lymphohistiocytose hémophagocytaire

CONT: Les lymphohistiocytoses hémophagocytaires (LH) se distinguent en primitives et secondaires. Dans le premier cas, la LH est liée à un déficit immunitaire primitif. Les LH secondaires sont liées à différents états pathologiques (infections en particulier, ainsi qu'affections néoplasiques et auto-immunes) qui aboutissent au tableau de LH à cause d'un déficit acquis des fonctions cytotoxiques des lymphocytes T et des cellules «natural killer».
PHR: Lymphohistiocytose hémophagocytaire familiale, primaire, primitive, secondaire.

CONT: Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes that commonly appears in infancy, although it has been seen in all age groups. ... The pathological hallmark of this disease is the aggressive proliferation of activated macrophages and histiocytes, which phagocytose other cells, namely RBCs [red blood cells], WBCs [white blood cells], and platelets, leading to the clinical symptoms. The uncontrolled growth is nonmalignant and does not appear clonal ... The spleen, lymph nodes, bone marrow, liver, skin, and membranes that surround the brain and spinal cord are preferential sites of involvement.
PHR: Acquired, familial, primary, secondary hemophagocytic lymphohistiocytosis.