Griscelli syndrome type 2

MeSH: C537302ORPHA: 79477

Overview

Griscelli syndrome characterized by silvery gray sheen of the hair, hypopigmentation of the skin and immunodeficiency with or without neurologic impairment that has material basis in mutation in the RAB27A gene on chromosome 15q21.3

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Griscelli syndrome type 2, sourced from HPO and Orphanet clinical annotations.

JaundicePetechiaeSeizureHypertoniaSplenomegalyDecreased total neutrophil countPancytopeniaFeverNausea and vomitingPulmonary infiltratesPremature graying of hairHepatomegalyLymphadenopathyImmunodeficiencyHyperlipidemiaHypopigmentation of hairPartial albinismIris hypopigmentationHemophagocytosis

Classification & Codes

MeSH Code

C537302

Orphanet Code

ORPHA:79477
Griscelli syndrome type 2
MeSHC537302
OrphanetORPHA:79477
Treatments0 drug(s)
Symptoms on record19 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO