Superior Mesenteric Artery Syndrome with Nutcracker Syndrome in a Patient with Type 1 Diabetes Mellitus / 대한내과학회지

Superior mesenteric artery (SMA) syndrome is an uncommon cause of a proximal intestinal obstruction. The most characteristic symptoms are postprandial fullness, nausea, and vomiting. The diagnosis is established by ultrasonography and contrast-enhanced computed tomography. Almost all patients respond well to conservative management. However, if conservative management fails, surgical options should be applied. In this article, we report a case of SMA syndrome with Nutcracker syndrome in a patient with diabetes mellitus. Establishing the diagnosis of Nutcracker syndrome is usually based on clinical suspicion and radiological findings. Several complications that have been reported to result from SMA syndrome include peptic ulcer disease, pancreatitis, metabolic alkalosis, and uremic syndrome. However, Nutcracker syndrome accompanied by SMA syndrome is extremely uncommon, as described in this case. To our knowledge, this association has not been reported previously.

Ileovesical Fistula Caused by Hepatocellular Carcinoma

Ileovesical fistula is a very rare clinical entity, the most frequent cause of which is Crohn's disease. Furthermore, it is an exceptionally rare complication of malignancies. We experienced one case of ileovesical fistula which had been caused by hepatocellular carcinoma (HCC) arising from the noncirrhotic liver. A 27-year-old man was diagnosed with HCC in a noncirrhotic liver. Despite treatment with transarterial chemoembolization (TACE), the disease status became more aggravated. The patient complained of dysuria, fecaluria, and intermittent lower abdominal pain. Pelvic CT scan showed a soft tissue mass of 6 cm abutting on the distal ileum which was downwardly displaced. Barium study of the small bowel showed a fistula between the small bowel loop and the urinary bladder. Upon operation, adhesion and fistula were found between the ileum and the urinary bladder. The microscopic findings of the surgical specimen were compatible with metastatic HCC. We confirmed that ileovesical fistula had been caused by metastatic HCC.

Bi-weekly Chemotherapy of Paclitaxel and Cisplatin in Patients with Metastatic or Recurrent Esophageal Cancer

Although various combinations of chemotherapy regimens have been tried for patients with esophageal cancer, their duration of survival is extremely poor. In this study, we investigated the safety and clinical efficacy of paclitaxel and cisplatin chemotherapy in metastatic or recurrent esophageal cancer. 32 patients enrolled in this study and the median age was 60 yr. Of all the 32, 28 patients (88%) had been treated previously, 22 of them with chemotherapy or radiation therapy. All patients in the study received biweekly paclitaxel (90 mg/m2) followed by cisplatin (50 mg/m2). One patient (3%) responded completely, and 12 patients (38%) showed a partial response; in 9 patients (28%) the disease remained stable, and in 10 patients (31%) it progressed. The objective response rate was 41%. The median duration of response was 4.8 months, and the median overall survival in all patients was 7 months. The 1-yr and 2-yr survival rates were 28.1% and 7.1%, respectively. Grade 3 or 4 of neutropenia and anemia were observed in 6 (19%) and 5 (16%) patients, respectively. The major non-hematologic toxicity was fatigue, but most of them could manageable. In conclusion, biweekly paclitaxel and cisplatin is effective in patients with metastatic or recurrent esophageal cancer.

The Clinical Efficacy of R-CHOP Chemotherapy in Patients with Previously Untreated Diffuse Large B-cell Lymphoma / 대한혈액학회지

BACKGROUND: In combination with standard-dose CHOP (cyclophosphamide, vincristine, adriamycin, and prednisolone), the addition of rituximab produces a better clinical response in the treatment of aggressive B-cell non-Hodgkin's lymphoma (NHL) than CHOP alone. METHODS: Thirty-four patients with previously untreated diffuse large B-cell NHL received at least three or four cycles of rituximab 375 mg/m2 or 500 mg per dose on day 1 of each cycle in combination with CHOP chemotherapy. RESULTS: The median age of patients were 61.5 years (range, 28-83 years). After the end of therapy, twenty-five patients (73.5%) experienced a complete response, four patients (11.8 %) had a partial response, and two patients (5.9%) were classified as having progressive disease. The median follow-up duration was 9.4 months (range, 0.2-19.5 months) and 1-year overall survival and progression free survival was 84.8+/-8.7% and 80.3+/-9.4%, respectively. Two patients (5.9%) experienced fever, myalgia, and skin eruption due to rituximab. Neutropenia of grade 3 or 4 occurred in thirty-one patients (91.2%). CONCLUSION: The benefits of rituximab in combination with CHOP chemotherapy include high response rates and good tolerance. However, further prospective, randomized studies are needed to draw definitive conclusions.

A case of peritoneal metastasis from gastric cancer successfully treated with docetaxel and cisplatin chemotherapy / 대한내과학회지

In case of unresectable or metastatic gastric cancer, though many trials have been going, treatment results are poor yet. We report a patient with peritoneal metastasis from gastric cancer effectively treated with docetaxel and cisplatin chemotherapy. The patient was a 33 year-old man who was confirmed poorly differenciated adenocarcinoma of stomach 5 years ago. At the diagnosis, the stage of gastric cancer was T2N3M0. He underwent subtotal gastrectomy with Billoth II anastomosis and 6th cycles of postoperative adjuvant chemotherapy consisting of FAMTX. After that, there was no evidence of recurrence. Three years later, he was admitted to our hospital complaining of abdominal pain and distension. Abdominal CT revealed that recurred gastric cancer in anastomotic site with carcinomatous peritonei and multiple lymphadenopathy. He was performed chemotherapy combined with docetaxel (75 mg/m2) and cisplatin (75 mg/m2). After 3rd chemotherapy, follow up abdominal CT showed nearly complete regression of bowel loops, lymph node and ascites. After completion of 7th cycles of chemotherapy, it remained as complete response for recurred gastric cancer and he has no evidence of recurrence for over 2 years.

Frequency of bcl-2 mbr/JH Rearrangement and Prognostic Implication of bcl-2, c-myc Protein Expression in Non-Hodgkin's Lymphoma / 대한혈액학회지

BACKGROUND: Deregulated bcl-2 appears to prolong cell survival and to cooperative with c-myc in promoting cell proliferation. We investigated whether there is any clinicopathologic correlation between the survival and the frequency of bcl-2/JH rearrangement and bcl-2, c-myc protein expression in non-Hodgkin's lymphoma. METHODS: We conducted a study for bcl-2 mbr/JH rearrangement with polymerase chain reaction and for bcl-2, c-myc expression with immunohistochemical staining in 46 formalin-fixed, paraffin-embedded non-Hodgkin's lymphoma tissues of patients treated with CHOP chemotherapy including 37 specimens of diffuse large cell type. RESULTS: The bcl-2 mbr/JH rearrangement was positive in 13% (6/46) of non-Hodgkin's lymphoma specimens. For bcl-2, strong positive reaction (3+) and 2+ positive reaction were seen in 16 (35%) and 16 cases (35%), respectively; while 3+ and 2+ reactions were found in 20 (44%) and 16 cases (35%), respectively, for c-myc by immunohistochemistry. Eighty one percent of positive cases for bcl-2 were also positive for c-myc simultaneously. The 6 cases with bcl-2 mbr/JH rearrangement were weakly positive at 3 cases and strong positive at 3 cases for bcl-2 by staining. In cases of diffuse large cell lymphoma, high expression (3+) of bcl-2 & c-myc protein tended to have a shorter 2 year survival, though it was statistically not significant. CONCLUSION: High expression of bcl-2 and c-myc protein suggest that bcl-2 cooperate with c-myc in tumorigenesis of aggressive non-Hodgkin's lymphoma. The prognostic implication of bcl-2 and c-myc expression in diffuse large cell lymphoma patients needs to be evaluated in a larger, prospective cohort to draw a definitive conclusion.

Clinical Effect of Granulocyte Transfusion Therapy in Neutropenia-Related Infection / 대한혈액학회지

BACKGROUND: After a zealous advocates of granulocyte transfusion therapy (GTX) in the 1970s and early 1980s, the use of GTX has diminished strikingly because of the several problems of GTX and the introduction of new antimicrobial agents and recombinant hematopoietic growth factors. Recently, GTX offers renewed interest because several investigators reported the transfusion efficacy of granulocytes collected by stimulating normal donors with recombinant human granulocyte-colony stimulating factor (G-CSF). METHODS: To evaluate the safety and efficacy of GTX, thirteen patients with neutropenia- related infections at Chonnam University Hospital from March 1997 to February 1998 were treated with dexamethasone- or G-CSF-stimulated granulocyte transfusions apheresed from normal donor. RESULTS: Patients received a mean number of 2.4 transfusions (range, 1-7) and a mean dose of 5.5x1010 granulocytes (range, 0.2-19.6). Six patients (46.2%) had favorable responses. Favorable responses occurred among patients with more fungal infection than bacterial infection (71.4 vs 28.6%, P<0.05) and more increment of absolute neutrophil count at 1 hour after transfusion (P<0.05). Adverse reactions of GTX were pulmonary edema in 2 patient (15.4%) and transient hypoxia in 1 patient (7.7%). One patient (7.7%) with pulmonary edema died of severe pulmonary reaction. Two of 20 donors received by G-CSF complained of mild myalgia and bone pain. CONCLUSION: G-CSF- or dexamethasone-stimulated GTXs were well tolerated and may be clinically beneficial for neutropenia-related infection, particularly in fungal infection, that is refractory to antimicrobial therapy.

Treatment of Acute Promyelocytic Leukemia (APL) by a Combination of All-Trans Retinoic Acid (ATRA) and Chemotherapy / 대한혈액학회지

BACKGROUND: All-trans-retinoic acid (ATRA) induces complete remission (CR) in the great majority of patients with PML/RAR -positive acute promyelocytic leukemia (APL). However, it is associated with a rapid rise in leukocytes in one third to half the patients, with potentially fatal "ATRA syndrome". Furthermore, most of the patients relapse with maintenance therapy using ATRA alone or low-dose chemotherapy. In this study, we have analyzed the outcome for APL patients who were treated with ATRA alone or combined with low-dose chemotherapy followed by postremission chemotherapy in Chonnam University Hospital from April 1993 to December 1997. METHODS: Sixteen patients with newly diagnosed APL were eligible to analysis. Patients received 45mg/m2 ATRA until CR occurred. If initial WBC were above 5,000/microliter, low-dose chemotherapy was concomitantly given, and if during the ATRA therapy WBC were above 5,000/microliter by day 5 or 10,000/microliter by day 10, or 15,000/microliter by day 15, low-dose chemotherapy was added. Four polychemotherapy cycles or allogeneic bone marrow transplantation were given as postremission therapy. RESULTS: Median age was 34 years (range, 17 to 67). Of 16 APL patients, 15 (93.8%) achieved CR and 1 (6.2%) died of intracerebral hemorrhage. After a median follow-up of 11.5 months (range, 0 to 47), the Kaplan-Meier estimated overall survival (OS) rate was 87.1 +/- 8.6% at 3 year, the event-free survival (EFS) rate was 87.1 +/- 8.6%, 58.0 +/- 24.4% and 29.0 +/- 23.9% at 1 year, 2 year and 3 year, and the disease-free survival (DFS) rate was 92.9 +/- 6.9%, 69.6 +/- 20.7% and 46.4 +/- 23.5% at 1 year, 2 year and 3 year, respectively. CONCLUSION: The present study suggests that ATRA with or without low-dose chemotherapy followed by postremission chemotherapy is a well-tolerated and effective regimen that is shown to improve the CR rate, reduce a early mortality rate and considerably prolong the overall survival in patients with newly diagnosed APL.

Characterization of Peripheral Blood CD34+ Cells Mobilized by Recombinant Human Granulocyte-Colony Stimulating Factor in Healthy Adult Donors / 대한혈액학회지

BACKGROUND: CD34+ cells are capable of engraftment and hematopoietic reconstitution. However, expression patterns of other surface antigens such as CD13, CD33, CD38 and HLA-DR on CD34+ cells in mobilized peripheral blood have remained unclear. This study analyzed the expansion kinetics of the CD34+ cells and subsets in the peripheral blood of healthy donors treated with recombinant human Granulocyte Colony-Stimulating Factor (rhG-CSF), the relative composition of CD34+ subsets in mobilized peripheral blood and apheresis product, the yield of apheresis product. METHODS: The 6 peripheral blood stem cell donors received a daily dose of 500 microgram (8.1~10 microgram/kg) of rhG-CSF subcutaneously for 6 or 7 days. The hematologic parameters and the number of CD34+ cells and subsets in peripheral blood were recorded at baseline and daily for a total 6 to 7 days. With monoclonal antibodies which were designed for two color direct immnunofluorescence (IF) analysis with combination of fluorescence isothiocyanate (FITC) and phycoerythrin (PE) conjugated, CD34CD38, CD34HLA-DR, CD34CD13, and CD34CD33 surface antigens were analyzed by flow cytometry. RESULTS: The number of CD34+ cells mobilized to peripheral blood peaked at day 4 or 5 with rhG-CSF treatment. Circulating CD34+ cell expanded by 11.5-fold from 4.5 +/- 1.8x106/L before rhG-CSF treatment to maximal increment 51.9 +/- 13.4x106/L after mobilization. Subsets of CD34+CD38-, CD34+HLA-DR-, CD34+ CD13-, and CD34+CD33- (in % ofthe CD34+ cell) were all decreased and subsets of CD34+CD38+, CD34+HLA-DR+, CD34+CD13+, and CD34+CD33+ were all increased after mobilization, so that the numbers of early committed and myeloid committed progenitors were higher than the those of multipotent stem cells in mobilized peripheral blood and leukapheresed products. Each apheresis product yielded a mean of 451.6x106 (7.7x106/kg of RBW) CD34+ cell and 172.3x105 (2.9x105/kg of RBW) CFU-GM. CONCLUSION: Sufficient amount of CD34+ cells capable of engraftment and hematopoietic reconstitution can be mobilized by administration of rhG-CSF to healthy adult donor. Subsets of mobilized CD34+ cells were mainly composed of early committed and myeloid committed progenitors, although absolute numbers of all progenitor cells including multipotent stem cells were significantly increased.