Hemophagocytic lymphohistiocytosis natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Complications

• Complications include
  • Immunodeficiency
  • Blindness
  • Coma
  • Leukemia and lymphoma
  • Posterior reversible encephalopathy syndrome

Prognosis

• Secondary HLH in some individuals may be self-limited because patients are able to fully recover after having received only supportive medical treatment. However, long-term remission without the use of cytotoxic and immune-suppressive therapies is unlikely in the majority of adults with HLH and in those with CNS involvement.
• Without treatment, most people with familial hemophagocytic lymphohistiocytosis survive only a few months.