Cargando…

Mature Type T-Lymphoblastic Leukemia/Lymphoma Presenting With Isolated Central Nervous System Symptomatology in a Patient With Giant Cell Arteritis on Long-Term Steroid Treatment

T-lymphoblastic leukemia/lymphoma (T-ALL/T-LBL) is a malignancy comprised of T-lymphoblasts that can present as one of four clinical subtypes (pro-T, pre-T, cortical T, and mature T). Clinical presentation is typically characterized by leukocytosis with diffuse lymphadenopathy and/or hepatosplenomeg...

Descripción completa

Detalles Bibliográficos
Autores principales: Smith, John Kolton, Zhang, Xinmin, Machnicki, Stephen C., Azhar, Salman, Vojnic, Morana
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elmer Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9990713/
https://www.ncbi.nlm.nih.gov/pubmed/36895291
http://dx.doi.org/10.14740/jh1037
Descripción
Sumario:T-lymphoblastic leukemia/lymphoma (T-ALL/T-LBL) is a malignancy comprised of T-lymphoblasts that can present as one of four clinical subtypes (pro-T, pre-T, cortical T, and mature T). Clinical presentation is typically characterized by leukocytosis with diffuse lymphadenopathy and/or hepatosplenomegaly. Beyond clinical presentation, specific immunophenotypic and cytogenetic classifications are utilized to diagnose mature T-ALL. In later disease stages it can spread to the central nervous system (CNS); however, presentation of mature T-ALL by way of CNS pathology and clinical symptomatology alone is rare. Even more rare is the presence of poor prognostic factors without correlating significant clinical presentation. We present a case of mature T-ALL in an elderly female with isolated CNS symptoms in combination with poor prognostic factors including terminal deoxynucleotidyl transferase (TdT) negativity and a complex karyotype. Our patient lacked the classical symptomatology and laboratory findings of mature T-ALL but deteriorated quickly upon diagnosis due to the aggressive genetic profile of her cancer.