The onset of<unk>  occurs before the age of one year in approximately 70 percent of cases. Familial<unk>  should be suspected if siblings are diagnosed with<unk>  or if symptoms recur when therapy has been stopped. Familial<unk>  is an autosomal recessive disease, hence each sibling of a child with familial<unk>  has a twenty-five–percent chance of developing the disease, a fifty-percent chance of carrying the defective gene (which is very rarely associated with any risk of disease), and a twenty-five–percent chance of not being affected and not carrying the gene defect. Patients with<unk> , especially when untreated, may need intensive therapy. Therefore,<unk>  should be included in the differential diagnosis of intensive care unit patients with cytopenia and hyperferritinemia. Patients in the earlier stages of<unk>  are frequently hospitalized at internal medicine wards.<unk>  clinically manifests with fever, enlargement of the liver and spleen, enlarged lymph nodes, yellow discoloration of the skin and eyes, and a rash. Laboratory findings may include elevated triglyceride levels, low fibrinogen levels, transaminitis, and elevated ferritin levels (among others).
Hemophagocytic lymphohistiocytosis
Diffuse infiltrative lymphocytosis syndrome
Congenital self-healing reticulohistiocytosis
Thymoma with immunodeficiency
Langerhans cell histiocytosis
Blastic plasmacytoid dendritic cell neoplasm
Hereditary pyropoikilocytosis
Angioimmunoblastic T-cell lymphoma