Redo pull-through for postoperative complications following pull-through in Hirschsprung disease: a single center experience

Purpose@#Although surgical management of Hirschsprung disease (HD) is effective in most patients, some patients experience long-term postoperative complications, and require redo pull-through (PT). The present study evaluated clinical outcomes of redo PT in HD patients at a single center. @*Methods@#Patients with HD who underwent redo PT procedures between 2003 and 2019 were retrospectively reviewed. @*Results@#Thirteen patients were included. Five (38.5%) had undergone initial PT surgery at our center and 8 (61.5%) at other centers. Redo PT procedures were transanal endorectal PT in 12 patients (92.3%) and the posterior sagittal approach in 1 patient (7.7%). Indications for redo PT included pathologic misdiagnosis in 8 patients (61.5%); stricture in 2 (15.4%); and rectal stenosis, obstructing Duhamel pouch and remnant septum in 1 each (7.7%). At a median follow-up of 68 months (range, 3–227 months) after redo PT, 8 patients (61.5%) had normal bowel function, 2 (15.4%) had incontinence, and 1 (7.7%) had constipation. @*Conclusion@#Redo PT procedures could be an effective approach for improving obstructive symptoms in HD patients with anatomic or pathologic reasons following primary PT. Careful selection of patients and discreet indications for redo PT are crucial.

The Treatments for Intermediate Risk Prostate Cancer

The population of intermediate-risk prostate cancer patients is a large heterogeneous group with diverse prognoses and challenges the struggle to develop more meticulous and standardized treatment recommendations. Furthermore, there are no specific treatment guidelines based on Korean patients although the cancer nature of this patient group is known to be somewhat different from those of western patients. This review will examine the treatment options for intermediate-risk prostate cancer patients in specific clinical situations. The literature provides evidence that combining androgen deprivation therapy (ADT) for 6 months with radiation therapy (RT) may have superior survival than RT alone. Also, patients with adverse pathologic features and lymph node metastasis will benefit from adjuvant RT and ADT respectively after radical prostatectomy (RP). In addition, patients with life expectancy ≥10 years will benefit from lymphadenectomy during RP when lymph node metastasis is suspected as well as neurovascular bundle saving during RP is recommended because of its superior functional results.

Clinicopathological Characteristics of Urinary Bladder Tumors in Korean Patients 20 Years or Younger

BACKGROUND: To investigate the clinicopathological characteristics of urinary bladder tumors, a rare malignancy, in patients 20 years or younger. METHODS: Using a retrospective chart review among patients who received bladder surgery at 2 institutions between July 1996 and January 2013, we analyzed the clinicopathological characteristics of urinary bladder tumors in 21 pediatric patients (male:female = 4.25:1.00; mean age, 12.1 years). RESULTS: Pathology revealed 9 urothelial tumors, 6 rhabdomyosarcomas, 1 low-grade leiomyosarcoma, 1 large cell neuroendocrine carcinoma, 1 inflammatory myofibroblastic tumor, and 3 cases of chronic inflammation without tumors (including 1 xanthogranulomatous inflammation). Urothelial tumors (mean patient age, 16.0 years) were benign or low-grade; and only transurethral resection of the bladder tumor was necessary for treatment. Patients with rhabdomyosarcomas (mean age, 5 years) underwent radiotherapy (if unresectable) or transurethral resection of the bladder tumor (if resectable), after chemotherapy. Of these patients, 2 underwent radical cystectomy, with the remaining patients not receiving a cystectomy. With the exception of one patient, all patients are currently alive and recurrence-free. CONCLUSION: Urothelial tumors were the most commonly found pediatric bladder tumor, with embryonal rhabdomyosarcoma being the second most common. Urothelial tumors are common in relatively older age. Since urothelial tumors in children typically have a good prognosis and rarely recur, transurethral resection of the bladder tumor is the treatment of choice. Rhabdomyosarcomas are common in younger patients. Since rhabdomyosarcoma is generally chemosensitive, chemotherapy and radiotherapy are the treatment of choice for bladder preservation in these patients.

Management of patients with refractory nocturnal enuresis

There are two types of refractory nocturnal enuresis. The first type corresponds to patients who are refractory from initial success, and the second type refers to refractory nocturnal enuresis after long-term success, in patients who cannot discontinue medications for enuresis. In the former type, it is necessary to determine whether the timing of medications is appropriate, whether the usage of antidiuretics is appropriate, whether any lifestyle changes have taken place, and whether there are secondary causes of enuresis. In the latter type, enuretic alarm treatment should be considered initially, and it is then important to investigate whether a respiratory obstruction is present in patients with nocturnal polyuria, whether the patient is constipated, and whether patients with non-monosymptomatic nocturnal enuresis have lower urinary tract symptoms or attention deficit hyperactivity disorder.

Magnification-Assisted Subinguinal Varicocelectomy with Testicular Delivery in Children: A Preliminary Report

PURPOSE: To evaluate the effectiveness of magnification-assisted subinguinal varicocelectomy (MASV) with testicular delivery in children with severe varicocele. MATERIALS AND METHODS: We retrospectively analyzed the clinical data of 23 children 15 years or younger (mean age, 12.3+/-1.8 years) who underwent MASV with testicular delivery and ligation of all collateral veins except arteries and deferential veins between January 2010 and January 2014. All patients had grade 3 varicocele on the left side. Varicocelectomy was decided upon due to scrotal hypotrophy (n=14, 60.9%), the existence of mass (n=6, 26.1%, including 1 recurrent case), and discomfort (n=3, 13.0%). The preservation of internal spermatic artery (ISA) was successful in 8 patients (34.8%). The mean follow-up time was 10.8+/-6.6 months. RESULTS: The surgical success rate of varicocelectomy was 100%. The overall symptom resolution rate was 91.3%. The scrotal mass and discomfort disappeared, but testicular catch-up growth did not occur in 2 among 14 patients with scrotal hypotrophy. The left testis volume increased from 6.5+/-4.3 mL to 10.6+/-7.5 mL (p=0.003). There were no significant inter-group differences in terms of the surgical success rate, symptom resolution, and catch-up growth between the ISA preservation group and the ligation group. None of the subjects demonstrated testicular atrophy or hydrocele after surgery. CONCLUSIONS: MASV with testicular delivery is an effective and safe method for children with severe varicocele.

Magnification-Assisted Subinguinal Varicocelectomy with Testicular Delivery in Children: A Preliminary Report

PURPOSE: To evaluate the effectiveness of magnification-assisted subinguinal varicocelectomy (MASV) with testicular delivery in children with severe varicocele. MATERIALS AND METHODS: We retrospectively analyzed the clinical data of 23 children 15 years or younger (mean age, 12.3+/-1.8 years) who underwent MASV with testicular delivery and ligation of all collateral veins except arteries and deferential veins between January 2010 and January 2014. All patients had grade 3 varicocele on the left side. Varicocelectomy was decided upon due to scrotal hypotrophy (n=14, 60.9%), the existence of mass (n=6, 26.1%, including 1 recurrent case), and discomfort (n=3, 13.0%). The preservation of internal spermatic artery (ISA) was successful in 8 patients (34.8%). The mean follow-up time was 10.8+/-6.6 months. RESULTS: The surgical success rate of varicocelectomy was 100%. The overall symptom resolution rate was 91.3%. The scrotal mass and discomfort disappeared, but testicular catch-up growth did not occur in 2 among 14 patients with scrotal hypotrophy. The left testis volume increased from 6.5+/-4.3 mL to 10.6+/-7.5 mL (p=0.003). There were no significant inter-group differences in terms of the surgical success rate, symptom resolution, and catch-up growth between the ISA preservation group and the ligation group. None of the subjects demonstrated testicular atrophy or hydrocele after surgery. CONCLUSIONS: MASV with testicular delivery is an effective and safe method for children with severe varicocele.

Impact of Adjuvant Androgen-Deprivation Therapy on Disease Progression in Patients with Node-Positive Prostate Cancer

PURPOSE: The survival benefits of adjuvant androgen-deprivation therapy (ADT) in prostate cancer and lymph node metastasis remain unclear. We assessed the role of ADT in disease progression after radical prostatectomy (RP). MATERIALS AND METHODS: Of 937 patients who underwent RP, we identified 40 (4.2%) who had lymph node metastasis. A total of 18 received adjuvant ADT (ADT group) and 22 were observed (observation group). Clinical progression-free survival (PFS), cancer- specific survival (CSS), and overall survival (OS) were compared in the 2 groups. Prognostic factors for clinical progression and biochemical recurrence (BCR) were analyzed. RESULTS: The 5-year PFS, CSS, and OS of the entire cohort were 75.0%, 85.0%, and 72.5%, respectively. In the ADT group, 6 patients (33.3%) showed clinical progression at a median 42.7 months. The 5-year PFS, CSS, and OS rates of this group were 72.2%, 83.3%, and 72.2%, respectively. In the observation group, 14 patients (63.6%) received salvage therapy owing to BCR. Nine patients (40.9%) with BCR in the observation group showed clinical progression at a median 43.4 months after RP. The 5-year PFS, CSS, and OS rates of this group were 77.2%, 86.4%, and 72.8%, respectively. In the observation group, the BCR rate was lower in patients with pT3a or less disease than in those with pT3b disease. CONCLUSIONS: Adjuvant ADT in node-positive prostate cancer did not reduce or delay disease progression or improve survival. Because a substantial number of untreated patients with pT3a or less disease did not experience recurrence, administration of ADT should be initiated carefully. However, in patients with pT3b disease, adjuvant ADT and radiotherapy could be considered.

Pediatric Pancreatic Tumors-Clinical Experience

Pancreatic tumors in children are relatively rare, and their prognosis differs from that in adults. The purpose of this study is to examine the clinical characteristics, treatment, and prognosis for children with pancreatic tumors. We retrospectively reviewed the medical records of children under 15 years of age with pancreatic tumors who were treated surgically at Asan Medical Center between January 1992 and November 2009. There were 16 patients, fourteen of whom were pathologically diagnosed with solid pseudopapillary tumor. The other two patients were diagnosed with pancreatoblastoma and acinar cell carcinoma, respectively. Six patients of the 16 patients (38%) were male, and there was a male-to-female ratio of 1:1.6. The initial presentations were upper abdominal pain in eight patients (50%), palpable abdominal mass in three, and vomiting in one. Four patients were diagnosed incidentally. Six patients' tumors were located in the pancreatic head, six in the pancreatic body, and four in the pancreatic tail, respectively. The surgical procedures performed included distal pancreatectomy (n=7, 44%), median segmentectomy (n=3), enucleation (n=3), pancreaticoduodenectomy (n=2), and pylorus-preserving pancreaticoduodenectomy (n=1). Three patients underwent laparoscopic surgery. The median tumor size was 6.5cm (1.8~20 cm). Early surgical complications included pancreatic fistula (n=4), bile leakage (n=1), and delayed gastric emptying (n=1). A late complication in one patient was diabetes. The median follow-up period was five years and four months, and all patients survived without recurrence. While pancreatic tumors in adults have a poor prognosis, pancreatic tumors of childhood are usually curative with complete resection and thus have a favorable prognosis.

Biochemical Recurrence-Free and Cancer-Specific Survival after Radical Prostatectomy at a Single Institution

PURPOSE: To analyze the biochemical recurrence-free and cancer-specific survival after radical prostatectomy in a consecutive series of patients with prostate cancer. MATERIALS AND METHODS: We retrospectively reviewed data for 1,822 patients who underwent radical prostatectomy with pelvic lymph node dissection at our institution between 1990 and 2009. After excluding 498 patients who were treated with neoadjuvant androgen deprivation therapy or who were followed up for < or =6 months, we included 1324 patients (mean age, 64.4 years; mean prostate-specific antigen [PSA] level, 12.3 ng/ml). We assessed patient age at the time of surgery, preoperative PSA concentration, biopsy and pathologic Gleason scores, pathologic stage, surgical margin status, disease progression, and survival. RESULTS: The mean follow-up time was 40 months (range, 6-193 months). The 5- and 10-year biochemical recurrence-free survival rates were 73.2% and 66.2%, respectively, and the 10-year cancer-specific survival rate was 92.4%. The mean time from surgery to biochemical recurrence was 18 months. In the multivariate analysis, Gleason score (4+3 vs. 2-6, p=0.004; 8-10 vs. 2-6, p<0.001), pathologic stage (pT3a vs. pT2, p=0.001; pT3b-4 vs. pT2, p<0.001; pN1 vs. pT2, p<0.001), and resection margin status (p<0.001) were statistically significant predictors of biochemical recurrence, with only pathologic stage (pT3b-4 vs. pT2, p=0.006; pN1 vs. pT2, p=0.010) being a statistically significant predictor of cancer-specific survival. CONCLUSIONS: Radical prostatectomy resulted in favorable cancer control in more than 70% of patients after 5 years and a low (<10%) cancer-specific mortality rate after 10 years. The factors predictive of biochemical recurrence were Gleason score, pathologic stage, and resection margin status.

Experience with Laparoscopic Pyeloplasty, Including Robot-Assisted Laparoscopic Surgery, for Ureteropelvic Junction Obstruction

PURPOSE: Laparoscopic pyeloplasty was developed as a minimally invasive alternative to an open procedure for the treatment of ureteropelvic junction (UPJ) obstruction. We present our experience with the first 30 consecutive cases of laparoscopic pyeloplasty performed at our institution. MATERIALS AND METHODS: We studied 30 patients with ureteropelvic junction obstructions who underwent laparoscopic pyeloplasty between March 2004 and March 2009. Of the 30 patients, 5 patients underwent robot-assisted laparoscopic pyeloplasty (RALP) since April 2008. Patients were divided into 4 groups according to operative procedure: group 1, early laparoscopic pyeloplasty-dismembered (E/LP-D, n=9); group 2, late laparoscopic pyeloplasty-dismembered (L/LP-D, n=9); group 3, laparoscopic pyeloplasty-Fenger's method (LP-F, n=7); and group 4, RALP (n=5). RESULTS: The mean age of the patients was 34.0+/-12.8 years (range, 17-61 years). A crossing vessel was present in 37.9% of cases. Mean follow-up was 30+/-14 months (range, 11-62 months). Mean operative time was 267.3+/-78.7 minutes (range, 154-460 minutes), and the average length of the postoperative hospital stay was 4.6+/-1.6 days (range, 3-10 days). There were no intraoperative complications or transfusion. The success rate was 73.3%. The success rates of E/LP-D, L/LP-D, LP-F, and RALP were 6/9 (66.7%), 7/9 (77.8%), 5/7 (71.4%), and 4/5 (80%), respectively, without significant difference (p>0.05). Operation time and length of hospital stay were shorter in the L/LP-D group than in the E/LP-D group. CONCLUSIONS: Laparoscopic pyeloplasty may be an alternative treatment for an ureteropelvic junction obstruction, but the technical complexity of the procedure has made it difficult for many surgeons to adopt. RALP is a technically feasible management option for UPJ obstruction.

Transperitoneal Laparoscopic Upper Pole Heminephrectomy in Pediatric Patients with Duplex Kidneys: Comparison with an Age-Matched Cohort of Open Surgery

PURPOSE: We evaluated the safety and feasibility of transperitoneal laparoscopic upper pole heminephrectomy (TLUHN) in pediatric patients by comparing an age-matched cohort undergoing open upper pole heminephrectomy (OUHN) by a single surgeon. MATERIALS AND METHODS: A total of 10 OUHNs were performed between 1995 and 2003 and 10 TLUHNs between 2003 and 2008. The 10 patients in each group were matched by age (p=0.94), gender (p=1.0), and body weight (p=0.91). RESULTS: There was no open conversion in the TLUHN group. The median operative time in the TLUHN and OUHN groups was 174 minutes (range, 98-205) and 190 minutes (range, 120-258), respectively (p=0.19). In the TLUHN and OUHN groups, the median blood loss was 25 ml (range, 20-30) and 35 ml (range, 20-100) (p=0.74), the median analgesic requirement was 0 hours (range, 0-42) and 0 hours (range, 0-87) (p=0.16), and the median hospital stay was 5.5 days (range, 3-7) and 7 days (range, 3-19) (p=0.01), respectively. There were no intraoperative complications in either group. Postoperative atelectasis occurred in two and five patients in the TLUHN and OUHN groups, respectively. Similar results were found in the subanalysis of patients younger than 24 months. CONCLUSIONS: TLUHN is safe and feasible even in small children and infants. Furthermore, decreased hospital stay is the main advantage of the laparoscopic approach compared with open surgery.

A Case of Infectious Aortitis Causing Recurrent Acinetobacter baumannii Bacteremia Following Aortic Valve Replacement

Infectious aortitis is an uncommon yet, life threatening disease. Early surgical treatment and prolonged antibiotic therapy is crucial to survival. Salmonella sp. and Staphylococcus aureus are the most common organisms isolated. There is no case report of infectious aortitis caused by Acinetobacter baumannii, which has recently emerged as a major cause of health care-associated infections. Here, we describe a 76-year-old male with infectious aortitis who experienced recurrent bacteremia due to A. baumannii in spite of adequate antimicrobial therapy after aortic valve replacement.

A Case of Infectious Aortitis Causing Recurrent Acinetobacter baumannii Bacteremia Following Aortic Valve Replacement

Infectious aortitis is an uncommon yet, life threatening disease. Early surgical treatment and prolonged antibiotic therapy is crucial to survival. Salmonella sp. and Staphylococcus aureus are the most common organisms isolated. There is no case report of infectious aortitis caused by Acinetobacter baumannii, which has recently emerged as a major cause of health care-associated infections. Here, we describe a 76-year-old male with infectious aortitis who experienced recurrent bacteremia due to A. baumannii in spite of adequate antimicrobial therapy after aortic valve replacement.

Clinical Factors that Predicts Successful Eradication of Helicobacter pylori / 대한소화기학회지

BACKGROUND/AIMS: Antibiotic resistance and compliance are regarded to be important which affect the eradication of Helicobacter pylori (H. pylori). However, it is not easy to apply the antibiotic resistance test in clinical field. We investigated other clinical factors predicting the successful eradication of H. pylori. METHODS: From January 2004 to March 2005, 195 patients with documented H. pylori infection received proton pump inhibitor (PPI)-based triple therapy for one week and were assessed for the underlying chronic illnesses, smoking, alcohol habit, therapeutic indication and compliance. RESULTS: The intention-to-treat (ITT) eradication rates were 69.2%, while per protocol (PP) analysis with 169 patients showed an initial eradication rate of 79.9%. The eradication rates of H. pylori according to the underlying disease were 73.9% (17/23) in diabetes, 66.7% (18/27) in hypertension, 66.7% (2/3) in renal disease, 100% (9/9) in liver disease, 63.7% (7/11) in cardiovascular disease and 64.3% (9/14) in chronic NSAIDs user. There was no statistical difference in the eradication rates according to the therapeutic indication, underlying disease, sex, age, smoking, alcohol, and PPI. However, the eradication rate was statistically lower in patients with multiple underlying diseases. Eradication rate was significantly higher in patients with good compliance than in those with poor compliance in taking medications (p<0.05). CONCLUSIONS: Underlying chronic disease does not affect the H. pylori eradication rate significantly. In clinical practice, apart from antibiotic resistance test, drug compliance is the most important factor affecting the H. pylori eradication rate.

Delayed activation-induced T lymphocytes death in aplastic anemia: related with abnormal Fas system

OBJECTIVES: To quantitate apoptosis and Fas antigen expression of T lymphocytes by activation in aplastic anemia (AA) and compare with that of normal controls and completely-recovered AA, and to investigate the apoptotic sensitivity to anti-fas antibody of activated T lymphocytes in AA. METHODS: We studied the expression of Fas antigen on fresh T lymphocytes of twenty patients with AA [13 newly diagnosed, 7 recorvered AA after immunosuppressive therapy (IST)], and investigated the activation-induced cell death (AICD) and Fas expression by activation [interleukin-2 (200 U/ml) and phytohemagglutinin (50 micrograms/ml)] in 5 newly-diagnosed AA, 5 normal controls and 5 AA in complete response (CR). Apoptotic sensitivity to anti-Fas antibody was assessed by the time-course kinetics of induction of cell death by anti-Fas antibody (500 ng/ml). RESULTS: There was no significant difference of Fas antigen expression on freshly-isolated T lymphocytes among newly-diagnosed severe AA, normal control s and patients with AA in CR after IST. In normal controls, T lymphocytes death was greatly increased at 3 days of activation, and Fas antigen expression on T lymphocytes was increased above baseline at day 1 of activation. In contrast, in newly-diagnosed AA, T lymphocytes showed delayed cell death, which correlated with a slowed increase of Fas antigen expression by activation. Also, anti-Fa s antibody sensitivity of activated T lymphocytes was decreased in newly-diagnosed AA. In completely recovered AA, these abnormal AICD and Fas antigen expressions by activation were recovered to normal range. CONCLUSIONS: Abnormal AICD plays a role in the immune pathophysiology of AA, and defective Fas system is involved in this process.

Invasive Pulmonary Aspergillosis Involving Right Subclavian Artery and Chest Wall / 대한내과학회지

Invasive aspergillosis is an infection that occurs in immunocompromised patients. Its prevalence was increased in the last decade with progression of antineoplastic chemotherapy and immunosuppressive therapy after transplantation. Because it carries a high mortality and morbidity, early diagnosis and aggressive treatment are critical for successful management. In many patients, invasive aspergillosis remains confined to the lung although direct extension to pleural cavity or pericardium has been reported. However great vessel involvement is rare. Therefore we report a case of invasive aspergillosis involving right subclavian artery and chest wall in a patient after chemotherapy for acute lympoblastic leukemia.

Cytomegalovirus Infection in Stem Cell Transplantation / 대한혈액학회지

BACKGROUND: Cytomegalovirus (CMV) infection causes the greatest morbidity and mortality after stem cell transplantation (SCT), and in many western studies, CMV infection develops in approximately 70 to 80% of patients treated with allogeneic bone marrow transplantation (BMT). There have been no reports regarding the prevalence and clinical features of CMV infection and disease in patients receiving SCT in Korea. Therefore, we investigated the frequency and clinical characteristics of CMV infection in 53 cases of SCT. METHODS: Underlying diseases were acute myelocytic leukemia (n=21), acute lymphocytic leukemia (n=14), chronic myelocytic leukemia (n=4), severe aplastic anemia (n=13) and non- Hodgkin lymphoma (n=1). Pre-transplant serostatus of donors and recipients was all positive for CMV infection. For screening test of CMV detection, CMV antigen assay or shell vial culture was done. CMV infection was defined as CMV antigenemia or recovery of CMV in culture or positive CMV PCR, and CMV disease was diagnosed when patients had symptoms and signs of specific organs with CMV infection. RESULTS: 1) The incidence of CMV infection was 18.9% (10/53) in all transplant recipients. No significant difference of rate of infection and disease between allogeneic BMT and autologous peripheral blood stem cell transplantation was observed. 2) Five of 10 patients with CMV infection had asymptomatic CMV infection, and the other 5 patients developed overt CMV disease. 3) There was no difference of incidence of CMV infection according to age, type of SCT and use of total body irradiation. But, patients with acute graft-versus-host disease (GVHD) had significantly higher incidence of CMV infection than those without acute GVHD in allogeneic BMT (33.3 vs. 7.4%; P=0.04). 4) CMV antigen assay was evaluated as the most sensitive method for the detection of CMV (83.3%), whereas CMV culture showed the lowest sensitivity (37.5%). CONCLUSIONS: The lower incidence of CMV infection of patients with SCT in our study than that of other western countries may be associated with low incidence of acute GVHD.

The Effect of Immunosuppressive Therapy on Aplastic Anemia: Long-Term Treatment Outcome / 대한혈액학회지

BACKGROUND: There has been much evidence that immune-mediated stem cell injury may have a significant role in the pathogenesis of aplastic anemia, and as a result immunosuppressive therapy has become known as an effective treatment for patients with aplastic anemia. There have been no reports regarding the long-term follow-up of immunosuppressive therapy of patients with aplastic anemia in Korea. Therefore, we evaluated the response to immunosuppressive therapy for 47 patients with aplastic anemia, investigating the long-term survival, relapse rate and secondary clonal hematologic diseases. METHODS: Antithymocyte globulin (ATG) or antilymphocyte globulin (ALG) was given with cyclosporin A (CsA) to 39 patients (ATG+CsA 27, ALG+CsA 12), and to 8 patients, ATG or ALG alone was given (ATG 6, ALG 2). ATG was administered for 5 (or 8) days, and ALG was administered for 5 days. CsA was orally begun with ATG or ALG for a median total of 4.5 (3~14) months. RESULTS: 1) Response: Among 47 patients, 30 (63.8%) responded; 11 showed complete response (CR) and 19 showed partial response (PR). 2) Factors affecting response: Responses were equally distributed when patients were stratified for age, neutrophil counts and drug regimens. 3) Survival: Median duration of follow-up was 36 (6~84) months. Actuarial survival at 1 year was 100% in responders and 76% in nonresponders, and the 7-year actuarial survival rate was 94% and 76%, respectively (P value = 0.13). 4) Relapse: Relapse occurred in 4 of 30 responding patients. Relapse in patients with CR was not observed during follow-up. The risk of relapse was 12% at 2 years and 22% at 6 years. 5) Treatment outcome according to disease duration: There was no significant difference in response rate between patients treated within 4 months and beyond 4 months after diagnosis. But the latter group showed a significantly higher relapse rate than the former (4% vs 60%; P value = 0.01). 6) Side effects and complications: There were no serious side effects requiring discontinuation of immunosuppressive therapy. Evolution to secondary clonal hematologic diseases was not observed during follow-up. CONCLUSION: Our results of immunosuppressive therapy show the excellent long-term outcome. A prospective study is needed for the establishment of the adequate treatment duration of CsA and the follow-up period for the evaluation of the response of treatment.

Monitoring of WT-1 gene expression in peripheral blood of patients with acute leukemia by semiquantitative RT-PCR; possible marker for detection of minimal residual leukemia

The expression of the WT-1 gene which is found exclusively in human leukemic blasts frequently disappears from bone marrow of leukemia patients in complete remission (CR). Using semiquantitative RT-PCR, we investigated the expression of the WT-1 gene in peripheral bloods (PBs) of 33 patients with acute leukemia (AML 26; ALL 7) and monitored its expression after achievement of CR. None of the 6 normal controls expressed detectable levels of WT-1 transcripts (< 10(-4), background level), whereas 31 (93.9%) of 33 patients expressed variable levels of WT-1 transcripts (range, 10(-4) to 10(1)) at diagnosis. The level of WT-1 expression was not different between AML and ALL. By monitoring WT-1 gene expression in PB of 31 patients during CR, 5 patients relapsed (two from the 18 patients with undetectable levels of WT-1 gene expression and three from the 13 with WT-1 gene expression in low levels). Three of the 5 relapsed patients showed preceding reappearance or rise of WT-1 gene expression. From these results, we reconfirmed that the WT-1 gene is a pan-acute leukemic marker, which can be used to monitor minimal residual leukemia (MRL) after chemotherapy or in patients with CR.

A Case of Rh-incompatible Allogeneic Bone Marrow Transplantation: Transplant of E- and c-positive Bone Marrow in a Recipient with Anti-E and Anti-c Antibodies / 대한수혈학회지

The effects of Rh incompatibility in bone marrow transplantation (BMT) has not been studied extensively as ABO incompatibility. But it has been known that Rh incompatibility does not impair the engraftment of donor bone marrow. The recipient's blood groups were AB, DCe, who has anti-E and anti-c in her serum. The sibling donor blood groups were B, DCEce. There was a minor ABO incompatibility and Rh incompatibility due to anti-E and anti-c between donor and recipient. We performed the plasma and RBC depletion of marrow using the cell separator before it was infused. The clinical course after BMT was not eventful except mild delayed hemolysis and delayed hematopoietic recovery, which may be attributable to low marrow cell dose.