[Efficiency of imatinib in polyserositis revealing a FIP1L1-PDGFRA-negative hypereosinophilic syndrome]. / Efficacité de l'imatinib au cours d'un syndrome hyperéosinophilique FIP1L1-PDGFRA négatif révélé par une polysérite.

INTRODUCTION: Hypereosinophilic syndromes rarely manifest as polyserositis. Imatinib mesylate is the reference treatment for myeloid variants of FIP1L1-PDGFRA-positive hypereosinophilic syndromes. A response to imatinib has also been reported in FIP1L1-PDGFRA-negative hypereosinophilic syndromes. CASE REPORT: We report a 25-year-old man who presented with a FIP1L1-PDGFRA-negative hypereosinophilic syndrome, with severe pericardial effusion and bilateral pleuritis. Imatinib mesylate at the dose of 100mg daily was started because of high-dose corticosteroids dependence. A response was noted after 15 days of treatment. Corticosteroids were discontinued after 7 months and the patient remained asymptomatic after 23 months of treatment. CONCLUSION: This report evidences the association of polyserositis with hypereosinophilic syndromes and the potential efficacy of imatinib mesylate even in FIP1L1-PDGFRA-negative patients.

[Carcinoma of unknown primary site]. / Les carcinomes de primitif inconnu.

PURPOSE: Carcinoma of unknown primary site is a common clinical syndrome, accounting for 2% of cancer patients. Diagnosis is a recurrent challenge for internists. Treatment is difficult and prognosis is still poor. This review presents one synthesis of diagnosis strategies and therapeutic trials. It envisages the interest of new molecular biology methods as well as therapeutic perspectives. CURRENT KNOWLEDGE AND KEY POINTS: Pathologic examination completed with immunohistochemical tests, and, depending on cases, with electron microscopy, cytogenetics, and molecular biology is a key-point for diagnosis. Diagnosis work-up, based on histological type and on individualization of some clinical presentation, proceeds in three steps. Positron emission tomography is recommended when a curative treatment is planed, particularly in cases of isolated metastasis. Functional status analysed using the performance status and simple biologic parameters (serum lactate dehydrogenase, serum alkaline phosphatase) permit us to assess prognosis. Chemotherapy is offered for patients with a good general health status. FUTURE PROSPECTS AND PROJECTS: Further evaluation of positron emission tomography, as well as cost-benefit analyses, is warranted. Further randomised trials are necessary to determine the optimal chemotherapy regimen in good-risk patients and the interest of chemotherapy in patients with poor-risk disease. Gene expression profiling and proteomic evaluation, as well as pharmacogenomic offer new investigation fields.

[Systemic manifestations of Parvovirus B19 infections]. / Manifestations systémiques des infections à Parvovirus B19.

PURPOSE: Parvovirus B19 (B19) causes many clinical disorders, of which the most common are erythema infectiosum, aplastic crisis complicating chronic hemolytic anemia, and hydrops fetalis. In young adults, the skin eruption caused by B19 is accompanied by polyarthritis and polyarthralgia in 60% of the cases. Rheumatoid factors and other antibodies including antinuclear antibodies, anti-ADN, and antiphospholipids can be produced in the wake of B19 infection. CURRENT KNOWLEDGE AND KEY POINTS: These features may simulate systemic diseases as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) (lupus-like eruption over the cheeks, cytopenia, etc.) or vasculitis (purpura, renal involvement). In addition, there have been a few reports of SLE, vasculitis and other connective tissue diseases developing shortly after a B19 infection associated with virus clearance suggesting that B19 can act as a trigger of systemic disease. However, studies in large series indicate that in fact B19 is probably an extremely rare cause of RA, SLE or vasculitis. FUTURE PROSPECTS AND PROJECTS: In fundamental studies B19 interacts with inflammatory cells by regulation of cytokines. More recently, two studies suggest that viral infection due to B19 may affect the course of SLE, leading to specific biological subsets. These preliminary findings require confirmation to elucidate the significance of the presence of B19 in systemic disease.

[Adult Kawasaki disease]. / La maladie de Kawasaki de l'adulte.

PURPOSE: Review of the literature on adult Kawasaki disease. CURRENT KNOWLEDGE AND KEY POINTS: Kawasaki disease is an acute multisystemic vasculitis affecting predominantly young children. Several studies have suggested that Kawasaki disease is mediated by bacterial superantigens. The diagnosis is established on clinical criteria since no specific laboratory test yet exists for this disorder. The severity of Kawasaki disease relates to the possible occurrence of coronary aneurysms in 20% of childhood cases. Treatment with intravenous immunoglobulins before day 10 is recommended to prevent aneurysm formation. The occurrence of Kawasaki disease is unusual in adults and 52 cases only have been reported in adult patients. Seventy-one per cent of cases occur between 18 and 30 years. The incidence of specific clinical features is quite similar between adults and children. However meningitis and thrombocytosis are more common in children than in adults, while conversely both arthralgias and liver function abnormalities are more common among adults. Coronary aneurysms are less common in the adults with Kawasaki disease. Other diseases with similar clinical presentation such as drug hypersensitivity reaction and the toxic shock syndrome must be ruled out. Kawasaki disease is often diagnosed after the acute phase at the step of desquamation, when it is too late to expect any beneficial effect from immunoglobulins. FUTURE PROSPECTS AND PROJECTS: Diagnostic criteria of Kawasaki disease have not been validated in an adult population. Criteria of exclusion are necessary to eliminate toxic shock syndrome and drug hypersensitivity syndrome. An international retrospective study to collect data on epidemiologic, clinical, laboratory, and cardiovascular features of adult Kawasaki disease is necessary to validate specific diagnostic criteria and to improve the knowledge on this disease.

[Persistent erythema multiforme associated with chronic hepatitis C virus infection. Efficacy of interferon alpha]. / Erythème polymorphe persistant associé à une infection chronique par le virus de l'hépatite C. Efficacité de l'interféron alpha.

INTRODUCTION: Persistant erythema multiforme is a rare form of erythema multiforme with subacute typical and atypical lesions that occur during several months. Some cases are associated with chronic viral infection. CASE REPORT: A 23 year-old man, with a past history of intravenous drug addiction and chronic hepatitis C virus infection, presented persistant erythema multiforme for 18 months. The histopathological picture was those of infectious erythema multiforme and the seric total complement level was low. Two courses of alpha-interferon treatment were quickly efficient on cutaneous lesions, and relapse occurred after discontinuation. DISCUSSION: In previously reported cases of persistant erythema multiforme, etiologic complementary investigations are not always specified. However, viral infections should be considered. In cases of chronic infection, hepatitis C may induce immune disorders through persistent antigenic stimulation.