Publication Date:
2019-11-13
Description:
The large granular lymphocyte (LGL) syndrome and large granular lymphocytic leukemia (LGLL) are rare hematological disorders. Through the Severe Chronic Neutropenia International Registry (SCNIR) we have followed the clinical course and treatment responses of 27 LGL patients presenting with severe chronic neutropenia without evidence of leukemia, rheumatoid arthritis or autoimmune diseases. This report focuses on their treatment responses to G-CSF and outcomes with and without other immunosuppressive therapies. Methods: Patients with severe chronic neutropenia and a diagnosis of LGL syndrome (at least 3 ANC values 〈 0.5 x 109/L over a 3 month period) were enrollment in the SCNIR if they do not have concomitant systemic autoimmune disease, myeloid or lymphoid malignancy or drug-induced neutropenia. Findings: The 27 LGL patients (8 males, median age 53; 19 females, median age 57) had chronic neutropenia for approximately 4 years (range 0.4 - 25.6 years) before enrollment. Diagnoses were primarily based on FACS analysis (12 bone marrow [50%]; 11 blood and bone marrow [46%]; 1 blood only [4%]). None of these patients presented with lymphocytosis, i.e., ALC 〉 5.0 x 109/L. Before enrollment, 7 patients had anti-neutrophil antibody tests (3 positive, 4 negative), 4 patients had antinuclear antibody tests (2 positive, 2 negative), and ANCA was positive for the one patient tested. After enrollment, additional results were: 1 patient had a negative anti-neutrophil antibody, 8 patients had antinuclear antibody tests (2 positive, 6 negative), and ANCA was positive in two additional patients. The diagnosis of LGL was based on increased LGL cells in the blood or bone marrow, most frequently increased CD 3+, CD 8+, CD 57+ cells. Increases in lymphocytes in the marrow, often in clusters were a common finding; lymphocytes in the marrow ranged from 6-79%. The range of CD 57+ in blood 11-19%, bone marrow 19-61%; CD 56+/NK cells in blood 0.06-14%, bone marrow
Print ISSN:
0006-4971
Electronic ISSN:
1528-0020
Topics:
Biology
,
Medicine
Permalink