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This neoplasm is not reportable.
Name
Hemophagocytic syndrome
Reportability
This neoplasm is not reportable
Alternate Names
Familial erythrophagocytic lymphohistiocytosis
Familial hemophagocytic histiocytosis
Familial hemophagocytic lymphocytosis
Familial hemophagocytic lymphohistiocytosis
Familial hemophagocytic reticulosis
FEL
FHL
FHLH
HPLH
Definition
Familial hemophagocytic lymphohistiocytosis is a disorder in which the immune system produces too many activated immune cells (lymphocytes) called T cells, natural killer cells, B cells, and macrophages (histiocytes).
Familial hemophagocytic lymphohistiocytosis also destroys blood-producing cells in the bone marrow, a process called hemophagocytosis. As a result, affected individuals have low numbers of red blood cells (anemia) and a reduction in the number of blood cells involved in clotting (platelets). A reduction in platelets may cause easy bruising and abnormal bleeding.
Affected individuals also have an increased risk of developing cancers of blood-forming cells (leukemia and lymphoma).
Familial hemophagocytic lymphohistiocytosis also destroys blood-producing cells in the bone marrow, a process called hemophagocytosis. As a result, affected individuals have low numbers of red blood cells (anemia) and a reduction in the number of blood cells involved in clotting (platelets). A reduction in platelets may cause easy bruising and abnormal bleeding.
Affected individuals also have an increased risk of developing cancers of blood-forming cells (leukemia and lymphoma).
Sources
National Center for Advancing Translational Sciences
Section: Genetic and Rare Disease Information Center
Pages: https://rarediseases.info.nih.gov/diseases/6589/hemophagocytic-lymphohistiocytosis
Section: Genetic and Rare Disease Information Center
Pages: https://rarediseases.info.nih.gov/diseases/6589/hemophagocytic-lymphohistiocytosis
