ABSTRACT
The median age of presentation for Hodgkin lymphoma (HL) is lower in developing countries with a higher proportion under 5 years of age possibly attributable to the high prevalence of Epstein-Barr virus-driven disease. It is unclear whether the clinical presentation and outcomes of this cohort are different with concern regarding late effects being most pronounced in this age group. We report the outcome of children under 5 years of age enrolled in the InPOG-HL-15-01, the first multicentric collaborative study for newly diagnosed children and adolescents with HL from India. Thirty-five (9%) of the study population was younger than 5 years with a striking male preponderance of 34:1. They were less likely to have bulky disease, mediastinal or splenic involvement. The outcomes appear to be at least as favorable as in the older patient group. Efforts need to be made to evolve treatment strategies that spare this very young cohort from potential late effects.
Subject(s)
Epstein-Barr Virus Infections , Hodgkin Disease , Adolescent , Child , Child, Preschool , Epstein-Barr Virus Infections/complications , Epstein-Barr Virus Infections/diagnosis , Epstein-Barr Virus Infections/epidemiology , Herpesvirus 4, Human , Hodgkin Disease/drug therapy , Hodgkin Disease/therapy , Humans , Male , Mediastinum/pathology , PrevalenceABSTRACT
Mixed phenotypic acute leukemia (MPAL) is a rare clinical entity. MPAL associated with myeloidsarcoma (MS) is still rarer with only three cases mentioned in English literature. MS has been described in myriads of location, most commonly in skin, gums and lymph nodes. Although theoritically possible, it is very rare to find MS involving the thyroid gland. The diagnosis of MS can be elusive, very often masquerades and mislabeled as lymphoma. A high index of clinical suspicion coupled with PET/CT findings along with morphological clues and thorough peripheral blood, and bone marrow evaluation is mandatory for arriving at the definitive diagnosis. We report the case of a 58-year-old female presenting with thyroid swelling that was subsequently diagnosed to be MS of the thyroid with underlying MPAL (mixed myeloid/B-cell) only after 18F-FDG PET/CT, which revealed an unusual abnormal pattern of multifocal intense FDG uptake in the thyroid gland.
