European journal of haematology · 2018
Chronic lymphoproliferative disorder of NK-cells: A single-institution review with emphasis on relative utility of multimodality diagnostic tools.
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Kurt Habibe, Jorgensen Jeffrey L, Amin Hesham M, Patel Keyur P, Wang Sa A, Lin Pei, Kanagal-Shamanna Rashmi, Loghavi Sanam, Thakral Beenu, Khogeer Haitham A, Jabbour Elias J, Li Shaoying, Yin C Cameron, Medeiros L Jeffrey, Khoury Joseph D
Аннотация
BACKGROUND: Chronic lymphoproliferative disorder of NK-cells (CLPD-NK) manifests as a persistent increase (≥2 × 109 /L, for > 6 months) of mature NK-cells in peripheral blood with an indolent clinical course. The disease is rare, and only limited case series have been published.
METHODS: We retrospectively studied 11 patients with CLPD-NK diagnosed at our institution between 2005 and 2017.
RESULTS: Patients included 7 men and 4 women with a median age of 60 years (range, 25-89 years). Ten patients (91%) had cytopenias. Bone marrow involvement by CLPD-NK ranged from 5-15%. The most commonly detected antigenic aberrancies by low cytometry immunophenotyping were as follows: CD7decreased/dim (30%), CD8uniform+ (36%), CD56-/partial (73%), CD94bright (55%), and KIR restriction (100%). JAK/STAT pathway mutations were detected in 8 of 10 (80%) patients and involved STAT3 (n = 7) and JAK3 (n = 1). The presence of mutations tended to correlate with the occurrence of other cytopenias (anemia/thrombocytopenia) and requirement for treatment. Seven patients received single-agent therapy, with amelioration of symptoms; 4 patients were observed. There were no disease-associated deaths or progression to more aggressive disease during the follow-up interval (median, 17 months).
CONCLUSIONS: Patients with CLPD-NK have an indolent clinical course and frequent hematologic manifestations that are responsive to single-agent therapy. Mutations in STAT3 are common and portend more pronounced clinical manifestations.
METHODS: We retrospectively studied 11 patients with CLPD-NK diagnosed at our institution between 2005 and 2017.
RESULTS: Patients included 7 men and 4 women with a median age of 60 years (range, 25-89 years). Ten patients (91%) had cytopenias. Bone marrow involvement by CLPD-NK ranged from 5-15%. The most commonly detected antigenic aberrancies by low cytometry immunophenotyping were as follows: CD7decreased/dim (30%), CD8uniform+ (36%), CD56-/partial (73%), CD94bright (55%), and KIR restriction (100%). JAK/STAT pathway mutations were detected in 8 of 10 (80%) patients and involved STAT3 (n = 7) and JAK3 (n = 1). The presence of mutations tended to correlate with the occurrence of other cytopenias (anemia/thrombocytopenia) and requirement for treatment. Seven patients received single-agent therapy, with amelioration of symptoms; 4 patients were observed. There were no disease-associated deaths or progression to more aggressive disease during the follow-up interval (median, 17 months).
CONCLUSIONS: Patients with CLPD-NK have an indolent clinical course and frequent hematologic manifestations that are responsive to single-agent therapy. Mutations in STAT3 are common and portend more pronounced clinical manifestations.
Предметные рубрики MeSH
AdultAgedAged, 80 and overBiomarkersBiopsyBone MarrowChronic DiseaseFemaleFlow CytometryHumansImmunohistochemistryImmunophenotypingIn Situ Hybridization, FluorescenceJanus KinasesKiller Cells, NaturalLeukemia, Large Granular LymphocyticLymphoproliferative DisordersMaleMiddle AgedRetrospective Studies