Subject(s)
Adrenal Insufficiency/etiology , Adrenocorticotropic Hormone/deficiency , Brain Injuries/complications , Alcoholism/complications , Humans , Hypoglycemia/etiology , Male , Middle Aged , Pituitary Gland, Anterior/injuries , Pituitary Gland, Anterior/metabolism , Recurrence , Subarachnoid Hemorrhage/etiologyABSTRACT
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Subject(s)
Humans , Male , Adult , Adrenocorticotropic Hormone/deficiency , Hyponatremia/complications , Hyponatremia/diagnosis , Hypoglycemia/complications , Adrenal Glands , Adrenal Glands/pathology , Adrenocorticotropic Hormone/administration & dosage , Adrenocorticotropic Hormone/adverse effects , Radiography, Thoracic , Glucocorticoids/deficiency , Androgens/deficiency , Adrenal Cortex Hormones/deficiency , Hyperprolactinemia/complicationsABSTRACT
Haemophagocytic syndrome is a rare disorder of immune regulation. Clinical features are fever, haemophagocytosis, hepatosplenomegaly, pancytopenia, lymph nodes enlargement, hypertriglyceridaemia and coagulopathy. 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 monocytes and macrophages 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 coagulopathy 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.
Subject(s)
Histiocytosis, Non-Langerhans-Cell/complications , Lymphoma, T-Cell, Cutaneous/complications , Skin Neoplasms/complications , Adult , Autopsy , Fatal Outcome , Histiocytosis, Non-Langerhans-Cell/pathology , Histiocytosis, Non-Langerhans-Cell/therapy , Humans , Lymphoma, T-Cell, Cutaneous/pathology , Lymphoma, T-Cell, Cutaneous/therapy , Male , Phagocytosis/physiology , Skin Neoplasms/pathology , Skin Neoplasms/therapy , SyndromeABSTRACT
El síndrome hemofagocítico (SHF) es un trastorno infrecuente de la inmunorregulación, cuyas manifestaciones clínicas son fiebre, hemofagocitosis, hepatoesplenomegalia, pancitopenia, linfadenopatías, hipertrigliceridemia y coagulopatía. Puede cursar también con afectación pulmonar, fracaso renal agudo o síndrome de secreción inadecuada de ADH (SIADH).Se ha descrito asociado a múltiples enfermedades, destacando los linfomas, sobre todo de células T, infecciones y enfermedades sistémicas, entre otras entidades. Las células hematopoyéticas son fagocitadas por monocitos y macrófagos en los ganglios linfáticos, médula ósea, hígado y bazo, lo que constituye un criterio diagnóstico del SHF. El tratamiento es difícil e incluye medidas de soporte y de las alteraciones de coagulación así como corticoides sistémicos a altas dosis e inmunosupresores, aunque en la mayoría de ocasiones no hay respuesta al tratamiento y la evolución es fatal. Los linfomas T que afectan al tejido celular subcutáneo y simulan una paniculitis han sido clasificados recientemente como linfomas cutáneos de células T (LCCT), siendo infrecuente su presentación como nódulos inflamatorios en extremidades inferiores (AU)