A Novel Case of Extreme Thrombocytosis in Acute Myeloid Leukemia Associated With Isochromosome 17q and Copy Neutral Loss of Heterozygosity

No abstract available.

Waldenstrom's macroglobulinemia associated with eosinophilia

No abstract available.

Waldenstrom's macroglobulinemia associated with eosinophilia

No abstract available.

Chronic myeloid leukemia with marked splenomegaly and pseudo-Gaucher cells

No abstract available.

A Rare Case of Microgranular Acute Promyelocytic Leukemia Associated with ider(17)(q10)t(15;17) in an Old-age Patient / 대한진단검사의학회지

We present a rare case of microgranular variant acute promyelocytic leukemia (APL) associated with ider(17)(q10)t(15;17)(q22;q12) of an old-age patient. The initial chromosome study showed a 46,XX,del(6)(?q21q25),der(15)t(15;17)(q22;q12),ider(17)(q10)t(15;17)[10]/47,sl,+ider(17)(q10)t(15;17)[3]/46,XX[16]. FISH signals from a dual color dual fusion translocation PML-RARA probe were consistent with the results of conventional cytogenetics. Because of the rarity of ider(17)(q10)t(15;17) in microgranular APL, further studies on both gene dosage effect of this chromosomal abnormality and the influence of ider(17)(q10)t(15;17) on clinical features such as prognosis, survival, and treatment response of APL cases are recommended.

Long-term outcomes of a 5-year follow up of patients with immune thrombocytopenic purpura after splenectomy

BACKGROUND: The long-term outcomes of adult patients with immune thrombocytopenic purpura (ITP) after splenectomy are not clear. METHODS: We retrospectively analyzed 31 patients who underwent splenectomy after diagnosis of ITP at our institution between 1990 and 2009. Long-term follow-up was defined as a follow-up that lasted 1 year or more from splenectomy to the last follow-up. RESULTS: The overall response rate to splenectomy was 84%. However, the response rate at 6 and 12 months decreased to 77% and 68%, respectively. During the 6 years of median follow-up after splenectomy, 11 patients (35%) relapsed. The long-term response rate was 55%. The long-term follow-up of 26 patients after responding to splenectomy showed that the median time from splenectomy to relapse was 19 months in the partial response (PR) group; however, there was no relapse after 9 months in the complete response (CR) group. Variables, including age, were not predictive of the long-term response after splenectomy. Additional treatment in patients who did not respond or relapsed after splenectomy was mostly effective. After a median follow-up of 7 years (range: 1-25 years) from the diagnosis, there were 2 deaths, including one due to spontaneous bleeding after repair of duodenal ulcer perforation. CONCLUSION: Although splenectomy is safe and effective, the response rate after splenectomy continuously decreases over time. The duration of response is different between the patients that achieved CR and those that achieved PR. Factors, including age, were not predictors of a response to splenectomy.

Renal and Gastrointestinal Amyloidosis in Castleman's Disease / 대한신장학회지

Castleman's disease is a rare disorder and thought to occur as a result of chronic antigenic stimulation due to an unknown infectious or inflammatory etiology. It has a heterogenous course: the symptoms persist in some cases for many years and have a progressive fatal course in others. Renal dysfunction in the form of nephrotic syndrome is quite a rare occurrence. Secondary amyloidosis due to Castleman's disease has also been reported in a few case reports. A 46-year-old female who had asymptomatic abdominal lymphadenopathy was diagnosed with Castleman's disease-plasma cell type in our hospital in 2006. Three years after diagnosis, she developed chronic diarrhea, weight loss, anemia and nephrotic range proteinuria. The etiology of symptom was found to be secondary amyloidosis based on renal and gastrointestinal biopsies. She was discharged with steroid therapy. Unfortunately, she had a progressive fatal course. One month after the treatment, she developed thrombocytopenia and died due to cerebral hemorrhage.

Complete Hematologic Response and Cytogenetic Remission after Imatinib and Dexamethasone Treatment of a Ph+ Precursor B-cell Acute Lymphoblastic Leukemia in Renal Transplantation Patient

In this report, we present a case of a patient with Philadelphia chromosome-positive (Ph+) B-cell acute lymphoblastic leukemia after renal transplantation. The patient, a 65-year-old man, had received a kidney transplantation 20 years prior to diagnosis with Ph+ precursor B-cell ALL. Because he was refractory to intensive chemotherapy and had refused to receive additional intensive chemotherapy, he was treated with imatinib and dexamethasone. While this patient experienced a complete hematologic and cytogenetic response, he did not show a complete molecular remission. Eighty days after imatinib combination therapy, the patient relapsed and died from intracerebral hemorrhage.

A Case of Organizing Pneumonia Associated with Rituximab / Journal of the Korean Cancer Association, 대한암학회지

Rituximab is a human/murine chimeric anti-CD20 mono- clonal antibody used to treat CD20-positive B-cell non- Hodgkin's lymphoma (NHL). Although most of the adverse effects associated with rituximab are usually reversible and temporary infusion-related reactions, including fever, chills, flushing and skin reactions, there are several reports of pulmonary events after long-term administration of rituximab. We present a case of asymp-tomatic nodular organizing pneumonia occurring during rituximab-based chemotherapy in a patient with non- Hodgkin's lymphoma.

Effects of the Expression of Leptin and Leptin Receptor (OBR) on the Prognosis of Early-stage Breast Cancers / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Obesity-related leptin and leptin receptor (OBR) have a relation to the development of cancer and metastasis and also the low survival rate for breast cancer patients. Leptin has been associated with increased aromatase activity and it displays functional cross-talk with estrogen. This study was designed to determine the relationship between the expression of leptin and OBR in breast cancer tissue and the prognosis of early-stage breast cancer patients, and especially for the tamoxifen-treated patients. MATERIALS AND METHODS: Ninety-five patients with early-stage breast cancer and who had undergone surgical treatment at Kyung Hee University Hospital between January 1994 and June 2004 were analyzed. The surgical specimens underwent immunohistochemical analysis for leptin and OBR. The patients' survival and clinical characteristics were obtained from the medical records. RESULTS: Of the 95 patients, 79 (83%) and 32 (33.7%) showed the expression of leptin and OBR in breast cancer tissue, respectively. The expression of leptin and OBR in breast cancer tissue was not significantly related to the clinicopathological characteristics, including obesity, the expression of hormonal receptor, the HER-2/neu expression, menopause, stage and the nuclear grade. The expression of leptin and OBR was not significantly related to the overall disease-free survival (DFS). For the tamoxifen-treated postmenopausal obese patients, the DFS of the leptin-positive group was higher than that of the leptin-negative group (p=0.017). CONCLUSION: The expression of leptin and OBR in breast cancer tissue may be not a prognostic factor for disease-free survival of breast cancer patients. In the future, further studies are needed to determine whether leptin expression could be a predictive factor for tamoxifen therapy in the postmenopausal obese subgroup among the early breast cancer patients.

Increased ERCC Expression Correlates with Improved Outcome of Patients Treated with Cisplatin as an Adjuvant Therapy for Curatively Resected Gastric Cancer / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: It has been reported that the overexpression of the excision repair cross-complementing 1 (ERCC1) gene, which is essential for the repair of cisplatin (CDDP)- DNA adducts, negatively influences the effectiveness of CDDP-based therapy for primary gastric cancer. We investigated whether the ERCC1 expression was associated with survival for gastric cancer patients in an adjuvant setting. MATERIALS AND METHODS: We retrospectively analyzed 44 patients who were diagnosed with stage II or higher disease after undergoing curative resection and they had also received cisplatin-based chemotherapy. The ERCC1 expression was examined by performing immunohistochemical (IHC) staining, and this was divided into two groups according to the percentage of IHC staining of the tumor cell nuclei (negative: 10% or less, positive: more than 10%). RESULTS: Among the 44 patients (ERCC1-negative/ERCC1-positive group=16/28), 32 patients were male and their median age was 52 years. There was no difference for the baseline characteristics of the two groups. The median follow-up duration was 41 months. The median disease-free survival (DFS) and the overall survival (OS) for the ERCC1-positive group were significant higher than those of the ERCC1-negative group (DFS: 40.4 vs. 14.6 months, p=0.02, OS: undefined vs. 20.4 months, p=0.008). CONCLUSION: The overall survival in gastric cancer patients who received cisplatin-based adjuvant chemotherapy after a curative resection is higher in those patients showing the overexpression of the ERCC1 gene. However, prospective studies using the ERCC1 gene expression as a prognostic marker for the DNA repair activity are needed.

Ankylosing Spondylitis associated with Plasmacytoma: a Case Report / 대한류마티스학회지

Plasmacytomas are tumors composed of plasma cells of variable maturity, which are histologically identical to those seen in multiple myeloma. Ankylosing spondylitis is a chronic inflammatory disease, probably resulting from the interaction of a genetic predisposition involving HLA-B27 with an environmental event such as enteric bacterial infection. Multiple myeloma has been intermittently reported in patients with ankylosing spondylitis. It has been proposed that the protracted stimulation of immunocytes by inflammatory lesions on the mucosal surfaces of the gastrointestinal, respiratory tracts may be implicated in the pathogenesis of multiple myeloma in some patients. We observed a 23 year old male patient with a history of plasmacytoma who subsequently developed ankylosing spondylitis. He was diagnosed as plasmacytoma 4 years ago and took a radiation therapy. There was no previous report of ankylosing spondylitis following plasmacytoma. The relationship between two diseases is uncertain until now and further study should be needed.

The Efficacy of a Modified Chronomodulated Infusion of Oxaliplatin, 5-Fluorouracil and Leucovorin in Advanced Colorectal Cancer (Preliminary Data) / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: To determine the efficacy and tolerability of a modified chronomodulated infusion of oxaliplatin, 5-fluorouracil (5-FU) and leucovorin in the treatment of advanced colorectal cancer. MATERIALS AND METHODS: Sixteen patients with relapsed or metastatic colorectal cancer were treated with an intravenous infusion of oxaliplatin 25 mg/m(2), 5-FU 700 mg/m(2) and leucovorin 20 mg/m(2) on days 1 to 5. The infusion of oxaliplatin was chronomodulated with a peak delivery rate at 16: 00 p.m., with 5-FU infused constantly overnight. Each course was repeated every 21 days. RESULTS: The response rate was 38.5% (95% confidence interval [CI], 13.9% to 68.4%) in the 13 measurable patients, including 1 complete response (7.7%) and 4 partial responses (30.8%). Five patients (38.5%) had a stable disease and 3 (23.0%) a progressive disease. Three patients without a measurable lesion had improved status. The median time to progression and overall survival were 29 weeks and 85 weeks, respectively. Grade 3 thrombocytopenia occurred in 2.5% (2 cycles) and grade 3 vomiting in 12.5% (2 patients). Anorexia, stomatitis, diarrhea, pruritus, alopecia and peripheral neuropathy were mild and tolerable. CONCLUSION: The modified chronomodulated infusion of oxaliplatin, 5-FU and leucovorin is effective and tolerable, but the number of patients was too small. Further study will be needed to confirm the efficacy of this regimen with a larger population of patients.

Randomized Phase III Trial of Cisplatin, Epirubicin, Leucovorin, 5-Fluorouracil (PELF) Combination versus 5-fluorouracil Alone as Adjuvant Chemotherapy in Curative Resected Stage III Gastric Cancer / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The combination of cisplatin, epirubicin, leucovorin and 5-fluorouracil (PELF) administration, as adjuvant chemotherapy after curative resection for gastirc cancer, was compared with 5-fluorouracil (5-FU) administration alone. This paper reports the results of a prospective randomized comparison of the two regimens, PELF and 5-FU. METHODS: From August 1996 to July 1999, 54 patients were selected subsequent to being diagnosed with stage III cancer after a curative resection for gastric cancer. The patients were stratified according to stage IIIA/IIIB and subtotal/total gastrectomy, and then they were randomized into each treatment group, i.e. the PELF or 5-FU alone groups. RESULTS: 54 assessable patients were enrolled in this study: 28 received PELF and 26 received 5-FU alone. 12 patients relapsed in each group and the median follow-up duration was 42 months (range: 10~77 months). The overall survival rate and disease-free survival rate (DFS) were not significantly different between two groups, (5-year survival of PELF vs. 5-FU: 57% vs. 64%, 5-year DFS: 54% vs. 51%). The PELF combination was more toxic in terms of anemia, anorexia, nausea and diarrhea than the 5-FU. CONCLUSIONS: This study showed that the PELF combination, as an adjuvant therapy for gastric cancer after a curative resection, was a less effective treatment, and it had more toxic effects than the 5-FU.

A case of primary biliary cirrhosis manifestated with idiopathic thrombocytopenic purpura and Sjogren's syndrome / 대한내과학회지

Primary biliary cirrhosis (PBC) is rare in Korea. Although its pathogenesis is not fully understood, immunologic dysregulation is suspected because it is frequently associated with other immunologic disorders. A few cases of primary biliary cirrhosis associated with idiopathic thrombocytopenic purpura (ITP) has previously been reported. However, PBC associated with ITP and Sjogren's syndrome simultaenously has not been reported yet. We experienced a 72-year-old woman had been finally diagnosed as PBC, ITP and Sjogren's syndrome. To the best of our knowledge, this is the first case in the world. We report this case with review of related literature.

High Dose Therapy Followed by Autologous Peripheral Blood Stem Cell Transplantation as a First Line Treatment for Multiple Myeloma: a Korean Multicenter Study

We conducted a phase II multicenter trial to estimate the response and survival of patients with newly diagnosed multiple myeloma to high dose melphalan therapy followed by autologous peripheral blood stem cell transplantation. Eligible patients who had undergone induction with vincristine, adriamycin and dexamethasone (VAD) should have adequate cardiac, pulmonary and renal function (creatinine <2 mg/dL). Melphalan at 200 mg/m2 was used as a conditioning regimen. Eighty patients were enrolled from 13 centers. The median age of the patients was 53 yr (range; 20 to 68 yr). The initial stage was IA/IIA/IIB/IIIA/IIIB in 3/8/1/54/14 patients, respectively. Beta2-microglobulin, CRP and LDH were increased in 74, 42 and 34% of the patients examined. Cytogenetic data were available in 30 patients, and 6 patients showed numeric or structural abnormalities. Two therapy-related mortalities occurred from infection. Among the 78 evaluable patients, CR/PR/MR/NC/PD were achieved in 48/26/2/1/1patients, respectively. After a median follow-up of 30 months, the median overall and event-free survivals were 66 months (95% CI: 20-112) and 24 months (95% CI: 18-29), respectively. This study verifies the efficacy and feasibility of high dose melphalan therapy with autologous stem cell transplantation in newly diagnosed multiple myeloma.

Pilot Study of Heptaplatin, UFT-E and Leucovorin in Advanced Gastric Carcinoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Heptaplatin (SKI-2053R, Sunpla ), a new platinum analogue which has a better toxicity profile than cisplatin, has been used with 5-fluorouracil (5-FU) continuous infusion for the treatment of advanced gastric carcinoma. However, continuous 5-FU infusion had a inconvenience to administration. The aim of this study was to evaluate the efficacy and toxicity of heptaplatin, UFT-E and leucovorin combination chemotherapy in advanced gastric cancer. MATERIALS AND METHODS: A total of 22 patients was enrolled in this study at Kyung Hee University and Korea University from September 1999 to May 2001. Heptaplatin 400 mg/m2 was given as intravenous infusion for 1 hour at day 1. Oral UFT-E 360 mg/m2 and leucovorin 45 mg/day were administered for 21 consecutive days followed by a 7-day drug free interval. This schedule was repeated every 4 weeks. RESULTS: The 22 enrolled patients received 81 courses of chemotherapy and the median number of course per patient was three with a range of one to six. Five of 21 patients achieved partial responses (23.8%; 95% confidence interval, 5.6% to 42%) without complete response. Out of the 5 responding patients, three had unresectable perigastric lymph-nodes, one patient had a ovarian metastasis, and one patient had a peritoneal metastasis respectively. Main toxicities were neutropenia and nausea/vomiting. Grade 3 and 4 neutropenia were observed in 4 patients (18%) and grade 3 nausea/vomiting were observed in 5 patients (22.7%). The median time to progression was 4 months (range, 0.5 to 13 months), and median survival duration was 7.5 months (range, 2.0 to 14 months). Median response duration was 5.0 months (range, 1.5 to 10 months). CONCLUSION: A combination chemotherapy of heptaplatin, UFT-E and leucovorin has a comparable efficacy with those of previously reported heptaplatin and intravenous regimen of 5-FU and controllable toxicity in advanced gastric carcinoma. Further study with large patient population is warranted to determine the usefulness of this regimen.

Prognostic Effect of Vascular Endothelial Growth Factor and Angiogenesis in Gastric Carcinoma / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Many studies have shown that angiogenesis has an important role in the growth, progression, and metastasis of solid tumors. Recently, several angiogenic factors have been identified. Vascular endothelial growth factor (VEGF) is a well characterized inducer of angiogenesis. In this study, we investigated the prognostic significance of the expression of VEGF in patients with an advanced gastric carcinoma. MATERIALS AND METHODS: Specimens from 54 gastric adenocarcinoma patients were stained using a polyclonal antibody against VEGF. Correlations of the expression of VEGF, microvessel density, and various other clinicopathological factors were analysed. RESULTS: Seventeen (31.5%) and 37 cases (68.5%) were VEGF-negative and positive, respectively. There was significant correlation between the expression of VEGF and pathological differentiation. There were no significant correlations between the expression of VEGF, stage and recurrence of a gastric carcinoma. The microvessel density was significantly higher in the VEGF-positive than the VEGF-negative tumors. Survivals of the VEGF-negative patients were significantly prolonged compared to those of the VEGF-positive patients. CONCLUSION: The results of this study show that the expression of VEGF may be a useful prognostic factor for patients with a gastric adenocarcinoma.

Analysis of 103 Patients with Unknown Primary Carcinoma: Retrospective Study / 대한암학회지

PURPOSE: Unknown primary carcinoma takes up approximately 0.5-10% of the oncology patients evaluated, and the patients have poor survival of between 3 to 11 months. Despite the short survival, certain clinically defined subsets of patients were reported to have a better prognosis. Thus, the objective of this study was to identify prognostic factors. MATERIALS AND METHODS: The present study was con ducted with 103 patients who were referred from January 1988 to July 1999. The primary end point was survival. The survival curves were estimated using the Kaplan-Meier method and compared using the Log-rank test and Cox's proportional hazards regression analysis. RESULTS: Most patients had histologic evidence of ade nocarcinoma or squamous cell carcinoma. Univariate and multivariate analyses identified good prognostic factors including performance status (grade 0-2), female and adenocarcinoma with more than moderate level of differentiation. The responders of chemotherapy in squamous cell carcinoma and lung, breast, ovary -estimated- cancer showed good survival rates. CONCLUSION: Unknown primary carcinoma tended to show a poor prognosis. However, when treatment modality of unknown primary carcinoma is to be determined through the prognostic factors, the patients quality of life can be improved through reducing the treatment side effects and economic burden on the patients.

A Case of Intracardiac Lymphoma as a Presentation of Non Hodgkin's Lymphoma / Journal of the Korean Cancer Association, 대한암학회지

Lymphomatous involvement of the heart is extremely rare at initial diagnosis and presentation of malignant lymphoma. Worldwide, only a few cases have been diagnosed and treated during life and only four cases were diagnosed before death in Korea. We report a case of non-Hodgkin's lymphoma with two right atrial masses detected by chest computed tomography and transesophageal echocardiography. The patient was an 80 year- old man and the presenting symptoms included generalized weakness, weight loss, constipation and low abdominal pain. For diagnosis, the mass of the perinephric area was biopsied under ultrasonographic guidance, and pathologically it was determined to be malignant lymphoma, diffuse large B cell type. The patient was treated with continuous low dose cyclophosphamide and prednisolone vice standard chemotherapy because of advanced age and renal dysfunction. After 2 months of treatment the masses in the atrium and the intraabdominal masses disappeared.