ABSTRACT
BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Subject(s)
Adult , Female , Humans , Male , alpha-Thalassemia , Anemia, Hemolytic, Congenital , beta-Thalassemia , Bilirubin , Cell Membrane , Diagnosis , Elliptocytosis, Hereditary , Erythrocyte Indices , Hemoglobinopathies , Internal Medicine , Jaundice , Korea , L-Lactate Dehydrogenase , Oxidoreductases , Pallor , Pathology, Molecular , Pediatrics , Phosphopyruvate Hydratase , Prevalence , Pyruvate Kinase , Reticulocyte Count , Retrospective Studies , Sex Ratio , Surveys and QuestionnairesABSTRACT
Adult T-cell leukemia/lymphoma (ATLL) is an often aggressive and fatal malignancy of mature CD4+ T lymphocytes, characterized by leukemia, lymphadenopathy, hypercalcemia and lytic bone lesions. Diagnosis of ATLL is based cm abnormal T lymphocytes in the peripheral blood and histologically and cytologically proven lymphoid malignancy with T cell surface antigens. Anti-HTLV-I serum antibodies and demonstration of clonality to HTLV-I proviral DNA are included. We report a case of ATLL in a 64-year-old man who had a painful nodule on the right cheek and multiple scattered papules and nodules on the trunk for 2 months. He had multiple cervical and axillary lymph node enlargements and hepatosplenomegaly. On laboratory finding peripheral leukocytes count was 33,400/mm3 with 63% atypical lymphocytes. Clover-shaped lyrnphocytes with multilobulated nuclei were found in peripheral blood and bone marrow. Histologic findings showed dense bandlike infiltrations of atypical lymphocytes in the dermis with angiocentricity. The surface phenotype of these abnormal cells were CD2+, CD3+, CD4+, CD30+ and HLA-DR+ and HTLV-I specific antibodies were found.
Subject(s)
Adult , Humans , Middle Aged , Antibodies , Antigens, Surface , Bone Marrow , Cheek , Dermis , Diagnosis , DNA , Human T-lymphotropic virus 1 , Hypercalcemia , Leukemia , Leukemia-Lymphoma, Adult T-Cell , Leukocytes , Lymph Nodes , Lymphatic Diseases , Lymphocytes , Lymphoma , Phenotype , T-LymphocytesABSTRACT
PURPOSE: Central Cancer Registry Center in Korea conducted a nation-wide hospital-based cancer registry to provide the basic data on cancer statistics. MATERIALS AND METHODS: In 1998, 124 hospitals participated in the cancer registry program. All cancer registry data, submitted from the participating hospitals by diskettes during the year, were reviewed and sorted out by the committee members who were all board-qualified clinical oncologists and pathologists. To avoid duplication, every resident registration numbers were compared by the computer. Cases diagnosed by histologic examination were preferentially chosen. RESULTS: Of 89,226 cases registered, 9,163 (10.3%) duplication cases were excluded. Of the remaining 80,063 cases, 3,195 cases (4.0%) of carcinoma in situ (morphology code /2) were excluded. Finally 76,868 cases were analyzed. Of the analyzed cases, 44,037 (57.3%) were male and 32,831 (42.7%) were female. The leading age groups in the order of relative frequency were 60~64 years of age (15.3%), followed by 55~59 (14.4%). The leading primary cancer sites in the order of relative frequency were stomach (20.9%), followed by liver and intrahepatic bile ducts (12.2%), bronchus and lung (11.9%), colorectum (9.6%), breast (6.1%). In male, the leading primary cancer sites were stomach (24.4%), followed by liver and intrahepatic bile ducts (16.4%), bronchus and lung(16.0%), colorectum(9.2%) and urinary bladder (3.5%). In female, stomach (16.3%) was the most common site, followed by breast (14.1%), uterine cervix (13.0%), colorectum (10.1%) and liver and intrahepatic bile ducts (6.5%). Among the 1,190 cases of childhood malignancies, leukemia (33.4%), CNS tumor (15.7%) and sympathetic nervous system tumor (8.4%) were common. CONCLUSION: We analyzed and reported the registered cancer data from 124 hospitals during 1998.
Subject(s)
Female , Humans , Male , Bile Ducts, Intrahepatic , Breast , Bronchi , Carcinoma in Situ , Cervix Uteri , Committee Membership , Korea , Leukemia , Liver , Lung , Stomach , Sympathetic Nervous System , Urinary BladderABSTRACT
Sarcomatoid carcinomas are rare neoplasms that have been described using various terms, pseudosarcoma, carcinosarcoma or spindle cell carcinoma. Sarcomatoid carcinomas are epithelial tumors with sarcomatoid components and characterized by the presence of cytokeratin in pleomorphic spindle cells. These tumors of gallbladder rarely have been reported predominantly in older women with associated gallstones and their prognosis was very poor. However, the clinical and histological data are very limited. We report a case of sarcomatoid carcinoma in gallbladder confirmed by light microscopy and immunohistochemistry.
Subject(s)
Female , Humans , Carcinosarcoma , Gallbladder , Gallstones , Immunohistochemistry , Keratins , Microscopy , PrognosisABSTRACT
PURPOSE: To investigate whether non-Hodgkin's lymphoma of Korea is pathogenetically associated with Epstein-Barr virus (EBV). MATERIALS AND METHODS: We analyzed fifty nine paraffin-embedded tissue and 22 fresh frozen tissue samples from non-Hodgkin's lymphoma patients for the presence of EBV sequences by polymerase chain reactions (PCR), in situ hybridization (ISH) and assessed the clonality of EBV infected cells by Southern blot hybridization. RESULT: On ISH using oligonucleotide probes corresponding to EBV-encoded small RNAs (EBERs), 17 (28.8%) of 59 paraffin-embedded tissue samples showed positive hybridization signals localized over the nuclei of the tumor cells, but PCR using primers from Internal Repeat I or EBV-determined nuclear antigen 1 gene showed positive results in only 6 (10.2%) and 5 (8.5%) samples, respectively. ISH and PCR did not detect EBV sequences in 15 paraffin-embedded tissue samples of tuberculous lymphadenitis patients. In 22 fresh frozen tissue samples, PCR detected EBV sequences in three samples from peripheral T cell lymphoma (PTCL). In two of those three samples, Southern blot analysis showed that these viral DNAs were monoclonal and of latent form. CONCLUSION: Approximately 28.8% of non-Hodgkin's lymphoma were related to EBV in Korea. Monoclonality of those EBV DNAs implies that virus infection preceded malignant transformation, suggesting that EBV may play a role in lymphomagenesis.
Subject(s)
Humans , Blotting, Southern , DNA , DNA, Viral , Herpesvirus 4, Human , In Situ Hybridization , Korea , Lymphoma, Non-Hodgkin , Lymphoma, T-Cell, Peripheral , Oligonucleotide Probes , Polymerase Chain Reaction , RNA , Tuberculosis, Lymph NodeABSTRACT
No abstract available.
Subject(s)
Carcinoma, Non-Small-Cell Lung , Cisplatin , Drug Therapy , Etoposide , Prospective StudiesABSTRACT
No abstract available.
Subject(s)
Humans , Colorectal Neoplasms , Fluorouracil , Interferon-gammaABSTRACT
No abstract available.
Subject(s)
Cyclophosphamide , Doxorubicin , Drug Therapy , Sarcoma , VinblastineABSTRACT
No abstract available.
Subject(s)
Humans , Drug Therapy , Drug Therapy, Combination , Fluorouracil , Stomach NeoplasmsABSTRACT
No abstract available.