Immunosuppressive therapy based on the use of antilymphocyte globulin (ALG) has become standard therapy for patients with splastic anemia who are not eligible for bone marrow transplantation. In this study, T cell subsets before and after ALG therapy, hematologic responses, complications and prognostic factors were analysed. Eleven (42%) out of twenty-six patients treated with ALG showed response, but two patients showed relapse. Most of the response (9 cases) was noticed within 6 months after the initiation of ALG therapy (median: 3 months). The main complications of ALG therapy were fever (91%), thrombocytopenia (86%), neutropenia (63%), and serum sickness (56%). Four patients were died just ALG therapy because of serum sickness (2 cases), intracranial hemorrhage (1 case), and shock (1 case). Short interval from diagnosis to treatment suggested to show good response (P=0.0575), but it was not significant statistically. Lymphocyte subsets were measured in the blood of 23 patients. Helper T/suppressor T cell ratio (T4/T8 ratio) at the initiation of ALG therapy (day 0) was higher significantly in patients who were responded (P=0.0299). The patients who showed above 1.0 of T4/T8 ratio on day 0 might be speculated good response (P=0.032). More difference of T4/T8 ratio between day 14 after ALG therapy and day 0 might show good response (P=0.0673). Then the actuarial probability of survival at 3 years in patients treated with ALG was 77%. Our data suggest that ALG therapy may be used as an alternative treatment to bone marrow transplantation, and T4/T8 ration of peripheral blood at the initiation of therapy may be used as one of the prognostic factors.
Anemia ; Anemia, Aplastic* ; Antilymphocyte Serum* ; Bone Marrow Transplantation ; Child* ; Diagnosis ; Fever ; Humans ; Intracranial Hemorrhages ; Lymphocyte Subsets ; Neutropenia ; Recurrence ; Serum Sickness ; Shock ; T-Lymphocyte Subsets ; Thrombocytopenia

No abstract available.
Child* ; Humans ; Incidence* ; Leukemia*

No abstract available.
Leukoencephalopathies*

To study the clinical characteristics and treatment results of childhood soft tissue sarcoma, the retrospective study was performed on 67 patients with soft tissue sarcoma, experienced at the Department of Pediatrics, Seoul National University Hospital from January, 1982 to July, 1990. The median age of 67 soft tissue sarcoma patients was 4 years 5 months and age distribution showed that 0-4 year age group was most common (55.2%). The sex ratio of male to female was 1.2:1. There were 3 cancers among relatives of soft tissue sarcoma patients, including one cancer among first-degree relatives. As for pathological classification, rhabdomyosarcoma (67.1%) was the most common childhood soft tissue sarcoma, followed by malignant Schwannoma (8.9%), extraskeletal Ewing's sarcoma (6.0%), infantile fibrosarcoma (4.5%), malignant fibrous histiocytoma (3.0%), malignant hemangiopericytoma (3.0%), and there were 1 case each of angiosarcoma, leiomyosarcoma, synovial sarcoma, malignant mesenchymoma and mesenchymal chondrosarcoma. The median age of 45 rhabdomyosarcoma patients was 3 years 8 months and age distribution showed that 0-4 year age group was most common (64.5%). Twenty three patients were male and 22 were female. The histologic subtype of rhabdomyosarcoma was embryonal type in 38 patients (84.5%), alveolar type in 5 patients (11.1%) and unclassified type in 2 patients (4.4%). As for primary site of soft tissue sarcomas, the most frequent site was the head and neck region (32.8%) including parameningeal region (13.4%) and orbit (6.0%), followed by extremities (20.9%), trunk (19.4%), retroperitoneum and pelvis (11.9%), urogenital region (7.5%), perineum and perianal region (4.5%) and other region (3.0%). As for primary site of 45 rhabdomyosarcoma cases, the most frequent site was also the head and neck region (37.8%). The most common initial symptom of soft tissue sarcoma patients was mass (68.7%). As for Intergroup Rhabdomyosarcoma Study clinical grouping system of 67 soft tissue sarcoma patients, clinical group III (58.2%) was most common, followed by clinical group II(20.9%), IV (14.9%) and I (6.0%). Of 10 cases of clinical group IV with distant metastasis, lung (8 cases) was the most common metastaic region and other metastatic regions were bone, kidney, liver and bone marrow. As for IRS clinical grouping system of 45 rhabdomyosarcoma patients, clinical group III was most common (68.9%). Of 6 cases of clinical group IV, lung (5 cases) was also the most common metastatic region, followed by kidney and liver. From 1982 to 1985, chemotherapy was done with pulse VAC or pulse VAdrC-VAC regimen based on IRS-I and IRS-II. From 1986, patients in clinical group I and II received vincristine and actinomycin-D for 1 year and patients in clinical group III, IV and II with alveolar histologic subtype(unfavorable histologic group) received vincristine, actinomycin-D, adriamycin, cyclophosphamide and cisplatinum based on IRS-III. Radiation therapy was administered to patients in clinical group II, III and IV. Of 67 cases of soft tissue sarcoma, 54 case were eligible for treatment analysis. The 3 year disease free survival (DFS) of all 54 cases was 54.1%, 3 year DFS of clinical group I and II was 83.9%,3 year DFS of clinical group III and IV before 1986 was 35.7% and after 1986 was 48.2%. Of 45 cases of rhabdomyosarcoma, 41 cases were eligible for treatment analysis. The 3 year DFS of all 41 cases was 49.1%,3 year DFS of clinical group I and II was 87.5%,3 year DFS of clinical group III and IV before 1986 was 27.2% and after 1986 was 45.0%. Patients in clinical group I and II who had no gross residual tumor after primary surgical excision had best prognosis with 3 year DFS approximating 90% with only 2 drugs regimen, significantly better than patients in clinical group III and IV with 3 year DFS below 50% even after intensifying chemotherapy since year 1986. This analysis suggests that total surgical removal is very important for improving prognosis and should be undertaken where possible in all patients without distant metastasis. Treatment results also showed that after year 1986 with intensification of chemotherapy, 3 year DFS of clinical group III and IV as well as early toxic deaths increased, and after lowering doses of chemotherapeutic agents of regimen 35 of IRS-III, treatment results improved much. Therfore to improve prognosis of patients with gross residual tumor after surgical excision of biopsy and patients with distant metastasis at diagnosis, intensified multiagent chemcherapeutic regimen with adequate dose modification should be done to lower early toxic deaths.
Age Distribution ; Biopsy ; Bone Marrow ; Chondrosarcoma, Mesenchymal ; Classification ; Cyclophosphamide ; Diagnosis ; Disease-Free Survival ; Doxorubicin ; Drug Therapy ; Extremities ; Female ; Fibrosarcoma ; Head ; Hemangiopericytoma ; Hemangiosarcoma ; Histiocytoma, Malignant Fibrous ; Humans ; Kidney ; Leiomyosarcoma ; Liver ; Lung ; Male ; Mesenchymoma ; Neck ; Neoplasm Metastasis ; Neoplasm, Residual ; Neurilemmoma ; Orbit ; Pediatrics ; Pelvis ; Perineum ; Prognosis ; Retrospective Studies ; Rhabdomyosarcoma ; Sarcoma* ; Sarcoma, Ewing ; Sarcoma, Synovial ; Seoul ; Sex Ratio ; Survival Rate ; Vincristine

No abstract available.
Child* ; Drug Therapy ; Humans

No abstract available.
Precursor Cell Lymphoblastic Leukemia-Lymphoma*

No abstract available.
Child ; Humans ; Kasabach-Merritt Syndrome* ; Kidney Failure, Chronic ; Peritoneal Dialysis, Continuous Ambulatory

No abstract available.
Child ; Humans ; Kasabach-Merritt Syndrome* ; Kidney Failure, Chronic ; Peritoneal Dialysis, Continuous Ambulatory

No abstract available.
Bone Marrow* ; Necrosis* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma*

Leiomyoma is commonly found in the female genital tract, but occurrence in the urinary bladder is very rare with only 235 cases reported in the literature. These tumors have been classified as intravesical (63%), intramural (7%) and extravesical (30%) depending on the direction of the growth. We report a case of intravesical leiomyoma of the urinary bladder in a 36 year-old woman who exhibited dysuria and urinary retention. The gross and microscopical findings of leiomyoma of the bladder are similar to those of the uterus. Immunohistochemical stains for estrogen receptor (ER) and progesterone receptor (PR) revealed diffuse nuclear staining in smooth muscle cells, supporting the hypothesis of hormonal influence in tumorigenesis.
Adult ; Carcinogenesis ; Coloring Agents ; Dysuria ; Estrogens ; Female ; Humans ; Leiomyoma* ; Myocytes, Smooth Muscle ; Receptors, Progesterone ; Urinary Bladder* ; Urinary Retention ; Uterus