Bruno Fattizzo1, Anna Zaninoni1, Dario Consonni2, Alberto Zanella1, Umberto Gianelli3, Agostino Cortelezzi4, Wilma Barcellini5. 1. U.O. Oncoematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. 2. U.O. Epidemiologia, Dipartimento della Medicina Preventiva, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. 3. Servizio di Ematopatologia, U.O. di Patologia, Dipartimento di Fisiopatologia e Trapianti, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano e Università degli Studi di Milano, Italy. 4. U.O. Oncoematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano e Università degli Studi di Milano, Italy. 5. U.O. Oncoematologia, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. Electronic address: wbarcel@policlinico.mi.it.
Abstract
AIM: To evaluate infections and oncohematologic evolution in adult patients with chronic idiopathic and autoimmune neutropenia in a prospective study. PATIENTS AND METHODS: 76 consecutive patients were enrolled from September 2008 to April 2012. Complete blood counts and clinical evaluation were performed at enrolment, at month 3, 6, and then every 6 months. Anti-neutrophil antibodies were tested by GIFT method. RESULTS: Patients (49 chronic idiopathic- and 27 autoimmune neutropenia) were followed for a median of 5 years (range 24-84 months). At enrolment, neutropenia was mild in 44 patients (median neutrophils 1.27×10(3)/μL), moderate in 23 (median 0.8×10(3)/μL), and severe in 9 (median 0.4×10(3)/μL). Neutrophil counts showed a great inter-subject but no intra-subject variability, with lower values in autoimmune neutropenia, in males, and in MGUS cases. Over time, no grade >3 infections occurred; 13/49 chronic idiopathic and 6/27 autoimmune neutropenia patients experienced a grade 2 event, irrespective of mean and nadir neutrophil values. Bone marrow evaluation at enrolment showed reduced cellularity in 23% of cases, and dyserythropoietic features in 55%, with no definite hematologic diagnosis. During the follow-up, 5 cases were diagnosed with NK expansion, 4 with hairy cell leukemia, and 3 with myelodysplasia (1 myelomonocytic leukemia, 1 refractory cytopenia with unilineage dysplasia, and 1 multilineage dysplasia), with a median time to evolution of 30 months. CONCLUSION: Chronic idiopathic and autoimmune neutropenia, although usually benign, deserve hematological follow-up with a bone marrow evaluation at diagnosis and a re-evaluation in the presence of worsening neutropenia, appearance of additional cytopenias, and lymphocytosis.
AIM: To evaluate infections and oncohematologic evolution in adult patients with chronic idiopathic and autoimmune neutropenia in a prospective study. PATIENTS AND METHODS: 76 consecutive patients were enrolled from September 2008 to April 2012. Complete blood counts and clinical evaluation were performed at enrolment, at month 3, 6, and then every 6 months. Anti-neutrophil antibodies were tested by GIFT method. RESULTS:Patients (49 chronic idiopathic- and 27 autoimmune neutropenia) were followed for a median of 5 years (range 24-84 months). At enrolment, neutropenia was mild in 44 patients (median neutrophils 1.27×10(3)/μL), moderate in 23 (median 0.8×10(3)/μL), and severe in 9 (median 0.4×10(3)/μL). Neutrophil counts showed a great inter-subject but no intra-subject variability, with lower values in autoimmune neutropenia, in males, and in MGUS cases. Over time, no grade >3 infections occurred; 13/49 chronic idiopathic and 6/27 autoimmune neutropeniapatients experienced a grade 2 event, irrespective of mean and nadir neutrophil values. Bone marrow evaluation at enrolment showed reduced cellularity in 23% of cases, and dyserythropoietic features in 55%, with no definite hematologic diagnosis. During the follow-up, 5 cases were diagnosed with NK expansion, 4 with hairy cell leukemia, and 3 with myelodysplasia (1 myelomonocytic leukemia, 1 refractory cytopenia with unilineage dysplasia, and 1 multilineage dysplasia), with a median time to evolution of 30 months. CONCLUSION: Chronic idiopathic and autoimmune neutropenia, although usually benign, deserve hematological follow-up with a bone marrow evaluation at diagnosis and a re-evaluation in the presence of worsening neutropenia, appearance of additional cytopenias, and lymphocytosis.
Authors: David C Dale; Audrey Anna Bolyard; James A Shannon; James A Connelly; Daniel C Link; Mary Ann Bonilla; Peter E Newburger Journal: Blood Adv Date: 2022-07-12
Authors: Bruno Fattizzo; Marc Michel; Juri Alessandro Giannotta; Dennis Lund Hansen; Maria Arguello; Emanuele Sutto; Nicola Bianchetti; Andrea Patriarca; Silvia Cantoni; María Eva Mingot-Castellano; Vickie McDonald; Marco Capecchi; Anna Zaninoni; Dario Consonni; Josephine Mathilde Vos; Nicola Vianelli; Frederick Chen; Andreas Glenthøj; Henrik Frederiksen; Tomás José González-López; Wilma Barcellini Journal: Blood Adv Date: 2021-12-28