A Clinical Study of Childhood Histiocytosis Syndrome

Lee, Jeong Hyun; Han, Sung Hye; Kim, Heung Sik; Kang, Chin Moo; Jeon, Dong Seok

Abstract

PURPOSE: We surveyed this study to find the factors related to clinical aspects of patients with histiocytosis syndrome.
METHODS
We analyzed the clinical data of thirty patients retrospectively who were diagnosed as histiocytosis syndrome from January 1992 to December 1997 at Keimyung University Dong San Hospital.
RESULTS
There were nine cases of Class I patients, twenty cases of Class II patients and one case of Class III patient. Male patients were eighteen, and female patients were twelve. Mean age at diagnosis was 4 years. The most common clinical manifestation was fever, and others were hepatosplenomegaly, pallor, respiratory symptom, and lymphadenopathy in order. Bone was involved in seven cases out of nine Class I patients. Single organ involvement happened in five cases out of Class I patients, two organ involvement happened in two patients, three or four organ involvement happened in one case of Class I patient respectively. Etiology of Class II were EBV in four patients, bacterial infection in four patients, and the others were candida, mycoplasma, mycobacterium tuberculosis. There were pancytopenia, coagulation defect, abnormal liver function tests on laboratory examinations. Most common histologic finding of Class I was proliferation and infilteration of histiocytes. Hemophagocytosis was common in bone marrow examination of Class II patients. Chemotherapy was undergone for seven patients out of nine Class I patients. Six of them showed complete remission. One of them died during chemotherapy. Thirteen patients out of twenty Class II patients are on complete remission, and five of them died. One Class III patient died during chmotherapy.
CONCLUSION
The survival rate depends on age, Lahey's organ dysfunction score, severity, and sites of involved organ. One year survival rate by Kaplan-Meier method of ClassI and II patients was 87.5% and 72.2% respectively. In this study, Class II patients showed high mortality rate, so early diagnosis and treatment will be important.