scholarly journals Silent T-cell receptor cutaneous T-cell lymphoma associated to a clonal plasma cell proliferation

2019 ◽  
Vol 11 (1) ◽  
Author(s):  
Ana Caballero ◽  
Silvana Novelli ◽  
Anna Mozos ◽  
Pilar Garcia Muret ◽  
Anna Monter ◽  
...  
Keyword(s):  
Cell Proliferation ◽  
T Cell ◽  
Plasma Cell ◽  
Cell Lymphoma ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Gamma Delta ◽  
T Cell Lymphomas ◽  
Dismal Prognosis ◽  
Gamma Delta T Cell

Within T-cell lymphomas (TCL) there are 2 entities expressing gamma-delta TCR: hepatosplenic gamma-delta T-cell lymphoma (HSGDTL) and the primary cutaneous gamma-delta T-cell lymphoma (PCGDTL). PCGDTL is a rare form of Tcell lymphoma with specific tropism for skin that have a dismal prognosis. Although even rarer, there have been reports of TCL with loss of expression of the TCR, which have been termed peripheral TCL TCR-silent type. We report the case of a cutaneous TCR-silent type lymphoma associated to a clonal plasma cell proliferation with an ominous outcome that led to a lot of discussion in its classification. Due to the aggressiveness of the disease and the scant evidence about therapy in this strange entity the outcome was fatal. We report a unique case of a TCR-silent cutaneous TCL with an exceptional histopathology, prolonged clinical evolution and a subsequent plasma cell clonal expansion.

scholarly journals An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs'-Negative Hemolytic Anemia

2015 ◽  
Vol 9 ◽  
pp. CMO.S35120 ◽  
Author(s):  
Feryal A. Ibrahim ◽  
Vignesh Shanmugam ◽  
Aliaa Amer ◽  
Halima El-Omri ◽  
Ahmad Al-Sabbagh ◽  
...  
Keyword(s):  
T Cell ◽  
Hemolytic Anemia ◽  
Cell Lymphoma ◽  
Treatment Options ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Γδt Cell ◽  
T Cell Lymphomas ◽  
Dismal Prognosis ◽  
Hepatosplenic T Cell Lymphoma

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that comprises <5% of peripheral T-cell lymphomas. The majority of cases harbor the γδT-cell receptor (TCR), but recently, a few cases have been shown to express the αß TCR. Comparison of these two subtypes (αβ and γδ) shows similar clinicopathologic and cytogenetic features; however, due to the paucity of reported cases, it is not clear whether they are prognostically distinct entities. We report a case of αβ HSTCL with a rather unusual presentation of Coombs'-negative hemolytic anemia. Diagnosis proved challenging due to an unusual blastoid morphology with the absence of typical intrasinusoidal distribution of tumor cells in the bone marrow. This unique case adds to the growing list of this rare subtype of T-cell lymphomas, which warrant urgent attention due to the lack of effective treatment options and dismal prognosis.

scholarly journals Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta

Blood ◽  
1990 ◽  
Vol 75 (11) ◽  
pp. 2213-2219 ◽  
Author(s):  
JP Farcet ◽  
P Gaulard ◽  
JP Marolleau ◽  
JP Le Couedic ◽  
T Henni ◽  
...  
Keyword(s):  
T Cell ◽  
T Cell Receptor ◽  
Cell Lymphoma ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Malignant Cells ◽  
Gamma Delta ◽  
T Cell Lymphomas ◽  
Morphologic Characteristic ◽  
Delta Gene

Peripheral T-cell lymphomas consist of a clinically heterogeneous group of malignant disorders whose immunophenotype usually corresponds to that of normal mature T cells. We describe and correlate the clinical, histopathologic, phenotypic, and genotypic findings in two patients with malignant lymphoma presenting with hepatosplenic disease. The morphologic pattern of lymphoma was that of a sinusal/sinusoidal infiltration in spleen, marrow, and liver. This morphologic characteristic was associated with the presence of a productive clonal rearrangement of the T-cell receptor (TCR) delta gene. Lymphoma cells expressed a CD3-TCR-gamma delta- phenotype. They were also double negative (ie, CD4-CD8-) and lacked the CD5 and CD7 antigens. In one patient, tumor progression was associated with phenotypic changes that resulted in a CD3-TCR-gamma delta- phenotype with the same delta-gene rearrangement as initially. These observations suggest the existence of a new type of peripheral T-cell lymphoma characterized by its hepatosplenic presentation, and by the sinusal/sinusoidal tropism and the TCR-gamma delta phenotype of the malignant cells.

scholarly journals Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta

Blood ◽  
1990 ◽  
Vol 75 (11) ◽  
pp. 2213-2219 ◽  
Author(s):  
JP Farcet ◽  
P Gaulard ◽  
JP Marolleau ◽  
JP Le Couedic ◽  
T Henni ◽  
...  
Keyword(s):  
T Cell ◽  
T Cell Receptor ◽  
Cell Lymphoma ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Malignant Cells ◽  
Gamma Delta ◽  
T Cell Lymphomas ◽  
Morphologic Characteristic ◽  
Delta Gene

Abstract Peripheral T-cell lymphomas consist of a clinically heterogeneous group of malignant disorders whose immunophenotype usually corresponds to that of normal mature T cells. We describe and correlate the clinical, histopathologic, phenotypic, and genotypic findings in two patients with malignant lymphoma presenting with hepatosplenic disease. The morphologic pattern of lymphoma was that of a sinusal/sinusoidal infiltration in spleen, marrow, and liver. This morphologic characteristic was associated with the presence of a productive clonal rearrangement of the T-cell receptor (TCR) delta gene. Lymphoma cells expressed a CD3-TCR-gamma delta- phenotype. They were also double negative (ie, CD4-CD8-) and lacked the CD5 and CD7 antigens. In one patient, tumor progression was associated with phenotypic changes that resulted in a CD3-TCR-gamma delta- phenotype with the same delta-gene rearrangement as initially. These observations suggest the existence of a new type of peripheral T-cell lymphoma characterized by its hepatosplenic presentation, and by the sinusal/sinusoidal tropism and the TCR-gamma delta phenotype of the malignant cells.

An Unusual Case of a Splenic Gamma/Delta T-cell Lymphoma with Angiocentric Tendency and Haemophagocytic Syndrome

1996 ◽  
Vol 23 (5-6) ◽  
pp. 631-634 ◽  
Author(s):  
August Zabernigg ◽  
Falko Fend ◽  
Josef Thaler ◽  
Claus Gattringer
Keyword(s):  
T Cell ◽  
Unusual Case ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Gamma Delta ◽  
Haemophagocytic Syndrome ◽  
Gamma Delta T Cell

scholarly journals Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin

Blood ◽  
1996 ◽  
Vol 88 (11) ◽  
pp. 4265-4274 ◽  
Author(s):  
CB Cooke ◽  
L Krenacs ◽  
M Stetler-Stevenson ◽  
TC Greiner ◽  
M Raffeld ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Killer Cell ◽  
Clinical Manifestations ◽  
Lymphoid Cells ◽  
T Cell Lymphoma ◽  
Survival Duration ◽  
Adult Males ◽  
Gamma Delta ◽  
Gamma Delta T Cell

We identified eight cases of T-cell lymphoma with evidence of a gamma delta phenotype over a 13-year period. Seven of these cases conformed to a distinct clinicopathologic entity of hepatosplenic gamma delta T- cell lymphoma. Nearly all of these patients were young adult males (five of seven), with a median age at presentation of 20 years. They presented with marked hepatosplenomegaly, without lymphadenopathy or significant peripheral blood lymphocytosis. Thrombocytopenia was seen in all patients, and five of seven were mildly anemic. The clinical course was aggressive, and despite multiagent chemotherapy, the median survival duration was less than 1 year. The morphologic findings were uniform; a monomorphic population of medium-sized lymphoid cells with moderately clumped chromatin and a rim of pale cytoplasm infiltrated the sinusoids of the spleen, liver, and bone marrow. The cells had a characteristic immunophenotype: CD2+, CD3+, CD4-, CD5-, CD7+, CD16+, CD57-, CD25-, T-cell receptor (TCR)delta +, beta F1-. CD8 was positive in four of seven cases tested, and CD56 was positive in five of six. All cases expressed the cytotoxic granule-associated protein, TIA1, but perforin was detected in only one case. All cases with assessable DNA had a TCR gamma gene rearrangement, and lacked Epstein-Barr virus sequences. Isochromosome 7q was identified in two cases with cytogenetic information. The one case of cutaneous gamma delta T-cell lymphoma differed in its clinical manifestations, histologic appearance, and immunophenotype. We conclude that hepatosplenic gamma delta T-cell lymphoma is a distinct clinicopathologic entity derived from cytotoxic gamma delta T cells, and should be distinguished from other lymphomas of T-cell and natural-killer cell (NK)-like T-cell derivation.

scholarly journals Secondary Gamma-Delta T-Cell Lymphoma Not Otherwise Specified (NOS) From Chronic Immunosuppression

Cureus ◽  
2021 ◽  
Author(s):  
Madeleine E Turcotte ◽  
Amar H Kelkar ◽  
Joanna Chaffin ◽  
Nam H Dang
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Gamma Delta ◽  
Not Otherwise Specified ◽  
Gamma Delta T Cell

Hepatosplenic Gamma-Delta T-Cell Lymphoma, a Rare and Challenging Diagnosis

2018 ◽  
Vol 18 ◽  
pp. S288
Author(s):  
Maxime Winant ◽  
Youssef Jounblat ◽  
Aspasia Gerogala ◽  
Marie Vercruyssen ◽  
Philippe Lewalle ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Gamma Delta ◽  
Gamma Delta T Cell
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