Malignant Peripheral Nerve Sheath Tumor in Neurofibromatosis Type I: Unusual Presentation of Intraabdominal or Intrathoracic Mass

A malignant peripheral nerve sheath tumor (MPNST) is an extremely rare soft tissue tumor in the general population. On the other hand, there is a higher incidence of MPNST in patients with neurofibromatosis type I (von Recklinghausen's disease). The common sites are the extremities, trunk, head and neck. However, an intraabdominal or intrathoracic manifestation is uncommon. This paper reports two patients, a 31 year-old woman with multiple neurofibromatosis presenting as an intraabdominal malignant peripheral nerve sheath tumor, and a 33 year-old woman with an intrathoracic malignant peripheral nerve sheath tumor. The patients were treated with chemotherapy followed by radiotherapy. However, one patient died as a result of disease progression 21 months after the diagnosis and the other patient is currently being treated with radiotherapy.

Docetaxel plus cisplatin combination chemotherapy in patients with advanced gastric cancer / 대한내과학회지

BACKGROUND: We evaluated the efficacy and toxicity of docetaxel plus cisplatin combination as first-line chemotherapy for advanced gastric cancer. METHODS: Patients with metastatic or recurrent gastric adenocarcinoma, performance score

Single Center Experience of Allogeneic Peripheral Blood Stem Cell Transplantation for Acute Myeloid Leukemia / 대한혈액학회지

BACKGROUND: Recently, peripheral blood (PB) stem cells have been used widely for allogeneic stem cell transplantation (SCT), instead of bone marrow (BM). The current study analyzed the outcome of allogeneic PBSCT for the patients with acute myeloid leukemia (AML). METHODS: Twenty-nine patients with AML excluding AML M3 were included for the analysis. PB stem cells were collected from HLA-matched sibling donors mobilized with G-CSF with or without GM-CSF. RESULTS: Engraftment for neutrophil and platelet were achieved in a median 15.0 days and 14.0 days, respectively. The incidence of grade II~III acute graft versus host disease (GVHD) was 82.8% (24/29 patients), while of chronic GVHD 78.6% (22/28 patients, limited 11, extensive 11). During the median follow-up of 340 days (range, 86~1,603 days), 3 years overall survival and disease free survival was 51.4% and 40.0%, respectively. CONCLUSION: PB may be considered as a feasible source of allogeneic SCT for patients with AML.

Clinical Outcomes according to Transplanted CD34+ Cell Dose in Allogeneic Peripheral Blood Stem Cell Transplantation / 대한혈액학회지

BACKGROUND: Recently, allogeneic peripheral blood stem cell transplantation (Allo-PBSCT) instead of bone marrow transplantation (BMT) has been widely used with the benefits of an earlier recovery of blood cells and a lower incidence of graft failure because of higher CD34+ cell dose. However, recent studies suggested that the higher dose of CD34+ cells might be related to the lower survival and the higher morbidity due to chronic graft versus host disease (cGVHD). We have analyzed the impact of transplanted CD34+ cell dose on clinical outcomes in unmanipulated allo-PBSCT from HLA identical siblings. METHODS: Thirty-one consecutive adult patients with hematological diseases, who survived until at least day 90 after allo-PBSCT and were evaluable for cGVHD, were included. Peripheral blood stem cells were collected from HLA-matched sibling donors mobilized with G-CSF and/or GM-CSF. The patients were classified into a "low" or "high" CD34+ cell dose group based on whether they received less or more than a median CD34+ cell dose of 11.17X10(6)/kg, respectively. RESULTS: The median CD34+ cell dose was 11.17X10(6)/kg (range, 4.12-58.80X10(6)/kg). Acute GVHD (grade II-IV) appeared in 24 patients (77.4%) and extensive cGVHD in 14 patients (45.2%). During the follow-up (median: 340 days, range: 111-1263 days), relapses were observed in 12 patients (38.7%) and 19 patients are still alive. There was a significant difference in the incidence of extensive cGVHD (20.0% vs 68.8%, P=0.011) and relapse (60.0% vs 18.8%, P=0.029) between low and high CD34+ cell dose groups, but no difference of the incidence of acute GVHD or the days of engraftments between the two groups. The estimated survival rate was significantly different between the two groups (3 year survival rate, 31.5% vs 79.8%, P=0.022) and the patients with extensive cGVHD showed a higher survival rate than those without extensive cGVHD (55.6% vs 12.5%, P=0.013). CONCLUSION: Better survival rate was observed in high CD34+ cell dose group for alloPBSCT, while a higher incidence of extensive cGVHD was noted. The optimal dose of CD34+ cells need to be determined to minimize the morbidity related to cGVHD and to improve survival.

The Effect of Preemptive Therapy with Intravenous Ganciclovir Followed by Oral Ganciclovir against Cytomegalovirus Infection after Allogeneic Peripheral Blood Stem Cell Transplantation / 대한혈액학회지

BACKGROUND: Recently, in vitro studies demonstrated faster immune reconstitution after allogeneic peripheral blood stem cell transplantation (PBSCT) compared to bone marrow transplantation (BMT). In consequence, it can be expected that better immune reconstitution against cytomegalovirus (CMV) will lead to a reduced CMV-related morbidity and mortality after allogeneic PBSCT. METHODS: Forty seven patients who received allogeneic PBSCT were enrolled. CMV was routinely sought by at least weekly screening for CMV-related matrix protein pp65 antigenemia after engraftment (WBC >1,500/nL) was achieved. CMV antigenemia was treated with ganciclovir 5mg/kg twice daily i.v. as preemptive therapy for at least 10 days. After then, ganciclovir i.v. was switched to oral ganciclovir for maintenance therapy. RESULTS: CMV antigenemia was detected 8 (17%) out of 47 patients and CMV disease developed in only 1 case (2.1%). The medianperiod of time until the detection of CMV antigenemia was 51.5 days (range, 35~230). In 7 out of 8 cases, CMV antigenemia disappeared with ganciclovir treatment in 7 days. One patient with CMV disease (CMV interstitial pneumonitis) showed persistent CMV antigenemia for 3 months and expired due to restrictive lung disease. CONCLUSION: The incidence of CMV antigenemia and resistance to ganciclovir treatment was lower than the incidence of those reported in allogeneic BMT trials. These findings suggest that faster immune reconstitution against CMV after allogeneic PBSCT might have a stronger role in the prevention of emergence of CMV antigenemia and ganciclovir treatment than after allogeneic BMT.

Report of Two Patients with Severe Aplastic Anemia Who Showed Trilineage Hematologic Response to Low-dose Erythropoietin / 대한혈액학회지

Aplastic anemia is characterized by multilineage bone marrow failure resulting in pancytopenia. There is no effective therapy for patients with severe aplastic anemia who were refractory to immunosuppresive therapy including antithymocyte globulin (ATG) or relapsed after allogeneic bone marrow transplantation. We report hereby that two patients with severe aplastic anemia who were relapsed after immu-nosuppresive therapy with ATG and cyclosporine A were successfully treated with low dose erythropoietin. Observed trilineage hematologic response was dependent on low dose erythropoietin therapy.

Peripheral Blood Stem Cell Collection through Large Volume Leukapheresis after Mobilization with GM-CSF and/or G-CSF in Normal Healthy Donors / 대한혈액학회지

BACKGROUND: The use of colony stimulating factor (CSF) has increased to mobilize hematopoietic progenitor cells for allo-PBSCT. The most effective mobilization regimen has not yet defined. The authors analyzed the results of the mobilized PBSC collection through large volume leukapheresis from 38 normal healthy donors using three different regimens, namely, a single regimen with GM-CSF (Leucogen ), a concurrent use of GM-CSF and G-CSF (Leucostim ), and a sequential regimen with GM-CSF followed by G-CSF. METHODS: This study was done on 38 healthy donors from Sep. 1998 to Jan. 2001. One donor was mobilized with G-CSF alone, 9 with GM-CSF alone, 20 with concurrent regimen and 8 with sequential regimen. After 5 days of mobilization treatment, PBSCs were collected by large volume leukapheresis through femoral vein catheter. We compared the results of each collected progenitor cells and observed the side effects. RESULTS: The average WBC count before apheresis was 22.6+-11.0x103/uL and circulating CD34+cell percent was 1.31+-2.24%. Total 66 times with an average of 1.46+-0.61 of largevolume leukapheresis were performed for the 37 donors. The mean collected MNC count was 4.61+-2.77x108/kg, CD3+cell count was 2.95+-1.82x108/kg and CD34+cell count was 9.76+-12.42x106/kg. A significant side effect observed after large volume leukapheresis was thrombocytopenia showing decrease from 199.1+-52.2 to 80.7+-25.2x103/uL without any bleeding tendency. The mean collected MNC counts provoed to be significantly higher in combination groups with GM-and G-CSF than GM-CSF alone (P<0.05). The CD34+cell counts showed to be statistically higher in a sequential group compared to the concurrent and single regimen groups (P<0.05). CONCLUSION: A mobilization protocol with combination regimens of GM-CSF and G-CSF seemed to be superior to a single regimen with GM-CSF. Large volume leukapheresis through femoral vein catheter after mobilization with combination regimens of GM-and G-CSF in normal healthy donors was safe and proved to be an excellent. method to harvest stem cells.

Single Center Report on the Variety of Clinical Applications of Allogeneic Peripheral Blood Stem Cell Transplantation / 대한혈액학회지

BACKGROUND: It is apparent that more stem cells can be harvested by mobilization treatment with recombinant human G-CSF and/or GM-CSF from normal healthy donors in allogeneic peripheral blood stem cell transplantation (allo-PBSCT) compared to allogeneic bone marrow transplantation (allo-BMT). It is also known to be more effective in inducing the graft-vs-tumor effects than allogeneic BMT because of higher T cell content. METHODS: A variety of clinical applications with allo-PBSCT was done for patients with he matological malignancies with a high risk of relapse in single transplantation center. We reported the preliminary results on trial of allo-PBSCT followed by planned prophylactic G- and/or GM-CSF primed donor lymphocyte infusion additionally reserved at harvest in hematological malignancies with a high risk of relapse and also presented the successful trial of non-myeloablative transplantation for old aged AML patient in 4th complete remission and cases with 2nd transplantation with allo-PBSCs. RESULTS: Seventeen patients with hematological malignancies with a high risk of relapse were enrolled in trial of allo-PBSCT followed by prophylactic donor lymphocyte infusion. All patients received allogeneic PBSCT from HLA- matched sibling donors. Seven out of 17 patients received additional PBSCs with a median number of CD3+ cells of 5.0x107/kg (range, 3.0 to 9.9x107/kg), between day 41 and day 120. Four surviving patients (4/7) were free of disease when last assessed (median follow-up duration, 538 days), but were suffering from chronic GVHD (1 limited and 3 extensive). A 56 year old acute myeloid leukemia patient in the 4th complete remission was successfully treated with allo-PBSCT with non-myeloablative conditioning regimen. One of 2 patients who received second transplantation with allo-PBSCT has shown a long term disease free survival. CONCLUSION: A merit of allo-PBSCT would allow us to design a variety of clinical applications. Allo-PBSCT might be preferable in special clinical setting such as non-myeloablative transplantation, second transplantation, or the situation in need of the strong GVL effect. And also CSF-primed PBSCs can be used for the purpose of donor lymphocyte infusion.

The Clinical Significance of Ann Arbor and Musshoff's Staging System in Primary Gastrointestinal Non-Hodgkin's Lymphoma-A Retrospective Analysis / 대한혈액학회지

BACKGROUND: Primary Gastrointestinal Non- Hodgkin's Lymphoma (GIL) represents 4 to 20 % of all Non-Hodgkin's Lymphoma(NHL) and gastrointestinal tract(GIT) is the most frequently involved extranodal site in NHL. It is known that the prognosis of GIL is better than that of other NHLs because of it's unique biologic behavior and anatomical location. We reviewed clinical aspects of GIL and analyzed survival data based on Ann-Arbor and Musshoff's staging system. METHODS: Sixty six cases were analyzed by age, sex, clinical manifestation, location, histology, clinical course, and two staging systems (Ann Arbor and Musshoff's modified staging). Histologies were reviewed according to REAL classification. RESULTS: The median age was 51.5 years. The most frequent gross finding was ulcerofungating lesion in upper GIL and mass lesion in lower GIL. Treatment results were as following : 76.9% of response rate, 59.5% of 5-year overall survival rate, and 54.8% of 5-year disease free survival rate. There was a significant difference of overall survival or disease free survival rate between group below stage IIE1 and above IIE2 according to Musshoff's staging system. There were no significant differences in survival between stage I and II, and between stage III and IV based on Ann Arbor staging system. CONCLUSION: There might be the necessity of discriminating localized disease (IIE1) and locally advanced lesion (IIE2) to predict the prognosis of GIL through Musshoff's staging system. Larger study will be needed to confirm the role of Musshoff's staging system.