Proliferation of Tγ Cells with Killer-Cell Activity in Two Patients with Neutropenia and Recurrent Infections

Aa, Bom-van Noorloos; Hg, Pegels; Rh, van Oers; Silberbusch, J.; Tm, Feltkamp-Vroom; Goudsmit, R; Wp, Zeijlemaker; Borne, von dem; Cj, Melief

doi:10.1056/nejm198004243021702

Cited by 133 publications

(23 citation statements)

References 21 publications

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“…In particular, normal peripheral blood does not contain large populations of CD16 ϩ T cells. 16,17,31,32 When it does, this phenotype correlates tightly with the ability to demonstrate clonal T-cell antigen receptor gene rearrangements and seems to characterize T-cell GLL. 16 Another phenotypic attribute that has been proposed as a relatively specific finding in T-cell GLL is coexpression of CD57 by T cells.…”

Section: Bone Marrow In T-cellmentioning

confidence: 86%

Distinct bone marrow findings in T-cell granular lymphocytic leukemia revealed by paraffin section immunoperoxidase stains for CD8, TIA-1, and granzyme B

Morice

Kurtin

Tefferi

et al. 2002

Blood

134

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Unlike other leukemia types in which the bone marrow findings are diagnostic, the bone marrow pathology of T-cell granular lymphocytic leukemia (GLL) is subtle and ill-defined. In this study, bone marrow biopsy specimens from 36 patients with T-cell GLL and from 25 control patients with cytopenias and relative or absolute increases in blood large granular lymphocytes were studied by immunohistochemistry using antibodies to the cytolytic lymphocyte antigens CD8, CD56, CD57, TIA-1, and granzyme B. The goals were to clarify the bone marrow pathology of T-cell GLL and to refine the diagnostic criteria for T-cell GLL. Most bone marrow specimens from the T-cell GLL patients contained interstitially distributed clusters of at least 8 CD8 ؉ (83%) or TIA-1 ؉ (75%) lymphocytes or clusters of at least 6 granzyme B ؉ (50%) lymphocytes. Interstitial clusters of CD8 ؉ , TIA-1 ؉ , or granzyme B ؉ cells were present in 36%, 12%, and 0%, respectively, of the control bone marrows (all values significantly different, P < .001). An additional T-cell GLL disease-specific finding was the presence of linear arrays of intravascular CD8 ؉ , TIA-1 ؉ , or granzyme B ؉ lymphocytes, found in 67% of cases of T-cell GLL and in none of the 25 control samples (P < .001). Staining for CD56 and CD57 was noncontributory. These findings clarify the bone marrow histopathology of T-cell GLL and provide an additional tool by which the discrete, abnormal lymphocyte population required for a diagnosis of T-cell GLL can be identified. IntroductionGranular lymphocytic leukemia (GLL), also termed large granular lymphocyte leukemia, is a chronic, indolent lymphoproliferative disorder with distinctive clinical and laboratory manifestations. [1][2][3][4][5] The hallmark feature of GLL is expansion of a discrete, or clonal, population of cytolytic lymphocytes (CTLs) in the peripheral blood. These lymphocytes have characteristic morphologic features, including the presence of abundant cytoplasm containing a variable number of azurophilic granules and relatively small nuclei with inconspicuous nucleoli. In GLL the expansion of large granular lymphocytes (LGLs) often occurs in individuals with autoimmune disorders and is associated with variably severe neutropenia, anemia, and thrombocytopenia, which are responsible for most of the disease-associated morbidity and mortality. Most GLL cases can be subclassified into cytolytic T-cell type for the cases in which the neoplastic cells express CD3 and exhibit clonal T-cell antigen receptor gene rearrangements. 1,[6][7][8][9] This disorder is now referred to as T-cell granular lymphocytic leukemia in the current World Health Organization classification of diseases of the hematopoietic and lymphoid tissues. 10,11 The individual neoplastic cells in GLL have few, if any, cytologic features that distinguish them from benign granular lymphocytes. [12][13][14] This attribute makes a morphologic distinction between GLL and conditions associated with a reactive, polyclonal increase in granular lymphocytes almost impossible in ...

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Section: Bone Marrow In T-cellmentioning

confidence: 86%

Distinct bone marrow findings in T-cell granular lymphocytic leukemia revealed by paraffin section immunoperoxidase stains for CD8, TIA-1, and granzyme B

Morice

Kurtin

Tefferi

et al. 2002

Blood

134

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“…Initially this disease entity was described as chronic lymphocytic leukemia of T cell origin (T-CLL), 1 T␥-lymphocytosis, 2 or large granular lymphocyte (LGL) leukemia. 3,4 Chronic T cell proliferative disease is relatively rare and has a heterogeneous clinical presentation, but with common immunological and morphological characteristics, which should be discriminated from other mature T cell malignancies such as T cell prolymphocytic leukemia, adult T cell leukemia-lymphoma, and Séz-ary syndrome.…”

Section: Introductionmentioning

confidence: 99%

Induction of clinical remission in T-large granular lymphocyte leukemia with cyclosporin A, monitored by use of immunophenotyping with Vβ antibodies

et al. 1998

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“…This classification has im portant clinical implications since patients from the second group usually present with an aggressive clinical course [2,10,13]. On the other hand, cases from the first group usually have an extremely chronic and rather benign disease [1,3,6,10,13] whose recognition as a true neoplasia has been questioned and, for these patients, the noncommitted term of abnormal ex pansion of GL has been proposed [12].…”

Section: Introductionmentioning

confidence: 99%

Unusual Phenotype (Leu 7+, OKT4+, OKMl+) Expressed by Cells from a Patient with an Abnormal Expansion of Granular Lymphocytes

Quinti¹,

Pacilli²,

Zoli³

et al. 1984

Acta Haematol

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We report the case of a 70-year-old female with a lymphocytosis which was casually detected during a routine examination. Immunological studies revealed the expansion of granular lymphocytes (GL) with the following, previously undescribed phenotype: Leu 7⁺, OKT3⁺ , OKT4⁺, OKT8^-, OKM1⁺ . These cells were tested for their functional activities and found to exert neither helper nor suppressor functions in in vitro tests. Cytotoxic activities demonstrated a strong ADCC and a markedly reduced NK function. 1 year later the clinical course has remained good without any treatment and we suggest that this case should be classified as an abnormal expansion of GL, despite the OKT4 positivity of the cells. Our data point out the importance of a careful immunological study of cells from these rare patients and suggest the existence of a normal GL population expressing the OKT4 phenotype, which is possibly expanded in this patient.

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Proliferation of Tγ Cells with Killer-Cell Activity in Two Patients with Neutropenia and Recurrent Infections

Cited by 133 publications

References 21 publications

Distinct bone marrow findings in T-cell granular lymphocytic leukemia revealed by paraffin section immunoperoxidase stains for CD8, TIA-1, and granzyme B

Distinct bone marrow findings in T-cell granular lymphocytic leukemia revealed by paraffin section immunoperoxidase stains for CD8, TIA-1, and granzyme B

Induction of clinical remission in T-large granular lymphocyte leukemia with cyclosporin A, monitored by use of immunophenotyping with Vβ antibodies

Unusual Phenotype (Leu 7<sup>+</sup>, OKT4<sup>+</sup>, OKMl<sup>+</sup>) Expressed by Cells from a Patient with an Abnormal Expansion of Granular Lymphocytes

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