PURPOSE: The prognosis of non-Hodgkins lymphoma (NHL) in elderly patients seems to be poorer than that in patients aged less than 60 years. This may be due to the lower tolerance for combination chemotherapy in the elderly. Aggressive combination chemo-therapy, which is the treatment of choice in intermediate and high grade NHL of adulthood, may be associated with unpredictab1y severe and lethal toxicity and worsened quality of life in the elderly. We investigated the treatment responses, toxicities and prognostic factors of NHL in elderly patients treated with combination chemotherapy. MATERIALS AND METHODS: We treated 116 elderly (>60 yrs) patients with NHL between January 1986 and June 1996 with adriamycin-containing regimens, such as CHOP (cyclo- phosphamide, adriamycin, vincristine, prednisolone), BACOP (bleomycin, adriamycin, cyclophosphamide, vincristine, prednisolone), and mBACOP (methotrexate, bleomycin, adriamycin, cyclophosphamide, vincristine, prednisolone). Patients in this study ranged from 60 to 81 (median 67) years of age. Fifty-five percent of patients were in stage I or II and the rest (45%) were in stage III or IV. The histologic grade was predominantly (91%) of intermediate and high grade type. RESULTS: The treatment responses were complete (CR) in 55% and partial (PR) in 25%. The median durstion of CR was 32 (3-132) months. The CR rate was significantly higher in patients treated with RDI (relative dose intensity) > 75% than that in the patients treated with RDI < 75% (p 0.003), but there was no significant difference in CR rate between treatment regimens (p-0.38). At a median follow up of 48-months (range, 12 to 132 months), the estimated 5-year ovetall survival was 46%. Ann Arbor Stage (I, II vs III, IV), ECOG performance (0-1 vs 2-3), RDI (>75% vs <75%) and the treatment response were important prognostic factors in the univariate analysis, and the treament response (CR vs non-CR) was the only independent prognostic parameter in the multivariate analysis. The most frequent and severe toxicity associated with chemotherapy was infection with or without neutropenia. The rate of severe infection was significantly decreased in the patients supported with G/GM-CSF but not in the dose-reduction group (RDI<75% vs >75%). CONCLUSION: Our data suggests that achievement of the CR after combination chemotherpy is the most important prognostic factor in the elderly patients with NHL. Suboptimal chemotherapy (RDI<75%) reduced the complete remission rate without reducing the likelihood of developing severe toxicities. Optimal chemotherapy with supportive cares involving the use of hematopoietic growth factors may be needed to improve the treatment response and the survival in the elderly patients with aggressive NHL.
Aged ; Bleomycin ; Cyclophosphamide ; Dimethoate ; Doxorubicin ; Drug Therapy ; Drug Therapy, Combination ; Follow-Up Studies ; Hodgkin Disease* ; Humans ; Intercellular Signaling Peptides and Proteins ; Lymphoma, Non-Hodgkin ; Multivariate Analysis ; Neutropenia ; Prognosis* ; Quality of Life ; Vincristine

No abstract available.
Shoulder*

Testicular microlithiasis is a rare cause of testicular enlargement. An 8.5-year-old boy presented with bilateral testicular enlargement accompanied by no other pubertal signs. His bone age was 8.5 years and serum levels of luteinizing hormone and follicle-stimulating hormone after gonadotropin-releasing hormone stimulation were within prepubertal limits. Scrotal ultrasonography showed multiple echogenic microcalcifications that are indicative of microlithiasis in both testes. During 2-year follow-up, he developed clinical manifestations of early puberty between 9.5 and 10.5 years of age. Testicular microlithiasis should be considered when boys show bilateral testicular enlargement without other findings of puberty.
Calculi ; Follicle Stimulating Hormone ; Follow-Up Studies ; Gonadotropin-Releasing Hormone ; Hypertrophy ; Luteinizing Hormone ; Puberty ; Testicular Diseases ; Testis

'Num-chin syndrome', isolated mental neuropathy, is a rare manifestaion of malignant hernatologic diseases, but important sign for early diagnosis and prediction of clinical course and prognosis. Here we report 7 cases of numb-chin syndrome; one in leukemic transformation of malignant lymphoma, one in blastic crisis of chronic myelogenous leukemia and 5 in acute leukemia Two cases of acute leukemia revealed the 'numb-chin sign' in early course of disease before the diagnosis of leukemia and other three in aggravating state of acute leukemia. The therapeutic response and prognosis was poor and 5 cases expired in a few months.
Diagnosis ; Early Diagnosis ; Hematologic Diseases* ; Leukemia ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Lymphoma ; Prognosis

Background: Approximately 8% of male patients presenting with primary mediastinal germ cell tumors (GCTs) have Klinefelter syndrome (KS), while patients diagnosed with retroperitoneal GCTs also exhibit a range of chromosomal abnormalities. The exact mechanism underlying the development of GCTs in Klinefelter syndrome is unknown, but KS frequently goes underdiagnosed as a result of its varied symptoms and a low general awareness of this condition. Thus, the Children’s Oncology Group recommends screening of Klinefelter syndrome in pediatric and adolescent male subjects who present with GCTs. Methods: We retrospectively reviewed the medical records of extragonadal germ cell tumor patients treated at Severance hospital, department of pediatrics or division of pediatric hematology-oncology over the last ten years. Results: A total of 95 patients with extragonadal germ cell tumors were included in this study. Karyotyping was done in eight patients out of 95 patients, three patients with KS and one patient with Down syndrome. Twelve of extragonadal GCT patients presented at mediastinum, with most common histology of mature teratoma, and three patients presented with chromosomal abnormalities, two with KS and one with Down syndrome. A total of nine patients were diagnosed with retroperitoneal GCTs and only one had KS. Conclusion We described the characteristics of 95 cases of extragonadal GCTs. Although the mechanism of extragonadal GCTs in KS is not clear, karyotyping in pediatric and adolescent extragonadal GCT patients could be helpful in figuring out chromosomal abnormalities including KS and their roles in GCT pathophysiology, which can contribute to improve one’s health.

Purpose: Histiocytic and dendritic cell neoplasms are rare hematologic tumors.This study aimed to describe the epidemiologic features of the entire spectrum of histiocytic and dendritic cell neoplasms, including clinicopathological variables and patient outcomes. Materials and Methods: We comprehensively reviewed 274 patients who were diagnosed with histiocytic and dendritic neoplasms at Severance Hospital, Seoul, South Korea between 1995 and 2018. Results: The most common neoplasm was Langerhans cell histiocytosis (LCH), followed by dermal xanthogranuloma. Among non-LCH sarcomas, histiocytic sarcoma (HS) showed a relatively high prevalence, followed by follicular dendritic cell sarcoma (FDCS). Disseminated juvenile xanthogranuloma (DJG), Erdheim-Chester disease (ECD), indeterminate dendritic cell tumor (IDCT), and interdigitating dendritic cell sarcoma (IDCS) rarely occurred. Generally, these tumors presented in childhood, although the non-LCH sarcoma (HS/FDCS/IDCS/IDCT) group of tumors and ECD occurred in late adulthood. Multiorgan involvement and advanced Ann-Arbor stage, as well as recurrence and death of disease, were not uncommon. The non-LCH sarcoma group had the worst overall survival, compared to the DJG, ECD, and LCH groups. Conclusion Our findings indicate that histiocytic and dendritic cell neoplasms exhibit heterogeneous epidemiologic characteristics and that some patients may have unfavorable outcomes, especially those with non-LCH sarcoma.

Cyclophosphamide-induced cardiotoxicity is an uncommon complication especially in patients who have never undergone mediastinal irradiation or cardiotoxic chemotherapy and do not have underlying cardiac diseases. Here, we describe the case of a 19-year-old female with chronic myeloid leukemia. She was previously treated with oral tyrosine kinase inhibitors and developed cardiomyopathy after receiving infusion of 60 mg/kg intravenous cyclophosphamide for two days with a conditioning regimen for allogenic hematopoietic stem cell transplantation. Severe thickening of the left ventricle and reduced ejection fraction without triggering agents were characteristic for cyclophosphamide-induced cardiomyopathy. Her NT-pro BNP and troponin T concentrations surged to >70,000 pg/mL (0=130 pg/mL) and 2,031 pg/mL (0-14 pg/mL), respectively, during the course of the therapy and multiple organ failure seemed imminent evidenced by unresponsive decline in blood pressure. However, with close monitoring and persistent conservative management which consisted of intravenous hydration, continuous hemodialysis, and mechanical ventilation, her condition recovered.
Blood Pressure ; Cardiomyopathies* ; Cardiotoxicity ; Cyclophosphamide* ; Drug Therapy ; Female ; Heart Diseases ; Heart Failure* ; Heart Ventricles ; Heart* ; Hematopoietic Stem Cell Transplantation ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Multiple Organ Failure ; Protein-Tyrosine Kinases ; Renal Dialysis ; Respiration, Artificial ; Troponin T ; Young Adult

Background: Acute complications within 100 days after allogeneic hematopoietic stem cell transplantation (HSCT) can increase immediate mortality as well as the risk of chronic complications and morbidity. A comprehensive review collecting systemic complications following transplantation would be important in pediatric patients. Methods: We report a retrospective study of pediatric patients who underwent allogeneic HSCT during the 11 years (2009-2020), and their acute complications after transplantation within 100 days. A total 227 pediatric patients’ (90 females, 137 males) data were collected. Results: Among the patients, 62.6% (N=142) suffered from acute graft-versus-host disease, and 118 (52.0%) patients had an acute infection. Pulmonary complications occurred in 52 (22.9%) patients followed by hepatic sinusoidal obstruction syndrome in 30 (18.1%) patients. In the study, 19 died within the first 100 days after HSCT (8.4%), and the 5-year overall survival rate of the patients was 65.4%. Conclusion This study widens the understanding of acute toxicities of pediatric HSCT. A significant number of children still have experienced a variety of acute infectious or non-infectious complications after allogeneic HSCT that contribute to morbidity and mortality. Therefore, continuous efforts are needed to reduce them.

OBJECTIVES: Infection and replication of the hepatitis B virus are closely related to the host immunity. Anticancer chemotherapy decreases the immune response of the host, Especially, glucocorticoid can activate the replication of hepatitis B virus directly. It is well known that hepatitis B virus infection and hepatic complications are more common in patients with hematologic malignancies like malignant lymphoma. We studied the incidence of hepatitis B virus infection and hepatic complications following anti-cancer chemotherapy in patients with malignant lymphoma. METHODS: Among 224 cases diagnosed as malignant lymphoma from January 1989 to December 1993 at Yonsei University Medical Center, 77 cases tested for hepatitis B virus serology was studied. RESULTS: 1) Eighteen cases (23%) was HBsAg positive. 2) The results of hepatitis C virus serology in six cases were all negative. 3) Eight (57%) of 14 follow-up cases had hepatic complications, Two patients had fulminant hepatitis, two nonicteric hepatitis and four icteric hepatitis. 4) Interferon-alpha was administered in three cases among the patients with hepatic complications. Loss of HBeAg was observed in one case and loss of HBsAg in another case. CONCLUSION: Serious hepatic complications can be occurred following anticancer chemotherapy in HBsAg-positive patients with malignant lymphoma. Therefore, we recommend that patients being considered as candidates for anticancer chemotherapy should routinely undergo serologic test for Hepatitis B virus. In addition HBsAg-positive patients with anticancer chemotherapy should be regularly monitored for hepatic injury. And with the careful use of steroid and interferon, prolongation of survival might be searched for these patients.
Academic Medical Centers ; Drug Therapy* ; Follow-Up Studies ; Hematologic Neoplasms ; Hepacivirus ; Hepatitis ; Hepatitis B ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens* ; Hepatitis B virus ; Humans ; Incidence ; Interferon-alpha ; Interferons ; Lymphoma* ; Serologic Tests

BACKGROUND: This study was conducted to verify the hypothesis that the suppression of lipid peroxidation with the antioxidant, U-74389G, could improve the survivability of paraquat intoxicated rats. METHODS: First, we obtained the 24-h mortality by using several paraquat dosages and calculated the 24-h LD50 in 24 male Wistar rats(250~350g). To examine the effect of U-74389G, we divided the rats in 4 groups: a control group and U-74389G only group, a paraquat only group, and a paraquat plus U-74389G group(n=10 each). Paraquat, 35mg/kg, was injected intraperitoneally at 0 h. U-74389G, 10mg/kg, was administered intraperitoneally at 0, 12 h or at 1, 12 h in the respective groups. The rats were observed for 24 hours. At 24 h, plasma and lung, liver, and kidney tissues were obtained after sacrificing the surviving rats to determine the degree of lipid peroxidation by using a thiobarbituric acid reactive substances(TBARS) quantitative analysis. RESULTS: The 24-h LD50 of paraquat was calculated as 40mg/kg in our rats. The 24-h mortality was as follows: control group and U-74389G group 0%, paraquat group 30%, and paraquat plus U-74389G group 10%. The TBARS analysis showed no differences between the U-74389G and the control groups. The paraquat group showed significantly increased TBARS levels in the serum and in the kidney and lung tissue compared to the control group(p<0.05). With U-74389G, the increased TBARS levels were significantly decreased in the plasma, kidney, and lung tissues compared to the paraquat group(p<0.05). However, in the liver tissue, there were no significant differences among the groups. CONCLUSION: A 21-aminosteroid antioxidant, U-74389G, improved the survivablity of paraquat-intoxicated rats through the suppression of lipid peroxidation. Our result suggests the possibility of clinical application of this drug as an antidote for paraquat poisoning.
Animals ; Humans ; Kidney ; Lethal Dose 50 ; Lipid Peroxidation ; Liver ; Lung ; Male ; Mortality ; Paraquat ; Plasma ; Poisoning ; Rats* ; Thiobarbituric Acid Reactive Substances