Emergency: requires immediate attention
NOCARH syndrome
Alerts and Notices
Synopsis
This syndrome presents with fever, rash, failure to thrive, hepatosplenomegaly, diarrhea, pancytopenia, and HLH starting in infancy. NOCARH syndrome is a fatal disease if not treated promptly; many pediatric patients have not survived this disease even with treatment.
Codes
ICD10CM:D61.818 – Other pancytopenia
D76.1 – Hemophagocytic lymphohistiocytosis
D76.3 – Other histiocytosis syndromes
M04.8 – Other autoinflammatory syndromes
SNOMEDCT:
234437005 – Hemophagocytic lymphohistiocytosis
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
- Hemophagocytic lymphohistiocytosis (HLH)
- Neonatal onset multisystem inflammatory disease (NOMID)
- Chronic infantile neurological cutaneous articular syndrome (CINCA-NOMID)
- Takenouchi-Kosaki syndrome
- Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature syndrome (CANDLE)
- NLRC4-related autoinflammatory syndrome
- Familial hemophagocytic syndrome
Best Tests
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Management Pearls
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Therapy
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References
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Last Reviewed:01/12/2022
Last Updated:08/30/2022
Last Updated:08/30/2022