A 16-year-old girl was transferred to the department of thoracic and cardiovascular surgery because of a spontaneous pneumothorax with prolonged air leakage. Chest computed tomography demonstrated a cystic lesion measuring 2×3 cm and involving the left upper lobe. Left upper lobectomy was performed via video-assisted thoracoscopic surgery. A pathologic examination of the specimen revealed a mesenchymal cystic hamartoma. Despite the rarity of pulmonary mesenchymal cystic hamartoma, it should be considered a potential cause of pneumothorax for patients with a large pulmonary cyst. Further, surgical resection must be considered because serious complications such as hemothorax, hemoptysis, and malignant transformation have been reported.
Adolescent ; Female ; Hamartoma* ; Hemoptysis ; Hemothorax ; Humans ; Lung* ; Pneumothorax ; Thoracic Surgery, Video-Assisted ; Thorax

A 49-year-old male patient presented at our clinic with back pain due to an insignificant injury that had occurred approximately 7 months earlier. Although the patient had been treated at primary clinics, the pain had gradually become aggravated and characterized by resting and night pain. We initially diagnosed the patient with myofascial pain syndrome and began treatment comprised of trigger point injection (TPI) on the back muscles to control the pain. His symptoms improved after the first treatment (TPI), but he complained of back pain again several days later. At that time he also reported that he had lost 10 kg over the 4 months preceding his initial visit. Plain radiographs of the thoracolumbar spine revealed L1-L5 compression fractures and generalized osteopenia. The patient was then diagnosed with multiple myeloma based on the results of a bone marrow biopsy. This case demonstrates the importance of using comprehensive diagnostic approaches when the patient manifests symptoms that are unresponsive to conventional treatment.
Back Pain ; Biopsy ; Bone Diseases, Metabolic ; Bone Marrow ; Fractures, Compression ; Humans ; Low Back Pain ; Male ; Middle Aged ; Multiple Myeloma ; Muscles ; Myofascial Pain Syndromes ; Spine ; Trigger Points

BACKGROUND: Therapy-related AML (t-AML) occurs as a late complication of chemotherapy administered to treat a prior disorder. Prognostic factors affecting the clinical outcome in t-AML have not yet been clearly defined; therefore, we evaluated these factors in this study. METHODS: Forty-eight patients diagnosed with t-AML within the past 10 years were enrolled, and their chemotherapy regimens categorized into 4 groups: alkylating agents (AK) only, topoisomerase II inhibitors (TI) and AK, TI only, and others. The prognostic factors affecting clinical outcome were evaluated. RESULTS: Five (10.4%), 21 (43.8%), 9 (18.8%), and 13 (27.0%) patients were treated with AK only, AK and TI, TI only, and others, respectively. Patients with an AML M3 phenotype showed significantly longer overall survival (OS; 55.1 vs. 14.3 months, P=0.040) and disease-free survival (DFS; 61.2 vs. 17.5 months, P=0.049) than other phenotypes. In contrast, patients with a complex karyotype showed significantly shorter OS (7.9 vs. 31.3 months, P=0.008) and DFS (9.5 vs. 38.6 months, P=0.046); additionally, patients with chromosome 5 or 7 abnormalities showed significantly shorter OS (9.1 vs. 30.7 months, P=0.011) than other phenotypes. Only the presence of a complex karyotype or AML M3 phenotype retained prognostic impact in a multivariate analysis. CONCLUSION: Only the AML M3 phenotype was identified as having a good prognosis, and this might suggest that it exhibits unique clinical features in t-AML patients. Moreover, our findings indicated that karyotype was the strongest prognostic indicator and predicted a poor prognosis for t-AML patients with a complex karyotype.
Alkylating Agents ; Chromosomes, Human, Pair 5 ; Disease-Free Survival ; Humans ; Karyotype ; Leukemia, Myeloid, Acute ; Phenotype ; Prognosis ; Topoisomerase II Inhibitors

The results of surgery on Klatskin tumors have improved during the era of the combined liver resection. However, some surgeons still have a negative point of view on liver resection because of the locally infiltrative characteristics of cancer and the high mortality and morbidity. We treated 98 patients with a Klatskin tumor between 1990 and 1996. The mean age was 57 year, and the sex ratio (M : F) was 2.2 : 1. Among them, 27 patients (27.6%) received a combined liver resection, 11 patients (11.2%) received a segmental resection of the extrahepatic bile duct, and the other 60 patients (61.2%) had non resective procedures (a bypass operation, an intubational operation, or a percutaneous drainage procedure). The mean survival of the combined liver resection group was 30.0 months-higher than those of the other groups (17.1 months for the segmental-resection group, 14.0 months for the non resective procedures group)(p<0.05). The cumulative 1-, 2-, and 3-year survival rates in patients undergoing a combined liver resection were 95.5%, 83.9%, and 55.9% respectively (significantly higher than the 72.7%, 11.4%, and 0% in the patients undergoing a resection and the 41.1%, 11.4%, and 5.7% in the patients undergoing a non resective procedures). There was no difference in the survival rates between the segmental-resection and non resective-procedure group. In the segmental-resection group, 5 patients had a negative resection margin, and 6 patients had a positive resection margin. The mean survivals were 18.4 and 16.0 months, respectively (p>0.05). The morbidity rate for the combined liver resection was 74%--higher than that for the segmental resection (45%) and for the non resective-procedure group (30%)(p<0.05). There was one operative mortality (1.0%), the patient died due to postoperative sepsis after a U-tube intubational operation. Based on a univariate analysis, significant prognostic factors after liver resection were lymph node involvement, and bilirubin (>5 mg/dl) at admission. A multivariate analysis showed no prognostic significance for either lymph node involvement or bilirubin at admission. These results indicate that segmental resection is not a curable treatment modality and that only aggressive radical surgery, including a hepatectomy, gives any chance of a cure for a Klatskin tumor. We conclude that combined radical liver resection is the treatment of choice for a Klatskin tumor and that accurate preoperative diagnosis and cautious perioperative care decrease the mortality and morbidity of a hepatectomy.
Bile Ducts, Extrahepatic ; Bilirubin ; Diagnosis ; Drainage ; Hepatectomy ; Humans ; Klatskin's Tumor* ; Liver ; Lymph Nodes ; Mortality ; Multivariate Analysis ; Perioperative Care ; Sepsis ; Sex Ratio ; Survival Rate

PURPOSE: Thrombocytopenia, which is common in neonatal intensive care unit (NICU), is most common among extremely low birth weight (ELBW) neonates despite recent improvement in survival of ELBW neonates. However, study of thrombocytopenia in ELBW neonates is rare and has not been studied in Korea. So we sought to determine the incidence, timing, causes, and outcome of thrombocytopenia among ELBW neonates. METHODS: We retrospectively reviewed the medical records of preterm infants with gestational age <32 weeks and birth weight <1,000 g who were admitted immediately after birth to the NICU at Dongguk University Ilsan Hospital between January 2006 and December 2010. Thrombocytopenia was defined as platelet count < or =150x109/L. The infants who had early onset thrombocytopenia comprised the study group, while remainder of infants made up the control group. The relationships between early onset thrombocytopenia and the outcomes were studied. RESULTS: Of the 88, 82 (93.2%) had one or more platelet count < or =150x10(9)/L. Most were detected during the early days of life. Early onset thrombocytopenia is more common among the smallest patients and associated with respiratory distress syndrome (RDS) and intraventricular hemorrhage (IVH), which occurs few days after birth. The mortality rate was higher in study group than control group. Almost half of patients, the cause of the thrombocytopenia went undiagnosed. CONCLUSION: We observed high incidence of thrombocytopenia among ELBW neonates. Early onset thrombocytopenia was associated with RDS, IVH and high mortality. New efforts to improve the outcome of ELBW neonates should focus on this common problem of thrombocytopenia.
Birth Weight ; Gestational Age ; Hemorrhage ; Humans ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Korea ; Medical Records ; Parturition ; Platelet Count ; Prognosis ; Retrospective Studies ; Risk Factors ; Thrombocytopenia

BACKGROUND: We aimed to evaluate the feasibility of using the allele burden of Janus kinase 2 (JAK2) V617F as a criterion for discriminating 3 subtypes of Philadelphia chromosome-negative myeloproliferative neoplasm (Ph-MPN): polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). METHODS: We collected 70 peripheral blood (PB) and 81 bone marrow (BM) samples from patients diagnosed with Ph-MPN. Real-time quantitative PCR (RQ-PCR) and Amplification Refractory Mutation System (ARMS) assays were performed for each sample. We compared the allele burden of JAK2 V617F for each subtype of Ph-MPN and determined the concordance rates of the results between the 2 tests. RESULTS: The JAK2 V617F allele burden differed significantly among the 3 disease categories in both PB (P=0.045) and BM (P=0.011) samples. Subsequent subgroup analysis revealed that the median allele burden of JAK2 V617F for ET (21.71% for PB and 24.95% for BM) was significantly lower than that for PV (56.88% for PB, P=0.047; 72.66% for BM, P=0.003) and PMF (56.16% for PB, P=0.050; 59.04% for BM, P=0.049). Concordance rate between the RQ-PCR and ARMS data was 90.7%. Of the 14 discrepant cases, 12 were RQ-PCR(+)/ARMS(-) and 2 were RQ-PCR(-)/ARMS(+). CONCLUSION: The allele burden of JAK2 V617F was significantly lower for ET than that for PV or PMF in both PB and BM samples. The JAK2 V617F allele burden is a diagnostic tool for differentiating PV or PMF from ET.
Alleles ; Arm ; Bone Marrow ; Diagnosis, Differential ; Discrimination (Psychology) ; Humans ; Janus Kinase 2 ; Myeloproliferative Disorders ; Philadelphia ; Polycythemia Vera ; Polymerase Chain Reaction ; Primary Myelofibrosis ; Real-Time Polymerase Chain Reaction ; Thrombocythemia, Essential

Multiplex reverse transcription polymerase chain reaction (mRT-PCR) has recently emerged as an alternative to cytogenetics. We designed and used simplified mRT-PCR system as a molecular screen for acute leukemia. Fifteen fusion transcripts were included: BCR-ABL1, PML-RARA, ZBTB16-RARA, RUNX1-RUNX1T1, CBFB-MYH11, DEK-NUP214, TCF3-PBX1, ETV6-RUNX1, MLL-AFF1, MLL-MLLT4, MLL-MLLT3, MLL-MLLT10, MLL-ELL, MLL-MLLT1, and MLL-MLLT6. A total of 121 diagnostic acute leukemia specimens were studied, comparing the mRT-PCR system with standard cytogenetics. Fifty-six cases (46.3%) had fusion transcripts revealed by our mRT-PCR assay. The concordance rate between mRT-PCR and cytogenetics was 91.7%. However, false negative results were found in three cases who have inv(16), t(4;11) or t(11;19)(q23;p13.1), respectively. Seven cryptic translocations including ETV6-RUNX1, MLL-MLLT3, MLL-MLLT4, and PML-RARA were detected. This mRT-PCR assay is a useful screening tool in acute leukemia because it provides rapid and reliable detection of clinically important chimeric transcripts. In addition, cryptic translocations provide additional genetic information that could be clinically useful.
Acute Disease ; Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Chromosome Inversion ; Cytogenetics ; Female ; Humans ; Infant ; Leukemia/*diagnosis/genetics/metabolism ; Male ; Middle Aged ; *Multiplex Polymerase Chain Reaction ; Oncogene Proteins, Fusion/*genetics ; Prognosis ; Translocation, Genetic ; Young Adult

The title of page 189 should be corrected.

No abstract available.
Dendritic Cells*