RESUMO
Mastocytoses are diseases caused by proliferating mast cells infiltrating one or more organs. The spectrum of mastocytosis includes the cutaneous forms urticaria pigmentosa and solitary mastocytoma (about 90% of mastocytoses) and systemic forms affecting other organs. Infiltrates are most often found in the bone marrow, spleen, lymph nodes and liver, but any organ may be affected. Patients with systemic mastocytosis may or may not have urticaria pigmentosa. About 35% of patients without urticaria pigmentosa have an associated malignant haematological disease and a poor prognosis. Symptoms caused by mast cell mediator release are best treated with antihistamines, but several other drugs may be used if the response is unsatisfactory. Many antineoplastic drugs have been tried to combat aggressive mastocytoses and mast cell leukaemia, but the results have been disappointing.
Assuntos
Mastocitose , Adulto , Feminino , Humanos , Mastocitose/diagnóstico , Mastocitose/tratamento farmacológico , Mastocitose/patologiaRESUMO
The term cryopathies includes conditions in which abnormal sensitivity to cold is a prominent feature and includes the cold agglutinin syndrome, the cold hemolysin syndrome, the cold urticarias, the cryoglobulinemias, and cryofibrinogenemia. The cryopathies may be secondary to lymphoproliferative, autoimmune, and infectious diseases, but in many patients no underlying disease can be found (essential cryopathy). Avoidance of cold is of prime importance in all patients. Underlying disease should be treated, if possible. Severe therapeutic problems may arise in patients with essential cryopathies.
