Division of Hematology/Oncology, Hematology/Oncology Flow Cytometry Laboratory, Children's Hospital of Michigan, Detroit, Michigan.
Published: May 2020
AI Article Synopsis
Article Abstract
Background: Proliferation of large granular lymphocytes (LGL) and T-cell LGL (T-LGL) in peripheral blood along with demonstration of clonality are the hallmarks of a heterogeneous group of disorders, including T-LGL leukemia or T-LGL lymphocytosis. They are often associated with neutropenia and responsive to immunosuppression. The true nature of this entity is not well understood. Some cases are reported as reactive phenomena with very limited experience in pediatric population.
Methods: Hematology/Oncology Flow Cytometry Laboratory database has been reviewed retrospectively. Patients with identifiable distinct CD5-dim T-cell population and positive clonal T-cell receptor rearrangement were included in the analysis. Clinical and laboratory data were then reviewed.
Results: Sixteen cases of children and young adults with increased peripheral blood clonal T-LGL population characterized by dim CD5 expression with wide range of underlying immune dysregulation/stimulation disorders were reviewed. Extended follow up with repeat testing suggested the reactive nature of persistent clonal T-LGL proliferations in this group.
Conclusions: Our observations indicate that clonal T-LGL proliferations in children and young adults are reactive in nature and some can be persistent with an indolent course with unknown consequentiality. Clonal T-LGL cells could be targeting the most prominent immunogenic stressor(s) involved as a control mechanism.
Background: Clonal T cell populations are frequently detected in patients with rheumatic diseases. The relevance of this finding is often uncertain, as the clinical spectrum can range from being asymptomatic to T cell leukemia. Former studies suggested that certain anti-rheumatic drugs might influence the course of the clonal T cell populations.
- Large Granular Lymphocytic (LGL) leukemia is a rare type of cancer that often occurs alongside Rheumatoid Arthritis (RA) and is characterized primarily by low neutrophil counts.
- Patients may show symptoms like spleen enlargement, resembling Felty's Syndrome, and while T-LGL leukemia is usually slow-growing, improper treatment can lead to serious health issues, primarily due to frequent infections.
- The text discusses two cases highlighting the potential of Rituximab as an effective treatment for patients suffering from both T-LGL leukemia and RA.
T-cell large granular lymphocytic (T-LGL) leukaemia is often linked to autoimmune disorders, with about one-third of patients also having rheumatoid arthritis (RA).
A notable portion of RA patients display clonal T-cell patterns, indicating a possible connection between the two conditions.
This text discusses a specific case of a patient with RA who was evaluated for low white blood cell count and enlarged spleen, ultimately leading to a diagnosis of T-LGL leukaemia.
T-cell leukemia from large granular lymphocytes (T-LGL leukemia) is a rare type of cancer, with γδT-LGL leukemia making up about 17% of cases and being linked to autoimmune diseases like rheumatoid arthritis (RA).
A study analyzed 15 patients with γδT-LGL leukemia and rheumatologic diseases, finding that most had RA, with notable symptoms such as splenomegaly and neutropenia.
The research highlighted that mutations were common, particularly in certain genes, and showed unusual characteristics of γδT-LGL leukemia, mainly affecting the spleen and not appearing in peripheral blood, prompting the need for further investigation into the relationship between this leukemia and RA.
Felty's syndrome, described in 1924, is characterized by a combination of rheumatoid arthritis, splenomegaly, and leucopenia, presenting ongoing diagnostic and treatment challenges nearly a century later.
The syndrome poses significant risks due to neutropenia, leading to life-threatening infections, with various suggested mechanisms like anti-neutrophil antibodies and neutrophil consumption complicating understanding.
Current treatment options mainly include methotrexate and rituximab, but evidence for alternatives is limited, highlighting a need for further research and updated treatment approaches.
