4(+) T cells, macrophages, multinucleated giant cells, forming granulomas in the media. This infiltrate is associated with a destruction of the arterial wall, a fragmentation of the internal elastic lamina and a vascular remodeling leading to intimal hyperplasia. Recent studies have clarified the role of Th17 cells in the initial phase of the disease, pro-inflammatory cytokines and vascular smooth muscle cells in vascular remodeling. This review aims to update data on giant cell arteritis pathogenesis and to propose clues of investigation for a better understanding of this condition.

Mise au pointL’ascite non liée à la cirrhose : physiopathologie, diagnostic et étiologiesNon-cirrhotic ascites: Pathophysiology, diagnosis and etiology

Ascites, in 20% of cases, is not linked to liver cirrhosis. The pathophysiology is most often different. The understanding of these pathophysiological mechanisms can lead to etiologic diagnosis. The diagnostic approach is mainly based on the biological study of ascites, especially protein concentration and albumin gradient between serum and ascites. In Western countries, tumors and heart diseases are the predominant causes, while developing countries are mainly concerned by infectious diseases, among which tuberculosis is the leading cause. Other uncommon causes must be recognized, as ascites may be the presenting feature of the disease. Their knowledge will facilitate the therapeutic approach.

L’ascite non liée à la cirrhose : physiopathologie, diagnostic et étiologies

Ascites, in 20% of cases, is not linked to liver cirrhosis. The pathophysiology is most often different. The understanding of these pathophysiological mechanisms can lead to etiologic diagnosis. The diagnostic approach is mainly based on the biological study of ascites, especially protein concentration and albumin gradient between serum and ascites. In Western countries, tumors and heart diseases are the predominant causes, while developing countries are mainly concerned by infectious diseases, among which tuberculosis is the leading cause. Other uncommon causes must be recognized, as ascites may be the presenting feature of the disease. Their knowledge will facilitate the therapeutic approach.

Vascularite après vaccin antigrippal

A 64 year-old man was found to have a vascular purpura with histologic features of cutaneous necrotizing vasculitis, fifteen days after influenza vaccination. No other cause (neoplasic, autoimmune, infectious or drug-induced) was apparent, and the reponsability of vaccination is retained while it is a very rare complication.

Goitre multinodulaire et artérite à cellules géantes intrathyroïdienne diffuse révélatrice d'une maladie de Horton

We describe a 62-year-old woman with slowly growing usual nodular goitre in whom diffuse giant cell arteritis (GCA) of the thyroid arteries was found upon thyroidectomy, revealing otherwise unsuspected biopsy-proven temporal arteritis. To our knowledge, this association had been previously reported in only three instances. In each case, GCA of the thyroid arteries appeared clinically silent, did not produce significant glandular dysfunction, and was uncovered thanks to a planned thyroidectomy for nodular goitre. These observations highlight that thyroid artery involvement by GCA, even widespread, as in our patient, may be overlooked clinically and may produce little or no thyroid dysfunction.

Association anticorps anticardiolipine et néoplasies: à propos de six observations

Neoplastic diseases are a rare etiology of anticardiolipin Antibodies (ACL); they are found in 3 % of cases. The authors report 6 malignant pathologies associated with ACL with 2 thrombotic complications. Then, prospective clinical studies are needed to determine the prognostic value of ACL for thrombosis in neoplastic diseases.

Maladies thyroïdiennes auto-immunes et artérite temporale de Horton

Abstract Among 115 patients with Giant Cell Arteritis (GCA), incidence of thyroid disease was evaluated. Eleven cases were encountered. These included hyperthyroidism in 5 cases, hypothyroidism in 4 cases (2 of them with serum thyroid antibodies) and autoimmune thyroid disease in 2 without hypothyroidism. It is concluded that there is not significant association between GCA and autoimmune thyroid disease.