All issues > Volume 32(11); 1989

Original Article

J Korean Pediatr Soc. 1989;32(11):1510-1519. Published online November 30, 1989.

A clinical study on histiocytosis X.

Sang Oh Na¹, Joong Gon Kim¹, Hyo Seop Ahn¹

¹Department of Pediatrics, College of Medicine, Seoul National University, Seoul, Korea

Received: August 30, 1989;  Accepted: August 30, 1989.

Abstract

We made a clinical study on 31 cases of histiocytosis X who had been admitted to the Dept, of Ped. of S.N.U.C.H. from Jan. 1981 to Dec. 1987. Among 31 cases, 28 patients received chemotherapy to include prednisolone, vinblastine, methotrex- ate and cyclophosphamide as induction therapy, and 6-mercaptopurine, methotrexate and cyclophos- phamide as maintenance therapy. The results obtained were as follows. 1) The sex incidence revealed male predominance with the ratio 1.6:1. 2) Among 31 cases, 13 cases were classified as eosinophilic granuloma, 15 cases as Hand-Schtiller- Christian disease and 3 cases as Letterer-Siwe disease. 3) The mean age of symptom onset was 4 10/12 years in all disease group, 6 3/12 years in eosinophilic granuloma, 3 7/12 years in Hand-Schiiller-Christian disease, and 1 10/12 years in Letterer-Siwe disease. 4) The common clinical symptoms and signs at diagnosis were mass, skin lesion, fever, and palpable lymph node. 5) The most frequent clinical impression without invasive study was osteomyelitis. 6) The most common organ involved among 9 organ systems was bone, and the number of organ systems involved were 1 in 13 cases (42%), 2 in 6 cases, 4 in 5 cases, 5 in 2 cases, and 6 and 9 organs in each 1 case. 7) The abnormal hematologic findings (low hemoglobin level and/or low total leukocyte count and/or low platelet count) were found in 11 cases. 8) The abnormal liver function (elevated liver enzyme and/or low serum protein and/or low serum albumin and/or elevated serum bilirubin) was found in 3 cases. 9) The common findings on tissue biopsy were histiocytic proliferation and infiltration, which were found in all 21 cases of bone biopsy. 10) Among 28 patients who recieved chemotherapy, 2 patients expired during induction chemother- apy, 2 patients were lost during induction chemotherapy, 3 patients were lost during maintenance chemotherapy, 3 patients have been undergone maintenance chemotherapy, 15 pateints are alive without evidence of disease, and the recurrence was found in remaining 3 patients. 11. The event free survival rate after initiating chemotherapy at 1 year, 2 years, and 3 years was 88%, 84%, 70% respectively.

Keywords :Histiocytosis X, Chemotherapy, Survival rate