Hemophagocytic syndromes are the clinicobiological translation of an unconnected macrophagic activity with hemophagocytosis. Their physiopathology is related with a deregulation of the T lymphocytes and an excessive production of cytokines. Acquired hemophagocytic syndromes are mostly associated with underlying pathology which they can reveal: immunodeficiency, infections (mostly of viral origin), hemopathies and cancers, auto-immune diseases. The main clincobiological features are fever, hepatosplenomegaly and peripheric bicytopeny. In the majority of cases, the diagnosis is confirmed by a myelogram which shows the presence of benign histiocytes, actively phagocyting the hematopoietic cells. The pejorative prognosis of hemophagocytic syndromes (actual mortality rate 30 to 45%) requirs an early therapy which associates etiological treatment of the underlying affection with pathogenic treatment (pulse of corticosteroids, immunoglobulins, immunosuppressors, or plasmapheresis).

Les syndromes hémophagocytaires sont la traduction clinicobiologique d'une activation macrophagique inappropriée avec hémophagocytose. Leur physiopathologie ferait intervenir une dysrégulation des lymphocytes T avec production excessive de cytokines. Les syndromes hémophagocytaires acquis sont le plus souvent associés à une pathologie sous-jacente, qu'ils peuvent révéler: immunodéficience, infections (surtout virales), hémopathies et cancers, maladies dysimmunitaires. Le tableau clinicobiologique associe une fièvre, une hépatosplénomégalie et constamment une bicytopénie périphérique. Dans la majorité des cas, le diagnostic est affirmé par le myélogramme révélant la présence d'histiocytes bénins, phagocytant activement les cellules hématopoïétiques. Le pronostic péjoratif des syndromes hémophagocytaires (mortalité propre: 30 à 45 % des cas) requiert une thérapeutique urgente associant traitement étiologique de l'affection sous-jacente et traitement à visée pathogénique (bolus de corticoïdes, immunoglobulines, immunosuppresseurs, plasmaphérèses).

INTRODUCTION:The prevalence of mucocutaneous herpetic infection is especially high in patients with the acquired immunodeficiency syndrome (AIDS). In these patients, 8 p. 100 of the chronic or recurrent herpetic lesions are due to a mutant strain.CASE REPORT:A case of very large (900 cm2) genital chronic herpetic infection is reported in a patient with AIDS (stade C3-CDC/WHO 1993). It was characterised by an acyclovir resistant strain which emerged after several anterior treatment with acyclovir. A treatment with foscarnet was administered and clinical improvement was observed as early as the fourth day, with complete reepithelialization 50 days later.DISCUSSION:We discussed essentially the pathogenicity of the herpetic infections due to mutant strains in immunocompromised patients and on the therapeutic modalities. At the present time, foscarnet is the treatment of choice for acyclovir-resistant mucocutaneous herpes simplex.

Clinical trials for cognitive disorders in the elderly require specific methodological guidelines. They must take into account the psychosocial dimension of the patient and his family and must be based on serious neurobiologic knowledge. In degenerative dementias the progress of research concern genetics, molecular intercellular recognition and astrocytic cells. Biology of cognition like hippocampal long term potentiation provides good pharmacologic basis for trials. In normal brain aging several ways must be developed: aminergic systems, free radicals, excitotoxic amino-acid, nerves growth factors. Clinical trials bring informations for pharmacology and epidemiology. Cholinergic neurons are the main pharmacologic target but there are many other ones: GABA-ergic system, Tau protein, amyloid. A rigourous selection of patients allows to precise the nosology of illness responsible of cognitive disorders and to point-out early clinical signs that represent a more sensitive target. Diagnostic criteria are useful in Alzheimer's disease, memory impairment, vascular dementias and other dementias. Evaluation of stage and evolution of dementia, comorbidity, limits of age and caregiver are practical problems. The effects of drugs used to treat cognitive functions are subtle so it is necessary to detect them to choose the best tests in function of each trial. Laboratory investigations can be used to evaluate the response to drug administration. Ethical point of view is represented by the fact that old people with cognitive impairment must not be away from therapeutic progress. In this field we must consider carefully the consequences of cognitive impairment on patient judgment and consent to clinical trial. Legal problems are regulated by supranational rules and French directives of Huriet law.

Angiomyolipoma is a rare tumor, frequently associated with tuberous sclerosis, but not observed in its isolated form. Usually, patients with tuberous sclerosis presented with pathognomonic cutaneous and central nervous system lesions. But the thesis of "formes frustes" without typical stigmata was suggested. A case of bilateral and multifocal angiomyolipoma in a young woman without evidence of phacomatosis is reported. The association with a pulmonary lymphangiomatosis make us suspected "a forme fruste" of tuberous sclerosis.

Angiomyolipoma is a rare tumor, frequently associated with tuberous sclerosis, but not observed in its isolated form. Usually, patients with tuberous sclerosis presented with pathognomonic cutaneous and central nervous system lesions. But the thesis of 'formes frustes'' without typical stigmata was suggested. A case of bilateral and multifocal angiomyolipoma in a young woman without evidence of phacomatosis is reported. The association with a pulmonary lymphangiomatosis make us suspected ''a forme fruste '' of tuberous sclerosis.

Clinical trials for cognitive disorders in the elderly require specific methodological guidelines. They must take into account the psychosocial dimension of the patient and his family and must be based on serious neurobiologic knowledge. In degenerative dementias the progress of research concern genetics, molecular intercellular recognition and astrocytic cells. Biology of cognition like hippocampal long term potentiation provides good pharmacologic basis for trials. In normal brain aging several ways must be developed : aminergic systems, free radicals, excitotoxic amino-acid, nerves growth factors. Clinical trials bring informations for pharmacology and epidemiology. Cholinergic neurons are the main pharmacologic target but there are many other ones : GABA-ergic system, Tau protein, amyloid. A rigourous selection of patients allows to precise the nosology of illness responsible of cognitive disorders and to point-out early clinical signs that represent a more sensitive target. Diagnostic criteria are useful in Alzheimer's disease, memory impairment, vascular dementias and other dementias. Evaluation of stage and evolution of dementia, comorbidity, limits of age and caregiver are practical problems. The effects of drugs used to treat cognitive functions are subtle so if is necessary to detect them to choose the best tests in function of each trial. Laboratory investigations can be used to evaluate the response to drug administration. Ethical point of view is represented by the fact that old people with cognitive impairment must not be away from therapeutic progress. In this field we must consider carefully the consequences of cognitive impairment on patient judgment and consent to clinical trial. Legal problems are regulated by supranational rules and french directives of Huriet law.

The Kasabach-Merritt syndrome was first described in children with cutaneous hemangiomas, but it can exceptionally be associated with visceral hemangiomas, especially in adults. Clotting and fibrinolysis within the hemangioma are thought to cause the coagulopathy observed in the so-called Kasabach-Merrit syndrome. This localised from of intra-vascular coagulation can progress to a secondary increased systemic fibrinolysis with fatal outcome for 20 to 30% of the patients. A transient control of hematologic abnormalities can frequently be obtained with blood product support (platelets, fibrinogen, fresh plasma, cryoprecipitates) and heparinotherapy. But in the adult, the only radical alternative is surgical excision if technically feasible. We reported here the case of a 43 year-old woman with a giant unresecable hepatic hemangioma complicated with a Kasabach-Merrit syndrome.

Initialement décrit chez l'enfant porteur d'un hémangiome cutané, le syndrome de Kasabach-Merritt peut exceptionnellement être observé chez l'adulte, l'hémangiome étant alors plus souvent viscéral. Le syndrome de Kasabach-Merritt est caractérisé par une coagulation intravasculaire localisée au niveau de l'hémangiome, pouvant être responsable d'une coagulopathie de consommation systémique d'issue fatale dans 20 à 30% des cas. Les traitements substitutifs (plaquettes, fibrinogène, plasma frais, cryoprécipités) et l'héparine permettent souvent un contrôle temporaire des troubles de l'hémostase. Le seul traitement radical chez l'adulte est étiologique et consiste en une chirurgie d'exérèse chaque fois qu'elle s'avère possible. Nous rapportons un cas de syndrome de Kasabach-Merritt observé chez une femme de 43 ans porteuse d'un hémangiome caverneux géant du foie inextirpable.

Introduction. The prevalence of mucocutaneous herpetic infection is especially high in patients with the acquired immunodeficiency syndrome (AIDS). In these patients, 8 p. 100 of the chronic or recurrent herpetic lesions are due to a mutant strain. Case Report. A case of very large (900 cm2) genital chronic herpetic infection is reported in a patient with AIDS (stade C3-CDC/WHO 1993). It was caracterised by an acyclovir resistant strain which emerged after several anterior treatment with acyclovir. A treatment with foscarnet was administered and clinical improvement was observed as early as the fourth day, with complete reepithelialization 50 days later. Discussion. We discussed essentially the pathogenicity of the herpetic infections due to mutant strains in immunocompromised patients and on the therapeutic modalities. At the present time, foscarnet is the treatment of choice for acyclovir-resistant mucocutaneous herpes simplex.

L'angiomyolipome est une tumeur rare, qui survient soit comme entité clinique isolée, soit dans le cadre de la sclérose tubéreuse de Bourneville. Bien que les sujets atteints de la maladie de Bourneville présentent des lésions cérébrales, cutanées et polyviscérales considérées comme « caractéristiques , l'hypothèse de l'existence de formes frustes a déjà été évoquée. Nous présentons un cas d'angiomyolipome rénal chez une femme de 30 ans, dont la bilatéralité et la multifocalité, et l'association à une lymphangiomyomatose pulmonaire, nous a fait suspecter une forme fruste de sclérose tubéreuse de Bourneville.

The Kasabach-Merritt syndrome was first described in children with cutaneous hemangiomas, but it can exceptionally be associated with visceral hemangiomas, especially in adults. Clotting and fibrinolysis within the hemangioma are thought to cause the coagulopathy observed in the so-called Kasabach-Merritt syndrome. This localised form of intra-vascular coagulation can progress to a secondary increased systemic fibrinolysis with fatal outcome for 20 to 30% of the patients. A transient control of hematologic abnormalities can frequently be obtained with blood product support (platelets, fibrinogen, fresh plasma, cryoprecipitates) and heparinotherapy. But in the adult, the only radical alternative is surgical excision if technically feasible. We reported here the case of a 43 year-old woman with a giant unresectable hepatic hemangioma complicated with a Kasabach-Merritt syndrome.