ABSTRACT
Functional gastrointestinal disorders (FGIDs) are common in children and adolescents, frequently resulting in extensive testing, school absenteeism, disability, and poor quality of life.1-3 FGIDs result from a complex interplay between genetic predisposition, biological triggers, and psychosocial triggers, and are best explained by the biopsychosocial model.1 Although this implies the necessity of multidisciplinary treatment, studies showing the efficacy of such an intervention are lacking. We describe the outcome of children with severe FGIDs treated in a multidisciplinary program.
Subject(s)
Abdominal Pain/etiology , Abdominal Pain/therapy , Combined Modality Therapy/methods , Gastrointestinal Diseases/complications , Gastrointestinal Diseases/therapy , Adolescent , Child , Child, Preschool , Female , Humans , Male , Retrospective Studies , Treatment OutcomeABSTRACT
The objective of our study was to investigate the clinicopathological features of the currently ill-defined subtype of primary cutaneous T-cell lymphoma of unspecified type (CTCLU) with a cytotoxic phenotype and no Epstein-Barr virus (EBV) association. A series of 27 patients with CTCLU (median age 49 years; range 25-87 years; 18 men) was reviewed. Performance status scores above 1 (7%), clinical stages above 2 (15%), B symptoms (26%), extracutaneous involvement (30%), and a fatal course within 1 year of diagnosis (19%) were observed infrequently. The International Prognostic Index was high or high to intermediate in 11%, and the Prognostic Index for Peripheral T-cell Lymphoma unspecified was above group 2 in 22%. Notably, the rates of spontaneous regression and T-cell receptor gene rearrangements by polymerase chain reaction analysis were seen in 26 and 17% of our cases, respectively. Histologically, 22 patients had subcutaneous involvement of whom eight showed a lethal clinical course, and five patients without subcutaneous involvement were all survivors. Immunophenotypical and morphological features allowed us to subclassify our cases according to the following four categories: (1) epidermotropic CD8+ T-cell lymphoma (n=5); (2) cutaneous gamma/delta T-cell lymphoma (n=8); (3) cutaneous alpha/beta pleomorphic T-cell lymphoma (n=8); and (4) cutaneous medium/large pleomorphic T-cell lymphoma, not otherwise specified (n=6). All four of these groups of lymphomas exhibited a relatively favorable clinical course compared to previous reports. However, epidermotropic CD8+ T-cell lymphoma appeared to be unique with a higher ratio (80%) of spontaneous regression, a lower ratio (40%) of subcutaneous involvement, and a more favorable clinical course than the other three subcategories.
