急性淋巴性白血病併發下視丘症候羣之一病例報告

Hypothalamic Hyperphagia, Obesity and Disturbed Behavior in a Patient with Acute Lymphocytic Leukemia

蔡裕銘(Yuh-Ming Tsai) ； 林守田(Shou-Tien Lin)

《Acta Paediatrica Sinica》 22卷3期 (1981/09) Pp. 181-185

摘要

一個7歲的男孩在2歲4個月時，被診斷為急性淋巴性白血病，以vincristine及prednisolone合併使用，誘導治療約一個月，得緩解，爾後採維持治療。2年8個月後再發，經誘導治療又得緩解。於發病後的3年7個月時，病兒食慾突然大增，體重急遞上昇，3個星期內增重7kg，雖然停止使用corticosteroid，但情況未獲改善。再經過1年1個月後，病兒性格方面發生變化，常亂出怪聲，終至整日昏睡，大小便失禁。2個月後，因肺炎死亡。從發脾到死亡期間，末梢血液像及骨髓檢查正常，腦脊髓液正常，眼底檢查正常。發病到死亡，共計4年10個月。

並列摘要

A 2-year-4-month old boy was admitted to the Taipei Municipal ChungHsing Hospital in January 1975, and acute lymphocytic leukemia was diagnosed. The treatment was started with prednisolone (2 mg/kg/day) and vincristine (1 mg/wk) for 5 doses. The patient responded well and one month later the enlarged lymph nodes disappeared and the liver receded to the costal margin. The patient was maintained on 6 MP and MTX alternately. Three months after acquisition of complete hematological remission the patient received prophylactic craniospinal irradiation (2,400 rads).
In September 1977, after a period of 2 years and 8 months remission, the patient was readmitted because of relapse, and the second remission was obtained within one month.
At the end of August 1978, his appetite increased enormously into definite hyperphagia. Excessive weight gain of about 7 kg in S weeks was noted. He became obese and couldn't walk far due to soreness of legs. In September 1979, his behavior became bizzare, alternating from periods of rage, restlessness and unreasonable crying to periods of frozen immobility. Neurological and funduscopic examination were negative. Treatment with prednisolone was stopped, because the side effects of corticosteroid developed.
In November 1979, the patient's condition deteriorated progressively, coma and incontinence of urine and stool were noted. Finally the patient died of pneumonia. Repeated peripheral blood examinations remained essentially normal before suffering from pneumonia. The patient survived 4 years and 10 months from the date of first diagnosis.
The reports concering hypothalamic syndrome in acute childhood leukemia are reviewed. The clinical manifestations, incidence and pathophysiology are discussed.

並列關鍵字

hypothalamic hyperphagia ； disturbed behavior ； obesity ； acute lymphocytic leukemia