Use of Fluorodeoxyglucose Positron Emission Tomography With Computed Tomography for the Evaluation of Autoimmune Lymphoproliferative Syndrome Lymphadenopathy Suggestive of Lymphoma
Colección de datos
Recopilados desde hoy en adelante - ProspectivoEnfermedades Autoinmunes+8
+ Enfermedades hemáticas y linfáticas
+ Enfermedades del sistema inmunitario
Resumen
Fecha de inicio: 6 de agosto de 2012
Fecha en la que se inscribió al primer participante.The Autoimmune Lymphoproliferative Syndrome (ALPS) is an inherited disorder associated with defective lymphocyte apoptosis, which is clinically characterized by prominent nonmalignant lymphadenopathy, hepatosplenomegaly, and overt autoimmune diseases such as hemolytic anemia, autoimmune thrombocytopenia, and neutropenia. Additionally, ALPS patients have a significantly increased risk of developing non-Hodgkin lymphoma (NHL) and Hodgkin lymphoma (HL). The diagnosis of lymphoma is particularly troublesome in ALPS because many ALPS manifestations overlap with clinical features suggestive of lymphoma. Therefore, individuals with ALPS may undergo repeated biopsies during the course of the disease. Fluorodeoxyglucose positron emission tomography combined with computed tomography (FDG-PET/CT) is a noninvasive test that may help us discriminate benign from malignant lymphadenopathy in patients with ALPS. For patients with ALPS and clinical symptoms suggestive of lymphoma, such as a sudden increase in focal lymphadenopathy and/or systemic B symptoms associated with lymphoma, we want to investigate whether FDG-PET/CT is useful in determining a plan of action by assisting in locating the most active lymph node and determining whether a surgical biopsy is warranted. Under this protocol, FDG-PET/CT scans will be done to rule out lymphoma. A lymph node biopsy may be necessary to determine the pathology of the lymph node and will not be done for research purposes alone.
Protocolo
Esta sección proporciona detalles del plan del estudio, incluyendo cómo está diseñado y qué se está evaluando.Elegibilidad
Los investigadores buscan pacientes que cumplan ciertos criterios, conocidos como criterios de elegibilidad: estado general de salud o tratamientos previos.Cualquier sexo
Sexo biológico de los participantes elegibles para inscribirse.De 5 a 99 años
Rango de edades de los participantes que pueden unirse al estudio.Voluntarios sanos no permitidos
Indica si personas sanas, sin la condición que se estudia, pueden participar.Condiciones
Patología
Criterios
INCLUSION CRITERIA: To qualify for enrollment, patients must meet all of the following: Fulfill current criteria for the diagnosis of ALPS, which includes documented chronic nonmalignant lymphadenopathy and/or splenomegaly, and either greater than or equal to 1.5% T-cell receptor alpha/beta+ DNTs in the peripheral blood or confirmed RAS mutation with or without elevated alpha/beta DNTs. Be enrolled in ALPS natural history protocol #93-I-0063. Have 1 or more of the following: Sudden enlargement of at least 1 lymph node or group of lymph nodes over baseline. Systemic symptoms suspicious for lymphoma (i.e., loss of weight, loss of appetite, fatigue, night sweats, fever, and pruritus). A histologically proven diagnosis of lymphoma or other malignancy. Be 5 years of age or older. EXCLUSION CRITERIA: Patients will be excluded if any of the following is present: Concurrent proven infection or inflammatory disease (e.g., sarcoidosis), which itself often shows increased FDG uptake by PET and which could interfere with the interpretation of study results. Hyperglycemia (regardless of etiology) determined by fasting glucose of >200 mg/dL Weight in excess of 400 lb, which will exceed the weight limit for the scanner table. Pregnancy or breast-feeding. For women of childbearing potential, a negative urine or serum pregnancy test is required within 24 hours prior to an FDG-PET/CT scan.