This neoplasm is not reportable.

Name

Hemophagocytic syndrome

Not Reportable

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
Primary hemophagocytic lymphohistiocytosis

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).

For more information: http://ghr.nlm.nih.gov/condition/familial-hemophagocytic-lymphohistiocytosis

Abstractor Notes

For more information, see the Histiocytosis Association website (http://www.histio.org/hemophagocyticsyndromes#.V1cWaUYw0eU)
Glossary