T‐γ large granular lymphocyte leukemia associated with amegakaryocytic thrombocytopenic purpura, Sjögren's syndrome, and polyglandular autoimmune syndrome type II, with subsequent development of pure red cell aplasia

T cell large granular lymphocytic (LGL) leukemia is a rare condition accounting for 2–3% of all mature lymphoid leukemias. Here, we present the case of a 73-year-old woman presenting with neutropenia and anemia (hemoglobin 9.9 g/dl). Hematological assessment revealed the presence of a T cell LGL leukemia. At the time of T cell LGL leukemia diagnosis, the patient developed xerophthalmia and xerostomia, and a diagnosis of Sjögren’s syndrome was made following salivary gland biopsy. The finding of large granular lymphocytes in the context of autoimmune disorders is well-known, though it often occurs with rheumatoid arthritis or in association with a positive autoantibody titer in the absence of an overt clinical picture. The concomitant presentation of T cell LGL leukemia with Sjögren’s syndrome is a rare event which is worth reporting. Our patient was managed with immunosuppressive therapy and is still alive.

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“…These include cases of thrombotic thrombocytopenic purpura, pure red cell aplasia, Behcet’s syndrome and Sjögren’s syndrome [9,10]. …”

Section: Discussionmentioning

confidence: 99%

T Cell Large Granular Lymphocytic Leukemia in Association with Sjögren’s Syndrome

Franco

Palazzolo²,

Liardo³

et al. 2010

Acta Haematol

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“…LGL leukemia associated muscular and glandular diseases occur in forms of myositis [136] and other musculoskeletal symptoms [58], Sjögren syndrome [52, 126, 129, 137, 138], autoimmune thyroiditis (Hashimoto’s disease) [50, 52, 94, 129, 138], autoimmune polyglandular syndrome (APS) [138], endocrinopathy, Grave’s disease (hyperparathyroidism) and Cushing’s syndrome. [52, 94] Other connective tissue disorders associated with LGL leukemia involve systemic lupus erythematosus (SLE) [94, 129, 139], Behçet disease [140], uveitis and celiac disease [141].…”

Section: Disorders Related To Indolent Lgl Leukemiamentioning

confidence: 99%

The root of many evils: indolent large granular lymphocyte leukaemia and associated disorders

Zhang

Shah

Loughran

2009

Hematological Oncology

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Large granular lymphocyte (LGL) leukemia can arise from either natural killer (NK) cells or cytotoxic T lymphocytes (CTL). The T-cell form of LGL leukemia has significant overlap with other hematological disorders and autoimmune diseases. Here we provide an overview of LGL biology. We also focus discussion on the indolent LGL leukemia related disorders and their causal relationships. We then discuss the potential relationships and distinctions between indolent LGL leukemia and non-malignant clonal lymphocyte expansion that occur in otherwise healthy individuals, especially elder people.

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“…1,10 LGL-leukemia-associated paraneoplastic syndrome can involve isolated cytopenias. 35,[43][44][45][46][47] Some authors hypothesize that the mechanisms of cytopenia include secretion of Fas-L/soluble Fas receptors that are constitutively expressed by LGL cells and also found elevated in patient's serum. 48,49 However, lineage-specific cytopenias cannot be easily explained by displacement of hematopoiesis or a global cytokine effect.…”

Section: Introductionmentioning

confidence: 99%

Pathologic clonal cytotoxic T-cell responses: nonrandom nature of the T-cell–receptor restriction in large granular lymphocyte leukemia

Wlodarski

O’Keefe

Howe

et al. 2005

Blood

112

111

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T-cell large granular lymphocyte (T-LGL)leukemia is a clonal lymphoproliferation of cytotoxic T cells (CTLs) associated with cytopenias. T-LGL proliferation seems to be triggered/sustained by antigenic drive; it is likely that hematopoietic progenitors are the targets in this process. The antigen-specific portion of the T-cell receptor (TCR), the variable beta (VB)-chain complementarity-determining region 3 (CDR3), can serve as a molecular signature (clonotype) of a T-cell clone. We hypothesized that clonal CTL proliferation develops not randomly but in the context of an autoimmune response. We identified the clonotypic sequence of T-LGL clones in 60 patients, including 56 with known T-LGL and 4 with unspecified neutropenia. Our method also allowed for the measurement of clonal frequencies; a decrease in or loss of the pathogenic clonotype and restoration of the TCR repertoire was found after hematologic remission. We identified 2 patients with identical immunodominant CDR3 sequence. Moreover, we found similarity between multiple immunodominant clonotypes and codominant as well as a nonexpanded, "supporting" clonotypes. The data suggest a nonrandom clonal selection in T-LGL, possibly driven by a common antigen. In contrast, the physiologic clonal CTL repertoire is highly diverse and we were not able to detect any significant clonal sharing in 26 healthy controls.

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T‐γ large granular lymphocyte leukemia associated with amegakaryocytic thrombocytopenic purpura, Sjögren's syndrome, and polyglandular autoimmune syndrome type II, with subsequent development of pure red cell aplasia

Cited by 41 publications

References 11 publications

T Cell Large Granular Lymphocytic Leukemia in Association with Sjögren’s Syndrome

T Cell Large Granular Lymphocytic Leukemia in Association with Sjögren’s Syndrome

The root of many evils: indolent large granular lymphocyte leukaemia and associated disorders

Pathologic clonal cytotoxic T-cell responses: nonrandom nature of the T-cell–receptor restriction in large granular lymphocyte leukemia

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