The American journal of pediatric hematology/oncology · 1993
Acute lymphoid leukemia associated with Maffucci's syndrome.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Rector J T, Gray C L, Sharpe R W, Hall F W, Thomas W, Jones W
Аннотация
PURPOSE: Maffucci's syndrome is a nonhereditary congenital disorder associated with multiple enchondromas, soft tissue hemangiomas, or lymphangiomas. It carries an associated high risk of the development of malignant neoplasms, particularly sarcomatous transformation of an enchondroma, as well as other malignant mesodermal and nonmesodermal neoplasms. Hematopoietic malignancies arising in Maffucci's syndrome are exceedingly rare. We report the case of a 14-year-old girl with Maffucci's syndrome who developed acute lymphoid leukemia.
PATIENTS AND METHODS: The patient presented at 18 months of age with enchondromatosis. Maffucci's syndrome was established at 10 years of age after the appearance of multiple hemangiomas.
RESULTS: At 14 years of age the patient developed fatigue, frequent nosebleeds, easy bruising, and weight loss, with circulating blasts in the peripheral blood. Bone marrow examination showed replacement of marrow spaces with leukemic blasts. Immunohistochemical and flow cytometric findings were consistent with a diagnosis of acute lymphoblastic leukemia with myeloid antigen expression.
CONCLUSIONS: The occurrence of acute leukemia in a patient with Maffucci's syndrome may represent predisposition to yet another malignancy and reflect further expression of a generalized mesodermal dysplasia in these patients. It also emphasizes the need for aggressive surveillance in patients with Maffucci's syndrome.
PATIENTS AND METHODS: The patient presented at 18 months of age with enchondromatosis. Maffucci's syndrome was established at 10 years of age after the appearance of multiple hemangiomas.
RESULTS: At 14 years of age the patient developed fatigue, frequent nosebleeds, easy bruising, and weight loss, with circulating blasts in the peripheral blood. Bone marrow examination showed replacement of marrow spaces with leukemic blasts. Immunohistochemical and flow cytometric findings were consistent with a diagnosis of acute lymphoblastic leukemia with myeloid antigen expression.
CONCLUSIONS: The occurrence of acute leukemia in a patient with Maffucci's syndrome may represent predisposition to yet another malignancy and reflect further expression of a generalized mesodermal dysplasia in these patients. It also emphasizes the need for aggressive surveillance in patients with Maffucci's syndrome.