Zhonghua nei ke za zhi · 2002
[Clinical and pathological features of 5 adult patients with Langerhans cell histiocytosis].
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Bai Yan, Li Longyun, Liu Hongrui, Yan Hongzhen
Аннотация
OBJECTIVE: To improve the identification of Langerhans cell histiocytosis (LCH).
METHODS: 5 adult cases of LCH treated in our hospital since 1990 were studied, the clinical data and pathological findings were analyzed.
RESULTS: The sex ratio of male to female was 3 to 2, the mean age in this series of 5 patients was 36.8 years. All patients presented with multifocal or disseminated form of the disease. The involved organs included lung, skeleton, central nervous system, skin, liver, spleen and lymph node. The radiographic presentation of lung involvement includes diffuse micronodular densities (1 case), interstitial fibrosis with cyst formation (2 cases), limited infiltration in unilateral lung field (1 case) and isolated mass (1 case). Sharply demarcated bony rarefaction in radiogram was found in 3 cases. The presence of specific Langerhans cell in the focal lesion, which was the pathological basis for the definitive diagnosis of LCH, was detected in all patients. Systemic chemotherapy with corticosteroid and anthracycline based regimen was given to 5 patients and it caused retraction of the enlarged liver and spleen in 1 case, amelioration of lymph nodes and skin involvement in 2 cases and diminution of enlarged thyroid gland in 1 case, while it had less benefit in lung and bone involvement or to diabetes insipidus.
CONCLUSIONS: LCH can be observed in young adults or middle-aged subjects. It should be suspected in patients who presented with diabetes insipidus, characteristic bony lesion and pulmonary involvement. The definitive diagnosis should be made pathologically in time. The proper treatment with low-dose radiotherapy and/or systemic chemotherapy should be taken based on the number and the function of systems involved.
METHODS: 5 adult cases of LCH treated in our hospital since 1990 were studied, the clinical data and pathological findings were analyzed.
RESULTS: The sex ratio of male to female was 3 to 2, the mean age in this series of 5 patients was 36.8 years. All patients presented with multifocal or disseminated form of the disease. The involved organs included lung, skeleton, central nervous system, skin, liver, spleen and lymph node. The radiographic presentation of lung involvement includes diffuse micronodular densities (1 case), interstitial fibrosis with cyst formation (2 cases), limited infiltration in unilateral lung field (1 case) and isolated mass (1 case). Sharply demarcated bony rarefaction in radiogram was found in 3 cases. The presence of specific Langerhans cell in the focal lesion, which was the pathological basis for the definitive diagnosis of LCH, was detected in all patients. Systemic chemotherapy with corticosteroid and anthracycline based regimen was given to 5 patients and it caused retraction of the enlarged liver and spleen in 1 case, amelioration of lymph nodes and skin involvement in 2 cases and diminution of enlarged thyroid gland in 1 case, while it had less benefit in lung and bone involvement or to diabetes insipidus.
CONCLUSIONS: LCH can be observed in young adults or middle-aged subjects. It should be suspected in patients who presented with diabetes insipidus, characteristic bony lesion and pulmonary involvement. The definitive diagnosis should be made pathologically in time. The proper treatment with low-dose radiotherapy and/or systemic chemotherapy should be taken based on the number and the function of systems involved.