The risk of hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type 2
Jessen B, Bode S, Ammann S, Chakravorty S, Davies G, Diestelhorst J, Frei-Jones M, Gahl W, Gochuico B, Griese M, Griffiths G, Janka G, Klein C, Kögl T, Kurnik K, Lehmberg K, Maul-Pavicic A, Mumford A, Pace D, Parvaneh N, Rezaei N, de Saint Basile G, Schmitt-Graeff A, Schwarz K, Karasu G, Zieger B, Stadt U, Aichele P, Ehl S. The risk of hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type 2. Blood 2013, 121: 2943-2951. PMID: 23403622, PMCID: PMC3624940, DOI: 10.1182/blood-2012-10-463166.Peer-Reviewed Original ResearchMeSH KeywordsAdaptor Protein Complex 3Adaptor Protein Complex beta SubunitsAdolescentAdultAnimalsChildChild, PreschoolCytotoxicity, ImmunologicFlow CytometryHermanski-Pudlak SyndromeHumansLymphohistiocytosis, HemophagocyticMiceMice, Inbred C57BLMice, KnockoutMutationrab GTP-Binding Proteinsrab27 GTP-Binding ProteinsRisk FactorsT-Lymphocytes, CytotoxicYoung AdultConceptsHermansky-Pudlak syndrome type 2Hemophagocytic lymphohistiocytosisLymphocyte cytotoxicityHPS2 patientsRAB27A mutationsPatients to hemophagocytic lymphohistiocytosisRisk of hemophagocytic lymphohistiocytosisFeatures of hemophagocytic lymphohistiocytosisHematopoietic stem cell transplantationHermansky-PudlakImpaired virus controlPerforin-deficient miceStem cell transplantationType 2Lymphocytic choriomeningitis virusCytotoxicity in vivoChediak-Higashi syndromeCytotoxicity defectsCell transplantationPatient yearsPearl micePatientsGenetic disordersMilder defectsDisease parameters
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