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.
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.
Authors: Ivonne A Montes-Mojarro; Bo-Jung Chen; Ana F Ramirez-Ibarguen; Carmen M Quezada-Fiallos; Wendy B Pérez-Báez; Daniela Dueñas; Sandro Casavilca-Zambrano; Marcela Ortiz-Mayor; Erica Rojas-Bilbao; Hernan García-Rivello; Maria F Metrebian; Marina Narbaitz; Carlos Barrionuevo; Carmen Lome-Maldonado; Irina Bonzheim; Falko Fend; Julia Steinhilber; Leticia Quintanilla-Martinez Journal: Mod Pathol Date: 2019-12-10 Impact factor: 7.842
Authors: Noemí Muñoz-García; María Jara-Acevedo; Carolina Caldas; Paloma Bárcena; Antonio López; Noemí Puig; Miguel Alcoceba; Paula Fernández; Neus Villamor; Juan A Flores-Montero; Karoll Gómez; María Angelina Lemes; Jose Carlos Hernández; Iván Álvarez-Twose; Jose Luis Guerra; Marcos González; Alberto Orfao; Julia Almeida Journal: Cancers (Basel) Date: 2020-11-25 Impact factor: 6.639