Indolent Lymphoproliferative T-Cell Disorders Associated With Gastrointestional Disease: Diagnostic Challenges and Outcomes

Masciopinto, Pierluigi; Bellitti, Emilio; Arcuti, Elena; Battisti, Olga; Cazzato, Gerardo; Perrone, Tommasina; Longo, Maria Chiara; Laddaga, Filomena Emanuela; Maiorano, Eugenio; Musto, Pellegrino; Ingravallo, Giuseppe; Gaudio, Francesco

doi:10.1016/j.clml.2022.04.002

Cited by 2 publications

(1 citation statement)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…LGL leukemia has been associated with other hematological diseases, such as myelodysplastic syndromes (MDS), aplastic anemia (AA), pure red cell aplasia (PRCA), paroxysmal nocturnal hemoglobinuria (PNH), myeloproliferative neoplasm, B-cell neoplasm ( Table 3 ), with a frequency of 3–10% [ 38 , 45 , 46 , 47 , 48 , 49 , 50 ].…”

Section: Hematological Diseases Associated With Lglmentioning

confidence: 99%

Molecular Features and Diagnostic Challenges in Alpha/Beta T-Cell Large Granular Lymphocyte Leukemia

Gaudio

Masciopinto

Bellitti

et al. 2022

IJMS

Self Cite

View full text Add to dashboard Cite

Large granular lymphocyte leukemia is a rare chronic lymphoproliferative disease of cytotoxic lymphocytes. The diagnosis, according to the WHO, is based on a persistent (>6 months) increase in the number of LGL cells in the peripheral blood without an identifiable cause. A further distinction is made between T-LGL and NK-LGL leukemia. The molecular sign of LGL leukemia is the mutation of STAT3 and other genes associated with the JAK/STAT pathway. The most common clinical features are neutropenia, anemia, and thrombocytopenia, and it is often associated with various autoimmune conditions. It usually has an indolent course. Due to the rarity of the disease, no specific treatment has yet been identified. Immunosuppressive therapy is used and may allow for disease control and long-term survival, but not eradication of the leukemic clone. Here, we discuss the clinical presentation, diagnostic challenges, pathophysiology, and different treatment options available for alpha/beta T-LGL leukemia, which is the most common disease (85%), in order to better understand and manage this often misunderstood disease.

show abstract

Section: Hematological Diseases Associated With Lglmentioning

confidence: 99%

Molecular Features and Diagnostic Challenges in Alpha/Beta T-Cell Large Granular Lymphocyte Leukemia

Gaudio

Masciopinto

Bellitti

et al. 2022

IJMS

Self Cite

View full text Add to dashboard Cite

show abstract

Clinical and Histopathological Features of an Italian Monocentric Series of Primary Small Bowel T-Cell Lymphomas

Lucioni

Fraticelli

Santacroce

et al. 2023

Cancers

View full text Add to dashboard Cite

The gastrointestinal (GI) tract is the most common extranodal site of occurrence of non-Hodgkin lymphomas. Most GI lymphomas are of B-cell lineage, while T-cell lymphomas are less frequent. The aim of our retrospective study was to depict the clinical–pathological profile of a series of patients affected by intestinal T-cell lymphomas (ITCL) and possibly define hallmarks of these neoplasms. A total of 28 patients were included: 17 enteropathy-associated T-cell lymphomas (EATL), 5 monomorphic epitheliotropic T-cell lymphomas (MEITL), 3 indolent T-cell lymphoproliferative disorders of the gastrointestinal tract (ITCLDGT), and 3 intestinal T-cell lymphomas not otherwise specified (ITCL-NOS). Celiac disease (CD) was diagnosed in around 70% of cases. Diagnosis of EATL showed a significant correlation with CD30 expression, whereas MEITL with angiotropism and CD56 positivity. ITCLDGT cases showed plasma cells infiltration. Peripheral lymphocytosis, the absence of a previous diagnosis of CD, an advanced Lugano clinical stage, and the histological subtype ITCL-NOS were significantly associated with worse survival at multivariate analysis. Our findings about the epidemiological, clinical, and histopathological features of ITCL were in line with the current knowledge. Reliable prognostic tools for these neoplasms are still lacking but according to our results lymphocytosis, diagnosis of CD, Lugano clinical stage, and histological subtype should be considered for patient stratification.

show abstract

Indolent Lymphoproliferative T-Cell Disorders Associated With Gastrointestional Disease: Diagnostic Challenges and Outcomes

Cited by 2 publications

References 20 publications

Molecular Features and Diagnostic Challenges in Alpha/Beta T-Cell Large Granular Lymphocyte Leukemia

Molecular Features and Diagnostic Challenges in Alpha/Beta T-Cell Large Granular Lymphocyte Leukemia

Clinical and Histopathological Features of an Italian Monocentric Series of Primary Small Bowel T-Cell Lymphomas

Contact Info

Product

Resources

About