Abstract

ARTIGUES BARCELO, A. et al. Haemophagocytic syndrome and cutaneous T-cell lymphoma. An. Med. Interna (Madrid) [online]. 2004, vol.21, n.3, pp.37-40. ISSN 0212-7199.

Haemophagocytic syndrome is a rare disorder of immune regulation. Clinical features are fever, haemophagocytosis, hepatosplenomegaly, pancitopenia, lymph nodes enlargement, hypertriglyceridaemia and coagulopaty. Systemic findings may also include pulmonary infiltrates, renal failure and an inappropriate antidiuretic state. It has been described associated to many diseases, like lymphomas, most of them T-cell lymphomas, infections and systemic diseases, among other entities. Haemopoietic cells are actively ingested by monocites and macro-phages in lymph nodes, bone marrow, liver and spleen. This is a diagnostic criteria for haemophagocytic syndrome. The treatment of Haemophagocytic syndrome is difficult and requires intensive supportive therapy, treatment of coagulopaty disorders, high-dose corticosteroids and immunosuppressive treatment, although in most cases there is no response to treatment and the evolution is fatal. The T-cell lymphomas that involve subcutaneous tissue and simulate panniculitis are classified recently as primary cutaneous T-cell lymphomas, and their presentation as inflammatory nodules of the legs are uncommon.

Keywords : Haemophagocytic syndrome; Cutaneous T-cell lymphoma.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )