The lymphoproliferative disease of granular lymphocytes. A heterogeneous disorder ranging from indolent to aggressive conditions

Abstract

A multiparameter analysis, which included the evaluation of clinical features, cell morphology, karyotype, phenotypic and functional immunologic findings, and T-cell receptor beta-chain configuration was performed on 34 patients with lymphoproliferative disease of granular lymphocytes (LDGL). The two-fold aim of the study was to identify the most useful tools that would more accurately characterize these patients and to deal with the problem of classifying these lymphoproliferative disorders. The data presented in this article suggest that a single parameter may not be sufficient to define the nature of the proliferating cells or to predict the clinical course of the disease and prognosis for the patient. The use of a multiparameter approach, however, may reach this goal, thus providing important prognostic and therapeutic information. Our study supports the concept that lymphoproliferative disease of granular lymphocytes is a heterogeneous disorder that ranges from indolent and possibly reactive conditions to the manifestation of aggressive malignancies.

Features of large granular lymphocytes (LGL) expansion following allogeneic stem cell transplantation: a long-term analysis

“…18 Although few cases of LGL expansion below 2 × 10 9 /l have been reported, 19 only patients presenting a significant number of lymphocytes (Ͼ2 × 10 9 /l) with LGL features were retained in this study as presenting a LGL expansion. 20 Morphological analysis of LGL was carried out on peripheral blood mononuclear cells and marrow aspirates and was systematically reviewed by two independent pathologists (DS and CA). The following monoclonal antibodies were used for immunophenotyping according to standard procedures: 21 CD2, CD3, CD4, CD8, CD14, CD16, CD25, CD28, CD45 RA, CD45 RO, CD56, CD57, anti-HLA-DR and a panel of commercially available anti-V␤ segments for clonality determination.…”

Large granular lymphocyte disorders: new etiopathogenetic clues as a rationale for innovative therapeutic approaches

“…[1][2][3] Polyclonal expansions of LGL are usually transient and due to a viral infection, such as Epstein-Barr virus (EBV) or cytomegalovirus (CMV), neoplasm or autoimmune diseases [1][2][3] ; sometimes these disorders develop after splenectomy. In contrast, clonal LGL proliferations are stably maintained for time whether or not the patients are symptomatic.…”

Variant lymphoproliferative disorder of granular lymphocytes (LDGL) following Hodgkin lymphoma

“…The diagnosis should be considered in patients presenting with cytopenia or autoimmune disease, particularly rheumatoid arthritis. ''Standard'' criteria for diagnosis of LDGL include evidence of granular lymphocytosis >2,000/mL lasting more than 6 months [8,9]. However, with the availability of newer diagnostic tools such as cytogenetic, molecular, and phenotypic markers, it is possible to document the diagnosis and establish clonality in patients with low LGL counts [10].…”