Presse Med
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Systemic mastocytosis is characterized by abnormal mast cell proliferation in different organs. The 2001 consensus classification distinguishes in separate categories indolent systemic mastocytosis, systemic mastocytosis with concomitant blood disease, aggressive systemic mastocytosis and mast cell leukemia. Clinical manifestations are caused by tissue infiltration by proliferating mastocytes and by release of mediators. ⋯ Serum tryptase levels reflect the total mast cell burden. Treatment must prevent release of mast cell mediators (histamine antagonists, cromolyn sodium, corticosteroids, or leukotriene-receptor inhibitors), limit bone involvement (bisphosphonates) and reduce the number of circulating mast cells (interferon, cladribine, or tyrosine kinase inhibitors). Enhanced understanding of the pathogenic mechanisms (mutation of c-kit and platelet-derived growth factor receptor alpha has led to the development of targeted treatments, including new inhibitors of tyrosine kinase and of nuclear factor Kappa B.
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Clinical analysis of multisystem amyloidosis in Lebanon, by histological type. ⋯ Multisystem amyloidosis is frequent in Lebanon and familial Mediterranean fever is still frequently associated with the secondary type. Accurate diagnosis and classification are essential for the prognosis and treatment of the disease. Poor prognosis was associated with the AL type, especially when accompanied by myeloma, and with cardiac amyloidosis.
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When psychological and behavioral disorders of Alzheimer's disease appear suddenly, somatic, iatrogenic and reactive or relational psychological causes must be ruled out or treated before concluding that the cause is lesional. Non-pharmacological interventions should be privileged for the prevention and management of behavioral manifestations of mild to moderate intensity: psychological support of the patient (short therapies), training the caregiver, work on daily habits, reorganization of the home, behavioral measures against apathy and especially agitation, rehabilitation strategies, and therapy involving music, light, aromas, etc. Pharmacological therapies are only moderately effective in these disorders. ⋯ Atypical neuroleptics are better tolerated than the classic ones. They are most effective in this context but must be reserved for specific indications and limited in time because of the increased risk of stroke. Other psychotropics (benzodiazepines, carbamates, antiepileptics) should be used cautiously in this context.