[Idiosyncratic drug-induced thrombocytopenia]. / Les thrombopénies médicamenteuses idiosyncrasiques.

Idiosyncratic drug-induced thrombocytopenia is a relatively rare and potentially serious side-effect in its moderate and profound forms (platelet count less than 100 x 10(9)L). It may be of central or peripheral mechanism, with consumption or immunological destruction. The main incriminated molecules are heparins, quinidine, sulfonamides and gold salts. Hemorrhagic manifestations are the rule and their severity is related to the level of thrombocytopenia and the involved molecule. Heparin-induced thrombocytopenia is more often associated with thrombotic events. Diagnosis relies on medical history and clinical criteria, which also establish the level of imputability. The role of serological tests is not yet clear but seems to be particularly interesting in difficult diagnostic situations and in heparin-induced thrombocytopenia. The treatment is based on the discontinuation of the incriminated drug and on supportive measures that depend on the severity of the clinical features.

Systemic lupus erythematosus and multiple myeloma: an uncommon association. Two cases and literature review.

The association of systemic lupus erythematosus and multiple myeloma is uncommon. We report two cases of systemic lupus erythematosus associated to multiple myeloma. The cases are discussed in the light of a review of the literature. The clinical, laboratory and radiographic findings of the patients, as well as the subsequent therapeutic approach are discussed. A systematic review of all the other cases of this association is performed. We report two female patients of 50 and 35 years old who developed a multiple myeloma seven and three years respectively after the diagnosis of systemic lupus erythematosus. In the second case, systemic lupus erythematosus was associated to monoclonal gammopathy. One patient died after three months and one patient is still in remission after three years of the diagnosis of multiple myeloma. The coexistence of systemic lupus erythematosus and multiple myeloma is very rare and the possible pathogenetic mechanisms underlying this association remain unclear.

[Prolonged fever of unknown origin in Behçet's disease. Pay attention to vessels!]. / Fièvre prolongée inexpliquée au cours de la maladie de Behçet. Attention aux vaisseaux!

Behçet's disease is a systemic vasculitis characterized by the association of recurrent oral and genital ulcers to systemic involvements, particularly ocular, nervous and vascular manifestations. Contrary to other vasculitis, prolonged fever of unknown origin is rare in Behçet's disease. We report a case of a 26-year-old man presenting prolonged fever for two months. Physical examination showed oral, genital ulcers and pseudofolliculitis. The sedimentation rate was increased. Chest and abdominal computed tomography revealed thrombus in the inferior vena cava and portal vena. Outcome was favorable with glucocorticoid and anticoagulant therapy. Prolonged fever occurring during Behçet's disease should prompt a search for a vascular injury.

Kaposi's sarcoma in association with Behcet's disease: case report and literature review.

OBJECTIVES: To describe a case of Behcet's uveitis associated with Kaposi's sarcoma occurring simultaneously in a patient and to review the literature on iatrogenic Kaposi's sarcoma. METHODS: We describe the case of a 44-year-old Moroccan man, who developed a Kaposi's sarcoma 8 months after immunosuppressive therapy for ocular Behçet's disease. He was treated with corticosteroids and cyclophosphamide (Exdoxan, Baxter) pulse for 6 months followed by oral azathioprine (Imurel, Glaxo Smith Kline). Literature searches were performed on iatrogenic Kaposi's sarcoma and other cases of such association and the potential pathogenic mechanisms involved. RESULTS: Iatrogenic Kaposi's sarcoma is widely reported to develop after renal transplantation during immunosuppressive therapy. Less commonly, Kaposi's sarcoma occurs in patients receiving long-term corticosteroids or immunosuppressive therapy for rheumatic diseases. It is considered to be induced by activation of latent human herpes virus 8. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with ocular Behçet's disease. Interferon-alpha is of value for patients with both conditions. CONCLUSION: This case report underscores the relationship between environmental and infectious factors, drug-induced immunosuppression, and the development of Kaposi's sarcoma.

Fever revealing Behçet's disease: Two new cases.

Behçet's disease (BD) is an uncommon cause of fever of unknown origin. We report two cases, both involving 42-year-old males, who initially presented with prolonged fever and who were ultimately diagnosed as having BD after a delay of 12 and 21 months, respectively. Both patients developed pulmonary aneurysms. Although fevers resolved after therapy, both patients died within the first year after diagnosis. Clinicians should be aware that long-term fever may be an inaugural sign of BD, especially in individuals living in countries along the ancient Silk Road or Mediterranean basin.

[Primary Gougerot-Sjogren's syndrome presenting as a cerebellar syndrome]. / Syndrome cérébelleux isolé révélant un syndrome de Gougerot-Sjögren primaire.

Primary Sjogren's syndrome is an autoimmune exocrinopathy with various extraglandular complications. Central neurological manifestations were described in 20 percent of cases. Cerebellar syndrome is uncommon in Sjogren's syndrome, but can occur after diagnosis, or rarely as an inaugural sign of the disease. We report the case of a 54-year-old man who presented a cerebellar syndrome with xerostomy and xerophthalmos. The diagnosis of primary Sjorgren's syndrome was confirmed on the accessory salivary gland biopsy. The patient received oral and intravenous corticosteroid therapy associated with cyclophosphamide monthly. Outcome was good.

[Neurological manifestations of vitamin B12 deficiency: a retrospective study of 26 cases]. / Les troubles neurologiques par carence en vitamine B12: étude rétrospective de 26 cas.

PURPOSE: Neurological symptoms of B12 vitamin deficiency are polymorph. Causes are dominated by Biermer's disease and B12 vitamin non dissociation that is frequent in the elderly. METHODS: We realized a retrospective study during 11 years. Patients with neurological symptoms associated to megaloblastosis were included. Treatment with B12 vitamin lead to haematological manifestation regression. RESULTS: 26 cases were analyzed. Mean age was 50+/-14.5 years and there were 11 women and 15 men. Neurological signs included combined medullar sclerosis (N = 10), peripheral neuropathy (N = 10), isolated paresthesia (N = 5) and inferior limb pyramidal syndrome (N = 1). Neurological signs revealed vitamin B12 deficiency in 4 cases. Mean haemoglobin rate was 6.2+/-2.6 g/dl, mean MCV was 109+/-56 fl. Eight patients had macrocytic anaemia, nine bi-cytopenia and eight deep pancytopenia. Electromyography (N = 8) confirmed neuropathy and medullar MRI (N = 2) showed antero-posterior cordonal demyelinisation of cervico-dorsal medulla. Causes of B12 vitamin deficiency were Biermer's disease (N = 11), non dissociation of B12 vitamin's syndrome (N = 8) and partial gastrectomy (N = 1). In 6 patients, no aetiology was found. Treatment with parenteral vitamin B12 induced neurological symptoms regression in 14 cases. Patient with neurological disorder had significantly higher platelet count and haemoglobin level as compared with patients without neurological disorder. Reticulocyte crisis was more precocious in patients with neurological disorders. CONCLUSION: Neurological symptoms in vitamin B12 deficiency are frequent. We insist on isolated forms, inaugural forms and on the interest of medullar MRI for early diagnosis.

[Systemic capillary leak syndrome: two case reports]. / Le syndrome d'hyperperméabilité capillaire idiopathique : deux nouvelles observations.

INTRODUCTION: Systemic capillary leak syndrome (SHCI) is a rare disease with poor prognosis, characterized by recurrent episodes of generalized edema and hypotension. EXEGESIS: Two patients a 41 and 34-year-old woman were admitted with a generalized edema and several episode of shock. One patient had muscular edema with compartment syndrome imposing the aponevrotomia of discharge in emergency. The association of elevated hematocrit, decreased albumin serum concentration confirmed the diagnosis of SHCI. Administration of intravenous fluids improved the clinical and biological symptomatology. CONCLUSION: These two new observations are comparable to the published cases. We didn't find dysglobulinemia whose existence is frequented. The treatment of shock requires macromolecules solutions. The preventive treatment remains empiric.

[Mixed cryoglobulinemia with monoclonal IgA and biclonal IgM during hepatitis C virus infection]. / Cryoglobuline mixte à IgA monoclonale et IgM biclonale au cours d'une hépatite C.

Cryoglobulins are immunoglobulins that precipitate at low temperature and dissolve when warmed. According to Brouet, their classification relies upon the immunochemical study: type I comprises monoclonal immunoglobulins (IGG), when types II and III include both monoclonal and polyclonal components. During C hepatitis, the presence of a cryoglobulin, essentially made of mixed G-IGG and M-IGG, is a common feature with a prevalence of 40 to 80%. The authors report a case of a 63-year patient who presented with a vascular purpura and a peripheral polyneuropathy in a context of C hepatitis infection. The cryoglobulin found was composed of a monoclonal kappa A-IgG associated with a biclonal kappa and lambda M-IgG. No decrease of normal IgGs was found. This type of cryoglobulin does not belong to Brouet's classification, and argues for a new and more pertinent classification to be proposed.

[Association of pulmonary artery aneurysm, right heart thromboses and antiphospholipid antibodies in Behcet's disease]. / Association anévrysme de l'artère pulmonaire, thromboses intracardiaques et anticorps antiphospholipides au cours de la maladie de Behçet. A propos d'un cas.

INTRODUCTION: Cardiac thromboses are unusual in the course of Behçet's disease and are frequently associated with endomyocardial fibrosis of the right heart. Vascular pulmonary involvement with either pulmonary aneurysm or parenchyma alterations is also often observed. However, pathogenesis of thromboses occurring in the course of Behçet's disease is still unclear. CASE REPORT: The authors report the case of a 28-year-old man who presented with Behçet's disease accompanied by pulmonary aneurysm, multiple thrombi of the right heart and antiphospholipid antibodies. CONCLUSION: The choice of therapeutical strategies for curative treatment of this association as well as the interpretation of the role of antiphospholipid antibodies in this polyvascular disease raises questions.

[Takayasu's disease in Morocco. Report of 47 cases]. / La maladie de Takayasu au Maroc. A propos de 47 observations.

PURPOSE: Takayasu's disease is a chronic inflammatory arteritis involving large vessels in young women. We studied Moroccan patients to evaluate clinical, biological, radiological and evolution features of this disease in our country. METHODS: Forty-seven patients with Takayasu's arteritis were studied retrospectively between 1988 et 1999. RESULTS: In our series involvement of the aortic arch and its branches was more frequent than the abdominal aorta and its branches. Stenotic lesions of renal arteries were rare. Ultrasound was useful in the diagnosis and the monitoring of the disease. Treatment with glucocorticoids gave good results, with improvement in half of the patients and remission with stabilisation in 40% of cases. Tuberculosis occurred in 8.5% of patients. CONCLUSION: The use of ultrasound and computed tomography angiography is helpful for the diagnosis and monitoring of the disease progression. Glucocorticoids help to induce long remission in about 80% of treated patients. We don't find any relationship between Takayasu's arteritis and tuberculosis.

[Tuberculosis of the thyroid. Diagnosis and treatment]. / La tuberculose de la thyroïde. Diagnostic et traitement.

Thyroid tuberculosis is rare. We present the case of a 25 year-old woman with tuberculosis of the thyroid. Although seldom observed, tuberculosis should be considered in the differential diagnosis of nodular lesions of the thyroid. Diagnosis is made by histological examination and demonstration of the tubercle bacilli from biopsy or aspiration specimen. The efficacy of fine-needle aspiration cytology in diagnosis of tuberculosis of the thyroid is proved. Administration of antituberculous drugs is considered as the treatment of choice. Abscess drainage is sufficient. In rare cases surgery is necessary. The prognosis is good.

[Cholesterol crystal embolism]. / Les embolies de cristaux de cholestérol.

Cholesterol crystal emboli are a serious complication of atheroma. The incidence of this syndrome appears to be much more common in patients in their sixties with severe atheromatous disease of the aorta (20 to 30% vs less than 5%). 80% of crystal embolism result from medical interventions (aortic or cardiac surgery, arterial invasive procedure of aorta, thrombolytic therapy). Embolisation of cholesterol crystals can give rise to a confusing clinical pictures, depending of the site (organ) of the embolisation. It also can simulate a systemic disease. The diagnosis must be discuss in all atheromatous patients. The prognosis is poor because of the patient's clinical context and because there is no specific treatment. The best treatment is prevention by improving the recognition of the high risk patients and avoiding in those cases the predisposing factors.

[Iterative surgery for goiter]. / Les réinterventions après thyroïdectomie pour goitre.

AIM: Identify and resolve problems related to iterative surgery for goiter. PATIENTS AND METHOD: Fifty-nine patients who underwent surgery for recurrent benign goiter between 1990 and 1999 were included in the study. RESULTS: Forty-two patients had lobectomy and isthmusectomy (71.2 and 17 patients had subtotal thyroidectomy (28.8%). The delay from initial surgery to recurrence was less than 5 years for 44% of the patients and was more than 10 years for 17.8%. There was no post-operative mortality and no recurrent laryngeal nerve injury. Early post-operative hypocalcemia occurred in 2 patients (3.3%). CONCLUSION: The goal of revision surgery for recurrent goiter is total thyroidectomy. Prevention is a rational management scheme for thyroid nodules.

[Cardiovascular involvement in Behçet's disease. (author's transl)]. / Manifestations cardio-vasculaires de la maladie de Behçet.

35 cases of Behçet disease are presented including 16 vascular manifestations: 14 veinous thrombosis with 9 vena cava obstruction; 1 femoral aneurysm; 2 pulmonar arteries thrombosis; 3 pericarditis; 2 myocardial infarction. In all these cases corticosteroids and anti-coagulation therapy were instituted. With a follow-up from 5 to 36 months, only 5 relapsed in the five first months of therapy. The authors suggest systematic anticoagulation or anti-agregant therapy in Behçet disease.