Lymphomatoid papulosis is a strange disease; clinically benign, histologically malignant. Clinically, it may simulate pityriasis lichenoides et varioliformis acuta. The diagnosis is based on the typical histopathological features suggestive of malignant lymphoma, due to the presence of polymorphous lymphoid infiltrate consisting of small lymphocytes intermingled with conspicuous large atypical cells. We experienced a case of lymphomastoid papulosis in 35-year-old woman. Initially, her skin lesions developed as erythematous papules on the extremities, gradually spreading centrifugally with a tendency to involute slowly without treatment, leaving brown wrinkled surface and shallow ulceration. These skin lesions tended to become worse in warm weather and better in cold weather. At first visit, multiple erythematous grouped, ulcerated papules and nodules are seen. 18 months after first visit, most skin lesions are regressed except 5 erythematous pinhead sized papules on right leg in spite of no treatrnent. Labcratory examiniations of CBC, VDRL, urinatlysis, blood chemistry and chest X-ray were all within normal limits. Histopathologically there were hygerkeratosis, mild acanthosis, exocytosis in epidermis, and numerous lymphoid cells were infiltrated especially on perivascular and periappendegeal area, and many atypical cells showing hyperchromatic nuclei, kidney-shaped nuclei and mitotic figures in dermis.
Adult ; Chemistry ; Dermis ; Diagnosis ; Epidermis ; Exocytosis ; Extremities ; Female ; Humans ; Leg ; Lymphocytes ; Lymphoma ; Lymphomatoid Papulosis* ; Pityriasis Lichenoides ; Skin ; Thorax ; Ulcer ; Weather

Intradermal gene administration was found to induce a more profound immune response than direct intramusclular gene injection. We performed intradermal vaccination of B10.PL mice with DNA encoding for the V 8.2 region of the T-cell receptors (TCR). Three weeks later, these mice were immunized with rat myelin basic protein (MBP). Daily mean clinical scores and mortality rate were lower in this group compared with controls. The proliferative responses of lymph node cells to rat MBP were slightly less in the vaccination groups than in the control groups (p<0.05). However, we detected no differences between the two groups with regard to the production of MBP-specific IgG, IgG1, & IgG2a antibodies. The levels of cytokine mRNA expression in the vaccination groups were observed higher than in the control groups without antigen-specific stimulation, but all of cytokine expressions between the vaccination and control groups after antigen-specific stimulation were identical. These results demonstrate that intradermal DNA vaccines encoding for TCR might prove to be useful in the control of autoimmune disease.
Animals ; Autoantibodies/blood ; Base Sequence ; Cytokines/genetics ; DNA, Complementary/genetics ; Encephalomyelitis, Autoimmune, Experimental/etiology/immunology/*prevention and control ; Female ; Gene Expression ; *Genes, T-Cell Receptor beta ; In Vitro ; Injections, Intradermal ; Lymphocyte Activation ; Mice ; Myelin Basic Proteins/immunology ; RNA, Messenger/genetics/metabolism ; Rats ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't ; Vaccines, DNA/*administration and dosage/genetics

Contrast enhancement during the dynamic MR imaging is important for the detection and characterization of focal liver lesions. The purpose of this study was to determine whether or not a timing examination with a injection of a 1.0-mL bolus of gadopentetate dimeglumine into the antecubital vein followed by rapid dynamic scanning and measurement of signal intensity of the aorta could help to obtain proper arterial-dominant phase images for the characterization of focal hepatic lesions during subsequent multiphase dynamic MR imaging. The imaging delay to acquisition of the first gadolinium-enhanced image for multiphase dynamic MR imaging was set to equal the time to peak aortic enhancement during the test examination. The first contrast-enhanced images of 80 patients with 160 focal liver lesions (hepatocellular carcinoma, n = 79; cavernous hemangioma, n = 51; metastatic tumor, n = 30) were then retrospectively reviewed. Peak aortic enhancement occurred between 10 and 28 seconds (mean, 16.5 seconds +/- 3.1) after starting the infusion of contrast material in 80 patients during the test-examination. Depending on the findings of intrahepatic vascular enhancement on the full-scale dynamic images, hepatic arterial phase (n = 11, 14%) or sinusoid phase (n = 65, 81%) imaging was obtained during the first gadolinium-enhanced acquisition in 76 (95%) of 80 patients. Three different lesions were well characterized and easily distinguished from each other (p < .0001) on the first-phase images depending on their enhancement pattern. In the majority of patients, timing examination with test-bolus injection was helpful in obtaining qualified images for the characterization of various focal lesions.
Adult ; Aged ; Aged, 80 and over ; Female ; Hepatic Artery/pathology ; Human ; Image Enhancement* ; Liver/pathology* ; Liver Neoplasms/secondary ; Liver Neoplasms/diagnosis ; Magnetic Resonance Imaging ; Male ; Middle Age ; Time Factors

The prognosis for patients with primary central nervous system (CNS) lymphoma (PCNSL) who relapse after the initial response is usually poor. A standard treatment for relapsed PCNSL has not yet been identified because of the heterogeneity of the therapies employed and the lack of large, prospective clinical trials. We describe a 46-year-old relapsed PCNSL patient who was successfully treated with intraventricular applications of rituximab to minimize neurotoxicity, 2 cycles of salvage chemotherapy with etoposide, ifosfamide, and cytarabine (VIA) regimen and high-dose chemotherapy with autologous stem cell rescue. The high-dose chemotherapy consisted of bischloroethylnitrosourea, etoposide, cytarabine, and melphalan (BEAM) regimen. Partial remission was detected after intraventricular rituximab therapy and the patient has been in complete remission without evidence of neurotoxicity for 28 months after high-dose chemotherapy with autologous stem cell rescue. This case indicates a new appropriate treatment guideline in relapsed PCNSL patient after initial intensive chemo-radiotherapy.
Antibodies, Monoclonal/*therapeutic use ; Central Nervous System Neoplasms/*drug therapy/*therapy ; Cytarabine/therapeutic use ; Etoposide/therapeutic use ; Female ; Humans ; Ifosfamide/therapeutic use ; Lymphoma, Non-Hodgkin/*drug therapy/*therapy ; Middle Aged ; Stem Cell Transplantation/*methods

We report an unusual case of acute myelogenous leukemia in a patient who showed an extramedullary relapse in her uterus, without bone marrow recurrence, two years after an allogeneic bone marrow transplant. She complained of irregular vaginal spotting, and magnetic resonance imaging demonstrated a uterine mass. A biopsy revealed a massive infiltration of immature myeloid cells. A variable number of tandem repeats (VNTR) based on an examination of peripheral blood cells showed full donor chimerism. After receiving chemotherapy, her uterine mass had completely resolved. She has remained in complete remission for more than 6 months. This case suggests that physicians should be aware of the possibility of a uterine relapse in female bone marrow transplant recipients with acute myelogenous leukemia.
Adult ; Female ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Human ; Leukemia, Myelocytic, Acute/*pathology/*therapy ; Neoplasm Recurrence, Local ; Sarcoma, Granulocytic/etiology/*pathology ; Uterine Neoplasms/etiology/*pathology

Hepatitis B virus (HBV) reactivation is the frequent complication after cytotoxic chemotherapy in HBsAg-positive non-Hodgkin's lymphoma (NHL) patients. Pre-chemotherapy viral load may be a risk factor and HBeAg-positive status is associated with increased viral load. The aim of this study was to investigate the long-term treatment outcome of lamivudine in preventing HBV reactivation and its associated morbidity according to HBeAg status. Twenty-four adult HBsAg-positive NHL patients were taken 100mg of lamivudine daily before the initiation of chemotherapy. The median duration of lamivudine therapy was 11.5 months (range: 1-54 months) and the median number of chemotherapy cycles was 6 (range: 1-16 cycles). The steroid containing chemotherapy regimens were used in 18 patients (75%), and the anti-CD20 monoclonal antibody containing chemotherapy regimen was used in 6 patients (25%). Four patients received autologous peripheral blood stem cell transplantation without resultant HBV reactivation. Hepatitis related to HBV reactivation was developed in 1 patient among 14 HBeAg-positive patients and no one among 10 HBeAg-negative. One patient developed HBV reactivation after lamivudine withdrawal, and 4 patients developed the YMDD (tyrosine-methionine-aspartate-aspartate) mutation during lamivudine therapy. There were no statistical differences in HBV reactivation rate during chemotherapy according to the HBeAg status. Our results demonstrate that lamivudine should be considered preemptively before the chemotherapy for all HBsAg-positive NHL patients to prevent HBV reactivation, regardless of pre-chemotherapy HBeAg status. Finally, compared with the chronic hepatitis B patients, similar rate of HBV reactivation after lamivudine withdrawal and development of YMDD mutation was observed in NHL patients.

Endoscopic injection sclerotherapy is an effective and relatively safe modality for controlling bleeding esophageal varices. Injection of sclerosant causes acute mural thrombosis with a necroinflammatory response and subsequent sclerosis in the venous system of the distal esophagus. A few cases of mesenteric venous thrombosis with small bowel infarction after sclerotherapy have been reported, and most of which were fatal. The association between mesenteric venous thrombosis and sclerotherapy has been strongly suggested, but this still remains unproved. We report here on a case of mesenteric venous thrombosis with small bowel infarction that developed after endoscopic injection sclerotherapy.
Esophageal and Gastric Varices ; Esophagus ; Hemorrhage ; Infarction ; Sclerosis ; Sclerotherapy ; Thrombosis ; Venous Thrombosis

BACKGROUND AND OBJECTIVES: Congenital long QT syndrome (LQTS) is a genetic disease that brings prolongation of the QT interval on an electrocardiogram and leads to syncope and sudden death by a fatal ventricular arrhythmia. In Korea, there have been studies about the clinical characteristics and treatment of LQTS, but there are no studies for the molecular and biological evaluation of its genetic mutation. SUBJECTS AND METHODS: Six nationwide university hospitals and laboratories segregated DNA from the blood of 10 patients diagnosed with LQTS to analyze the genetic mutation. RESULTS: Nine out of ten individuals were female. Eight showed genetic mutations. Three had an abnormality in the KvLQT1, 6 in the HERG and 2 had abnormalities in both KvLQT1 and HERG. None had abnormalities in KCNE1 and 2 showed no abnormalities in KvLQT1, HERG or KCNE1. CONCLUSION: Congenital LQTS shows various genetic mutations, and this indicates the necessity for further organized study in more individuals for confirmation of the relationship between the results of clinical diagnosis and genetic analysis.
Arrhythmias, Cardiac ; Death, Sudden ; Diagnosis ; DNA ; Electrocardiography ; Female ; Hospitals, University ; Humans ; Korea ; Long QT Syndrome* ; Syncope

BACKGROUND: The changes in the epidemiology of native valve endocarditis have been known in western countries recent years due to the decrease in the inci-dence of rheumatic heart disease, increased longevity of patients with valvular or congenital heart diseases, and the increase in degenerative heart disease due to the in-crease in the average life span of the general popula-tion. In this study, we analyzed and compared the epide-miological and clinical characteristics of patients with na-tive valvular endocarditis fro two different time periods. METHODS: We compared native valve endocarditis patients diagnosed from 1979 - 1984(group I) with those diagnosed from 1991 - 1996(group II). We used modified Duke' s criteria for the diagnosis and statistical analysis was done using SPSS window program. RESULTS: In our study, mean age of the population was higher in group II and significantly larger number of patients were over the age of 50 in group II. Involve-ment of multiple valves with vegetations and peri-valvular abscess were found more frequently in group II. Also, significantly higher percentage of patients from group II underwent surgical treatment. CONCLUSION: The results of this study suggest that the epidemiolocaland clinical characteristics of infective endocarsitis in Korea may change to resemble those in western countries. Further studies regarding this subject are needed.
Abscess ; Diagnosis ; Endocarditis* ; Epidemiology ; Heart Diseases ; Humans ; Korea ; Longevity ; Rheumatic Heart Disease

Background: AML is a heterogeneous disease, and despite intensive therapy, recurrence is still high in AML patients who achieve the criterion for cytomorphologic remission (residual tumor burden [measurable residual disease, MRD]<5%). This study aimed to develop a targeted next-generation sequencing (NGS) panel to detect MRD in AML patients and validate its performance. Methods: We designed an error-corrected, targeted MRD-NGS panel without using physical molecular barcodes, including 24 genes. Fifty-four bone marrow and peripheral blood samples from 23 AML patients were sequenced using the panel. The panel design was validated using reference material, and accuracy was assessed using droplet digital PCR. Results: Dilution tests showed excellent linearity and a strong correlation between expected and observed clonal frequencies (R>0.99). The test reproducibly detected MRD in three dilution series samples, with a sensitivity of 0.25% for single-nucleotide variants. More than half of samples from patients with morphologic remission after one month of chemotherapy had detectable mutations. NGS-MRD positivity for samples collected after one month of chemotherapy tended to be associated with poor overall survival and progression-free survival. Conclusions Our highly sensitive and accurate NGS-MRD panel can be readily used to monitor most AML patients in clinical practice, including patients without gene rearrangement. In addition, this NGS-MRD panel may allow the detection of newly emerging clones during clinical relapse, leading to more reliable prognoses of AML.