Lymphohistiocytosis: A multi-factorial syndrome of macrophagic activation clinico-pathological study of 38 cases

摘要: Lymphohistiocytosis (LH) is defined by a widespread infiltrate of non-malignant lymphocytes and macrophages, involving principally the liver, spleen central nervous system associated with severe lymphoid atrophy. For this histological study, 38 juvenile cases LH were selected reviewed. Morphological histochemical studies revealed that macrophages activated. They appeared to be OKM1, OKT9 HLA Dr positive showed patent or occult erythro thrombocytophagocytosis. Comparatively, most cells did not bear any immunoglobulin label wide panel monoclonal antibodies including T3, T4, T6, T8, T11. The review clinical biological data these suggests does represent single entity but can regarded as non-specific response various causes. Three different conditions isolated. first group consists patients in whom disease characterized an early onset, high familial incidence inevitably fatal course. This category contains largest number (29 out 38) corresponds classic description Familial Erythrophagocytic Reticulosis Farquhar Claireaux. second includes mainly male children over 2 years old. Inheritance predominant feature. course comparatively long. A viral infection was present three seven cases. shares many clinico-pathological features Virus Hemophagocytic Syndrome presumably related chronic latent infection. third identified on basis presence pigmentation abnormalities immunodeficiency disorders probably cytoskeletal abnormalities. Chediak-Higashi Griscelli's disease. fact some genetic factors are groups significant condition brought about multiple factors--of which common denominator apparent activation Mononuclear Phagocytic System.