BACKGROUND: There is a long-standing controversy whether melanocytes in vitiligo of more than 1 year duration are actually lost or still present. Resolving this matter is essential in understanding the underlying pathology and for the development of the treatment. On previous immunohistochemical and ultrastructural studies of vitiligo lesions, damage of melanocyte and keratinocyte in early lesions were reported and complete absence of melanocyte in long standing lesions were known. OBJECTIVE: This study aimed to determine the existence of the differences in pathologic changes in melanocytes according to the duration of the lesion. METHODS: We investigated the vitiliginous skin samples from 31 patients with early(less than 1 year duration) vitiligo and 30 patients with long standing(l to 5 years duration) vitiligo under the electron microscopy. RESULTS: Multiple degenerative changes in melanocytes were observed in the early and long standing lesions. In long standing lesions, degeneration of melanocytes including pyknotic, in-dented nuclei, vacuolated cytoplasms and blunted dendrites were more pronounced than early lesions. Even in long standing lesions, definite or presumptive melanocytes were observed in 16(53.3%) of 30 cases. CONCLUSION: Our results suggest that the melanocytes of vitiligo lesions were damaged and that the percentage of degenerative changes increase in accordance with the duration of the lesion. However, in long standing lesions as well as in early lesions, some residual melanocytes can be observed ultrastructurally.
Cytoplasm ; Dendrites ; Humans ; Keratinocytes ; Melanocytes ; Microscopy, Electron ; Pathology ; Skin ; Vitiligo*

Fractures of the zygoma are rarely encountered in pediatric patients. This report presents a case of a 3-year-old child who presented with a vertically split fracture of the marginal tubercle of the zygoma. The marginal tubercle, a bony portion present on the posterior border of the frontal process, assists in attaching the temporalis fascia. This patient was treated surgically with bony fixation using tissue glue. To the best of our knowledge, no cases of fracture of the marginal tubercle of the zygoma have been reported in the literature. Fractures of the marginal tubercle of the zygoma in pediatric patients may be overlooked because of their anatomic location and the musculoskeletal characteristics of these patients. Here, we discuss the clinical features of marginal tubercle fractures of the zygoma.

Malignant hyperthermia is a metabolic and genetic disease which present with multiple signs of variable intensity and time course. Most of signs are nonspecific to malignant hyperthermia an4 it is not unusual for malignant hyperthermia-susceptible patients to under- go their first anesthesia uneventfully. Thus,the accurate prediction of preanesthetic susceptibility and early diagnosis of malignant hyperthermis can be lifesaving. Recently, some episodes of signs and symptoms suggestive of malignant hyperthemia after spinal an- esthesia those were suspected to be malignant hyperthermia have been reported. In our hospital, two patients suffered from signs and symptoms suggestive of malignant hyperthermia after spinal anesthesia. One patient showed severe muscle rigidity on the un- blocked upper extremities and thorax, tachycardia (120-160beat/min) and hypertension (180-160/120-100mmHg) and later he showed high fever(38-40degrees C), generalized seizure and severe acidosis. He expired the next day of operation in spite of intensive care. The other patient showed shivering, high fever(39degrees C) and elevated serum CPK level(more than 1,500IU/ L) after spinal anesthesia. Intensive treatment with cooling was immediately initiated. Then, he recovered completely 6 hours later. His mother and a brother showed increased serum CPK level(91,112IU/L, respectively), too. Although we could not performed confirmatory diagnostic test, signs and symptoms were very similar to those of malignant hyperthermia. So, we suspected that it might be malignant hyperthermia.
Acidosis ; Anesthesia ; Anesthesia, Spinal* ; Diagnostic Tests, Routine ; Early Diagnosis ; Humans ; Hypertension ; Critical Care ; Malignant Hyperthermia* ; Mothers ; Muscle Rigidity ; Seizures ; Shivering ; Siblings ; Tachycardia ; Thorax ; Upper Extremity

BACKGROUND: The ability of non-myeloablative allogeneic stem cell transplants to eradicate host neoplastic cells is based on the accumulating evidence of a graft-versus-malignancy (GVM) effect. Stable mixed chimerism (MC) is associated to the lower risk for the development of graft-versus-host diseases (GVHD), but this possibly occurs at the expense of the GVM effect. Therefore, assessment of the chimerism status is critical to allow immune intervention to maintain a state of donor-host tolerance and to prevent loss of the graft. METHODS: Serial post-transplant peripheral blood samples were collected from 17 patients with various malignant diseases following non-myeloablative allogeneic stem cell transplantation. DNA was amplified from the T-cells, and the polymerase chain reaction (PCR) products were quantified by an automated fluorescent DNA analyzer. RESULTS: All 17 patients showed T-cell MC at post-transplant, but this varied in degree and duration, and then 3 patterns emerged. Group 1: 5 patients experienced a short interval of T-cell MC prior to conversion to complete donor chimerism (CC) (median: 25 days). Group 2: 5 patients showed a rapid increase of host cells after a brief MC at a median of 21 days. They never achieved CC, and they relapsed or showed progressive diseases. Group 3: 7 patients showed persistent T-cell MC for 40-50 days, and they subsequently gradually converted to CC after a median of 112 days. CONCLUSION: All the patients achieved T-cell MC in post-transplant, but the CC development differed in frequency and speed. GVHD preceded the onset of T-cell CC in the majority of the patients. Serial engraftment monitoring of the T-cell chimerism status during the first 100 days after non-myeloablative stem cell transplantation is important in aiding the clinical management of such patients.
Chimerism* ; DNA ; Graft vs Host Disease ; Humans ; Polymerase Chain Reaction* ; Stem Cell Transplantation* ; Stem Cells* ; T-Lymphocytes* ; Tissue Donors ; Transplants

PURPOSE: This study was designed to assess the CT findings of a juxtapapillary duodenal diverticulum (JPDD) and to determine if there is an association between a JPDD and biliary disease using MDCT multiplanar reformation (MPR). In addition, a study was performed to determine if MPR is more useful than an axial image only for an analysis of duodenal diverticula. MATERIALS AND METHODS: A total of 49 patients who had JPDD as identified on an MDCT image were retrospectively included in this study. Patients were divided into two groups: patients with biliary disease (Group 1) and patents without biliary disease (Group 2). A total of 23 patients (46.9%) had biliary disease. We analyzed the size, location, content of the diverticulum, compression of the biliary duct by a diverticulum and the site of a duodenal papilla with an axial image only and MPR images. RESULTS: The frequency of biliary disease was increased when the papilla was located inside a diverticulum (p = 0.033). The use of an MPR image was more useful than an axial image alone for the evaluation of a site of a duodenal papilla. CONCLUSION: A JPDD is associated with the development of biliary disease and the risk of biliary disease is increased when the papilla is located in a diverticulum. An MPR image can provide more precise information about the site of a papilla than an axial image for the evaluation of a JPDD.
Bile Duct Diseases ; Diverticulum ; Duodenal Diseases ; Humans ; Retrospective Studies

BACKGROUND: Sequential monitoring of chimerism after hematopoietic stem cell transplantation is useful to document engraftment and predict graft failure or relapse. The introduction of highly sensitive methods such as in situ hybridization or PCR techniques allows the detection of mixed chimerism more frequently than do previous analyses using cytogenetics and Southern blotting. We studied the feasibility of a new commercial PCR assay with multiplex amplification of 16 short tandem repeat (STR) loci and fluorescent quantitation. METHODS: Serial post-transplant peripheral blood samples were collected from 22 patients follow-ing allogeneic bone marrow transplantations including eight AML, seven CML, two MDS transformed to AML, two NHL, and one case each of ALL, CLL, and SAA. DNA was amplified using a PowerPlex 16 kit and PCR products were quantified by an automated fluorescent DNA analyzer. RESULTS: There were 5-12 (average, 9.5) informative loci in the 16 patients who had received trans-plants from matched related donors compared to 11-14 (average, 12.2) informative loci in the six patients who had received unrelated donor transplants. Most patients achieved a complete donor chimerism (CC) rapidly around the day 30, which was sustained during follow-up. One patient each of AML, CML, MDS, and ALL who had CC at the first chimerism analysis showed an increase in recipient cells thereafter mostly preceding a morphological relapse. The CLL and one of the NHL patients never achieved CC and the most recent STR analysis showed persistent recipient cells. CONCLUSIONS: This method can provide a rapid, semi-quantitative assessment of chimerism in post-transplant patients. In cases lacking disease-specific markers, serial blood chimerism analysis offers an alternative for the potential early recognition of disease relapse when low levels of circulating neo-plastic cells are present.
Blotting, Southern ; Bone Marrow ; Chimerism* ; Cytogenetics ; DNA ; Follow-Up Studies ; Hematologic Neoplasms ; Hematopoietic Stem Cell Transplantation* ; Humans ; In Situ Hybridization ; Microsatellite Repeats* ; Multiplex Polymerase Chain Reaction* ; Polymerase Chain Reaction ; Recurrence ; Tissue Donors ; Transplants ; Unrelated Donors

No abstract available.
Liver Cirrhosis* ; Liver* ; Polycythemia Vera* ; Polycythemia*

BACKGROUND: Adult acute lymphoblastic leukemia (ALL) is a hematologic malignant disease characterized by an uncontrolled proliferation of immature lymphocytes and their progenitors. Because specific chromosomal abnormalities are associated with ALL, cytogenetic studies can help classifying the disease, providing the clues of disease, progression and being used to monitor remission after chemotherapy. So, we have performed cytogenetic studies to identify the incidence of chromosomal abnormalities and their prognostic significance in patients with ALL. METHODS: From August 1996 to July 1999, we evaluated chromosomal abnormalities in 25 patients with acute lymphoblastic leukemia by high resolution banding technique. Among them, 22 patients were treated with vincristine, prednisone, daunorubicin, L-asparagenase (VPDL) to induce complete remission. We divided patients who had hyperdiploidy, normal karyotype into good risk group (A), and t(9;22), undetermined prognostic karyotype group into poor risk group (B). RESULTS: The incidence of chromosomal ab-normalities was 50% (11/22). The median follow up duration of 22 evaluable patients was 11.1 months. The complete remission (CR) rate was 82% (18/22). The CR rate in group A and B were 100% (14/14), 50% (4/8) respectively (P=0.01). The median remission duration was 30.1 months. The median remission duration of group B was 7.3 months, and that of group A was 30.1 months (P=0.0188). Overall median survival duration was 13.7 months, and the median survival duration in A and B group was 32.5 months and 3.5 months respectively (P= 0.0261). CONCLUSION: The incidence of chromosomal abnormalities in patients with acute lymphoblastic leukemia were relatively high and chromosomal abnormalities were one of useful prognostic factors in remission duration and survival duration. Tailored therapy according to chromosomal abnormalities is desired for more effective results.
Adult* ; Chromosome Aberrations* ; Cytogenetics ; Daunorubicin ; Drug Therapy ; Follow-Up Studies ; Humans ; Incidence ; Karyotype ; Lymphocytes ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prednisone ; Vincristine

Imatinib mesylate is a selective Bcr/Abl kinase inhibitor and an effective anticancer agent for Bcr/Abl-positive chronic myelogenous leukemia. Most patients in chronic phase maintain durable responses; however, many in blast crisis fail to respond, or relapse quickly. Mutations within the BCR/ABL kinase domain are the most commonly identified mechanism associated with relapse. To overcome the imatinib resistance in CML, many investigators have tried to clarify molecular mechanism for imatinib resistance in cells of patients who failed to respond to imatinib. Our aim was to invesitigate underlying mechanism for imatinib resistance in SR-1 cells, which were derived from a CML patient in blast crisis. We detected the new mutation of BCR/ABL, resulting in premature termination and loss of BCR/ABL fusion protein expression, which might be possible mechanism for the resistance to imatinib in SR-1 cells.
Amino Acid Sequence ; Base Sequence ; Cell Line, Tumor ; Drug Resistance, Neoplasm/*genetics ; Fusion Proteins, bcr-abl/chemistry/*genetics ; Humans ; Leukemia, Myeloid/*genetics ; Molecular Sequence Data ; Mutagenesis, Insertional/*genetics ; Nucleotides/genetics ; Piperazines/*pharmacology ; Point Mutation/*genetics ; Pyrimidines/*pharmacology ; Research Support, Non-U.S. Gov't

A pathologic splenic rupture refers to a rupture without trauma. A splenic rupture as the initial manifestation of acute myeloid leukemia is extremely rare. In this study, we described a rare case of acute myeloid leukemia presenting principally as an acute abdomen due to a pathologic splenic rupture in a 35-year old male patient. We can assert that a pathologic splenic rupture in hematologic diseases is a potentially life-threatening complication, which necessitates immediate operative intervention. Any such patient complaining about left upper abdominal tenderness should be closely observed, and further diagnostic investigations (ultrasonograph of the abdomen, abdominal CT scan) should be initiated in order to rule out a splenic rupture. The oncologist should be aware of this rare initial presentation of acute myeloid leukemia (AML) M2, as the condition generally necessitates a prompt splenectomy.
Adult ; Humans ; Leukemia, Myeloid, Acute/*diagnosis/pathology/radiography ; Male ; Splenic Rupture/*diagnosis/pathology/radiography ; Tomography, X-Ray Computed