Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

Purpose: This multicenter, open-label, phase II trial evaluated the efficacy and safety of bortezomib combined with dexamethasone for the treatment of relapsed/refractory cutaneous T-cell lymphoma (CTCL) in previously treated patients across 14 institutions in South Korea. Materials and Methods: Between September 2017 and July 2020, 29 patients with histologically confirmed CTCL received treatment, consisting of eight 4-week cycles of induction therapy followed by maintenance therapy, contingent upon response, for up to one year. The primary endpoint was the proportion of patients achieving an objective global response. Results: Thirteen of the 29 patients (44.8%) achieved an objective global response, including two complete responses. The median progression-free survival (PFS) was 5.8 months, with responders showing a median PFS of 14.0 months. Treatment-emergent adverse events were generally mild, with a low incidence of peripheral neuropathy and hematologic toxicities. Despite the trend toward shorter PFS in patients with higher mutation burdens, genomic profiling before and after treatment showed no significant emergence of new mutations indicative of disease progression. Conclusion This study supports the use of bortezomib and dexamethasone as a viable and safe treatment option for previously treated CTCL, demonstrating substantial efficacy and manageability in adverse effects. Further research with a larger cohort is suggested to validate these findings and explore the prognostic value of mutation profiles.

Essential thrombocythemia (ET) is a chronic myeloproliferative disease characterized by a markedly elevated platelet count in the peripheral blood due to excessive proliferation of bone marrow megakaryocytes. When the disease affects women during pregnancy, an adverse obstetric outcome is possible: miscarriages, intrauterine growth restriction, preterm delivery, intrauterine fetal death, preeclampsia. Maternal complications, both thrombotic and hemorrhagic, were reported relatively infrequently. Various treatments as acetylsalicylic acid, hydroxyurea, anagrelide, heparin, interferon-alpha and plateletpheresis have been proposed to improve the pregnancy. Our case was a 38 years old multiparous women at 37 weeks of gestation with preeclampsia, intrauterine growth restriction. Under the impression of oligohydramnios and fetal distress, an emergency cesarean section was performed under epidural anesthesia. During cesarean section, sudden cardiac arrest with unknown cause was developed, and successful resuscitation was done. After cesarean section, patient continued to elevate platelet count. So bone marrow aspiration and biopsy were performed and showed essential thrombocythemia. We report a case of essential thrombocythemia diagnosed during pregnancy with brief review of the literature.
Abortion, Spontaneous ; Adult ; Anesthesia, Epidural ; Aspirin ; Biopsy ; Bone Marrow ; Cesarean Section ; Death, Sudden, Cardiac ; Emergencies ; Female ; Fetal Death ; Fetal Distress ; Fetal Growth Retardation ; Heparin ; Humans ; Hydroxyurea ; Interferon-alpha ; Megakaryocytes ; Oligohydramnios ; Platelet Count ; Plateletpheresis ; Pre-Eclampsia ; Pregnancy* ; Resuscitation ; Thrombocythemia, Essential*

OBJECTIVE: To compare an isotonic lumbar extension exercise program utilizing lumbar extension exercise machines with modified combination program of isotonic lumbar extension exercises, including dynamic stabilization exercise, to improve and maintain trunk stability in the patient with microscopic lumbar discectomy. METHOD: We studied 41 male workers who underwent microscopic lumbar discectomy. Group 1 (n=24) was treated with the isotonic lumbar extension exercise program. Group 2 (n=17) was treated with the modified combination program of dynamic lumbar stabilization exercise and isotonic lumbar extension exercise. The categories that were evaluated and measured were trunk stability, isometric peak torque of lumbar extensor, weight distribution rate of both leg and trunk muscle balance, and Oswestry low back pain (LBP) disability index. RESULTS: After 3 months, group 1 revealed higher isometric peak torque, weight distribution rate of both leg and trunk muscle balance compared with that of group 2. At the end of 6 months, group 2 revealed higher isometric peak torque compared with that of group 1. CONCLUSION: We suggested that combined exercise program, that included the dynamic lumbar stabilization exercise and the isotonic lumbar extension exercise, was a valuable treatment for postoperative lumbar rehabilitation.
Diskectomy* ; Exercise ; Humans ; Leg ; Low Back Pain ; Male ; Rehabilitation ; Torque

BACKGROUND: Metastatic cancer of unknown primary site occupies 0.5~10% of all diagnosed cancer patients and includes various tumors with diverse responses to systemic chemotherapy. Adenocarcinoma of unknown primary site (ACUPS), the most common subtype, has no standard treatment, rarely responds to conventional treatment and has a poor survival rate. METHODS: The retrospective study was performed to investigate the clinical characteristics and the treatment outcomes of ACUPS. RESULTS: Eighty-one patients with ACUPS diagnosed at Samsung Medical Center from May 1995 to July 1999 were included. The median age was 58 years (range, 29~77). The common sites of metastases were the lymph node, liver, lung and bone in order. In 49 of 81 patients (60.5%), the dominant tumor location was below the diaphragm. The majority of patients (76 of 81) were initially treated with systemic chemotherapy including cisplatin. Responses were evaluable in 70 of 76. Eighteen of 70 patients (25.7%) responded to chemotherapy and complete remission was observed in 6 patients. The overall median survival of 81 patients was 5.6 months. The median survival of the responding patients was 18.3 months but the median survival of the nonresponding patients was 4.6 months (p<0.01). In univariate and multivariate analysis, age, performance status and response to initial chemotherapy were significant prognostic factors for overall survival. CONCLUSION: We observed poor response to the treatment and survival rate in ACUPS, but complete remission and long-term survival were observed in a small number of patients.
Adenocarcinoma/*drug therapy/*secondary ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols/*therapeutic use ; Female ; Human ; Male ; Middle Aged ; Neoplasms, Unknown Primary/*drug therapy/*pathology ; Retrospective Studies ; Survival Analysis ; Treatment Outcome

BACKGROUND: Mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach has recently been defined as a distinct clinicopathologic entity, often associated with Helicobacter pylori (H. pylori) infection. Characteristics and treatment outcomes of 57 patients with gastric MALT lymphoma were analyzed. METHODS: Retrospective analysis of 57 cases of gastric MALT lymphoma who underwent treatment with various modalities at Samsung Medical Center from Mar. 1995 to Jul. 2000 was performed. RESULTS: The median age of the patients was 47 years (ranged from 22 to 75 years) and the ratio of males to females was 1.1:1. The presenting symptoms were abdominal pain, indigestion and GI bleeding. By Modified Ann Arbor system, stage IE accounted for 70.2%, stage II1E 14.0%, stage II2E 14.0%, and stage IV 1.8%, respectively. H. pylori had been evaluated histologically in 49 cases of which 81.6% was positive. Low grade histology accounted for 71.9% and high grade histology 28.1%. Treatment modalities included H. pylori eradication, surgery, chemotherapy, radiotherapy and their combination therapy. In one case, the patient was observed without treatment. Complete remission rate was 98.2%. H. pylori eradication alone resulted in lymphoma regression successfully in 20 out of 23 patients. With median follow-up of 33 months (3-61 months), median survival was not reached. Overall 3 year survival rate was 94.7%. CONCLUSION: Regardless of treatment modality, high survival rate (3 year survival rate 94.7%) was obtained. H. pylori eradication was feasible and safe in the cases of low grade, stage I, and H. pylori-positive lymphoma, and allowed stomach preservation. Longer follow-up evaluation is required to determine the long-term efficacy and side effects of H. pylori eradication.
Abdominal Pain ; Drug Therapy ; Dyspepsia ; Female ; Follow-Up Studies ; Helicobacter pylori ; Hemorrhage ; Humans ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Male ; Radiotherapy ; Retrospective Studies ; Stomach ; Survival Rate

PURPOSE: There has been controversy over an adequate operative method for peptic ulcer perforation, but currently there is general agreement in the surgical literature that perforated duodenal ulcers in patients who constitute excessive surgical risk should be managed by the simplest possible procedure and in the absence of surgical risk, definitive operations are advocated. However, an accurate description of the degree of severity of concurrent medical disease and surgical risk factor is not available and the question as to whether the postoperative mortality is influenced by the magnitude of the procedure or determined only by the patient's risk remains unanswered. METHODS: This retrospective study reviewed the case histories of all patients who underwent operations for perforated duodenal ulcer at Gil Medical Center from January 1993 through 1998 and evaluates the influences of prognostic factors, APACHE II, SAPS, age, duration of peritonitis, concurrent major medical disease and ulcer size, and operative procedures on postoperative mortality in high risk and low risk groups. RESULTS: Large APACHE II score (> or =15) and SPSS (> or =10), delayed operation, large ulcer (> or =2 cm), age (> or =60), and major medical illness that severely compromised cardiorespiratory, hepatic, renal, and immunologic function were associated significantly with mortality in patients with a perforated peptic ulcer. CONCLUSION: Age, duration of peritonitis, major medical disease, APACHE II score, and ulcer size should be pre-sumed to be important prognostic factors. Although further study is necessary in a larger number of patients, it appears that operative procedures have no influence on postoperative mortality.
APACHE ; Duodenal Ulcer* ; Humans ; Mortality ; Peptic Ulcer ; Peptic Ulcer Perforation ; Peritonitis ; Retrospective Studies ; Risk Factors ; Surgical Procedures, Operative ; Ulcer

A 11-month-old male with severe combined immune deficiency (SCID) received allogeneic bone marrow transplantation (BMT). He had suffered from recurrent infection and chronic diarrhea. Two older brother died of pneumonia 2 months after birth, but his HLA identical sister was healthy. He had very low number of T lymphocyte and NK cell. Although number of B lymphocyte was normal, level of immunoglobulin was extremely low. First BMT was done when he was 11 months old. Eighteen milliliter of bone marrow was simply infused without conditioning or GVHD prophylaxis. T lymphocyte appeared and fever which persisted despite use of antibiotics disappeared at day 7. Grade II GVHD developed, but was well controlled with corticosteroid. T lymphocyte subpopulation became normal at day 42. But pancytopenia developed and persisted despite use of G-CSF. Second BMT was done 4 months after 1st BMT. The conditioning regimen included busulfan (8 mg/kg) and cyclophosphamide (200 mg/kg), and ATG, cyclosporine and short-course MTX were used for GVHD prophylaxis. He achieved ANC> 500/uL at day 20 and platelet> 20,000/uL at day 29. BM examination on day 45 showed that 100% of marrow cells were donor origin. Acute and chronic GVHD did not develop. Since T lymphocyte was observed on day 21, various immunological parameters were normalized sooner or later. Immunological reconstitution was complete on day 280. Vaccination was given after 1 year of BMT and he is healthy now.
Anti-Bacterial Agents ; Bone Marrow ; Bone Marrow Transplantation ; Busulfan ; Cyclophosphamide ; Cyclosporine ; Diarrhea ; Fever ; Granulocyte Colony-Stimulating Factor ; Humans ; Immunoglobulins ; Infant ; Killer Cells, Natural ; Lymphocyte Subsets ; Lymphocytes ; Male ; Pancytopenia ; Parturition ; Pneumonia ; Siblings ; Tissue Donors ; Vaccination

BACKGROUND: Donor leukocyte infusion (DLI) is an effective therapy for patients who relapse with leukemia after allogeneic bone marrow transplantation (BMT). This is due to the fact that the immune reactivity of infused allogeneic lymphocytes on relapsed leukemia cells plays a major role in the control of leukemia. However, severe graft-versus-host disease (GVHD) and pancytopenia compromise the success of this treatment in a substantial number of patients. METHODS: To evaluate the effect of DLI, we surveyed 6 BMT centers regarding their use of DLI for relapsed leukemia after BMT. Detailed forms were used to gather data regarding the original BMT, relapse, response to DLI, complication and survival. Reports of 11 patients were consequently available for analysis. RESULTS: Five (83.3%) of 6 patients with chronic myeloid leukemia (CML) achieved complete remission (CR) [time-to-CR; 116 (27~180) days after DLI], and currently 4 are alive in CR (49~436 days). Five patients (83.3%) developed GVHD, and 2 developed pancytopenia which was related to DLI. In acute leukemia, all patients received salvage chemotherapy prior to DLI. Only 1 of 3 patients with acute lymphoblastic leukemia (ALL) who had early relapse achieved CR, but durable remission was not yet confirmed (62+ days). Both 2 patients with acute myeloid leukemia (AML) achieved CR, and their CR durations were 242+ and 326 days after DLI, respectively. CONCLUSION: This study demonstrates that DLI can exert considerable effects against myeloid forms of leukemia, especially in CML. Further investigations of separating GVHD from the graft- versus-leukemia effect and finding more effective anti-leukemia approaches on acute leukemiaare necessary to improve the current DLI limitations.
Bone Marrow Transplantation* ; Bone Marrow* ; Drug Therapy ; Graft vs Host Disease ; Humans ; Leukemia* ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Leukocytes* ; Lymphocytes ; Pancytopenia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence ; Tissue Donors*