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Hemophagocytic Lymphohistiocytosis

Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Hemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by excessive activation of the immune system resulting in over production of inflammatory cytokines...

DIAGNOSIS

HISTORY

  • High fever, jaundice, malaise are the common presenting symptoms.

  • Some patients can also present with a nonspecific rash, usually a purpuric morbilliform eruption.

  • Rarely neurologic man...

TREATMENT

GENERAL MEASURES

  • Short-term strategy: Control the hyperinflammatory state.

  • Long-term strategy: definitive treatment with allogeneic hematopoietic stem cell transplantation (HSCT)

  • Treat underlyi...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Monitor the patient for resolution of fevers, hepatosplenomegaly, and neurologic abnormalities. Labs should be monitored routinely for anemia, t...

REFERENCES

1
Sepulveda FE, de Saint Basile G. Hemophagocytic syndrome: primary forms and predisposing conditions. Curr Opin Immunol.  2017;49:20–26. [View Abstract on OvidMedline]
2
Henter JI, Horne...

CODES

ICD10

D76.1 Hemophagocytic lymphohistiocytosis 

ICD9

  • 288.4 Hemophagocytic syndromes

  • 288.8 Other specified disease of white blood cells

SNOMED

  • 234437005 Hemophagocytic lymphohistiocytosis

  • 724641002 Prim...

CLINICAL PEARLS

  • HLH is a rare clinical syndrome characterized by excessive activation of the immune system resulting in over production of inflammatory cytokines.

  • Primary HLH is usually seen in childre...

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