Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome in a Child: A Successful Treatment with the BFM-NHL-90 Protocol / 임상소아혈액종양

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct cutaneous lymphoma subtype that is characterized by pleomorphic T-cell infiltration of the subcutaneous tissue. SPTCL is usually associated with indolent clinical course. However, it can be complicated by hemophagocytic syndrome (HPS), which leads to worse prognosis. Childhood SPTCL is rare and there is no standardized treatment regimen of SPTCL with HPS. Here we report a pediatric case of SPTCL with HPS who responded favorably with multi-agent chemotherapy of the BFM (Berlin‐Frankfurt‐Münster)-NHL (non-Hodgkin lymphoma)-90 protocol.

Clinical Characteristics and Treatment Outcomes in Children, Adolescents, and Young-adults with Hodgkin's Lymphoma:a KPHOG Lymphoma Working-party, Multicenter, Retrospective Study

Background@#Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. @*Methods@#We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. @*Results@#A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. @*Conclusion@#This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

Epidemiological Study of Hereditary Hemolytic Anemia in the Korean Pediatric Population during 1997–2016: a Nationwide Retrospective Cohort Study

Background@#Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. @*Methods@#We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. @*Results@#A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. @*Conclusion@#In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

Risk Factor Analysis for Secondary Malignancy in Dexrazoxane-Treated Pediatric Cancer Patients / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients. MATERIALS AND METHODS: Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected. RESULTS: Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis. CONCLUSION: Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.

Clinical characteristics and treatment courses for cytomegalovirus-associated thrombocytopenia in immunocompetent children after neonatal period

BACKGROUND: Cytomegalovirus (CMV) causes severe diseases in premature infants and immunocompromised hosts, and antiviral therapy is often required for disease control. However, the clinical manifestations and treatment courses for CMV-associated thrombocytopenia in immunocompetent children are unclear. METHODS: Medical records of the children who suffered from thrombocytopenia, and showed positive CMV polymerase chain reaction and CMV-like symptoms were retrospectively analyzed at three university hospitals in Daegu from January 2000 to March 2017. Patients suffering from leukemia, immunodeficiency, and other infections were excluded. RESULTS: Among 1,065 children with thrombocytopenia, 29 (2.7%) displayed CMV-associated thrombocytopenia. The median age at diagnosis was 15 months and the median platelet count was 26,000/µL. They were classified into the CMV-induced thrombocytopenia (23/29) and CMV-related secondary immune thrombocytopenia (ITP, 6/29) groups. Fourteen subjects had hepatic dysfunction, four had Evans syndrome, two had pneumonitis, and one had gastritis. IVIG was used for 21 patients, and six patients among them showed recurrence, for whom IVIG or antiviral therapy was used. All, except one, recurrent or chronic cases belonged to the CMV-induced thrombocytopenia group. Antiviral therapy was used more frequently for the CMV-induced thrombocytopenia group (8/23, 34.8%) than for the CMV-related secondary ITP group (0/6); however, the results were not statistically significant (P=0.148). CONCLUSION: CMV is a rare but unique etiology of thrombocytopenia, and observed even in healthy children after the neonatal period. About one-third patients need antiviral therapy for disease control. Further, CMV-induced thrombocytopenia is more complex than CMV-related secondary ITP.

Voriconazole plus caspofungin for treatment of invasive fungal infection in children with acute leukemia

BACKGROUND: Invasive fungal infections (IFIs) are a life-threatening problem in immunocompromised patients. Despite timely diagnosis and appropriate antifungal therapy, clinical outcomes of IFIs remain unsatisfactory, necessitating treatment with a combination of antifungal agents. Therefore, childhood leukemic patients treated with voriconazole plus caspofungin were evaluated for the safety and efficacy of the combination antifungal therapy to treat IFIs. METHODS: In this retrospective study, medical records were retrieved for patients admitted to the Pediatric Department of Yeungnam University Hospital, Daegu, South Korea, between April 2009 and May 2013. Medical records of 22 patients were analyzed. RESULTS: Of the 22 patients studied, nine (41%) had been diagnosed with probable IFI, and 13 (59%) with possible IFI. All patients, except one, were already receiving antifungal monotherapy for the treatment of neutropenic fever. After a diagnosis of IFI was confirmed, antifungal monotherapy was replaced with combination therapy. The study's overall response rate was 90.9%, with complete responses in 86.3% of the patients. Two patients experienced a side effect of a small increase in liver enzyme levels. CONCLUSION: Voriconazole plus caspofungin combination therapy is an effective and safe treatment for serious IFI in pediatric patients with acute leukemia.

Myeloablative Hematopoietic Stem Cell Transplantation with a Non-total Body Irradiation Regimen for Treating Pediatric Acute Lymphoblastic Leukemia / 임상소아혈액종양

BACKGROUND: Total body irradiation (TBI) has been traditionally used as a conditioning regimen prior to hematopoietic stem cell transplantation (HSCT) in patients with pediatric leukemia. However, TBI can cause late sequelae such as growth impairment, cataract, hormone abnormalities, infertility, neurocognitive effects, and secondary malignancy in pediatric patients. METHODS: This single center retrospective study included 22 patients with acute lymphoblastic leukemia who were aged <18 years and underwent HSCT between May 1999 and December 2014; seven patients received a TBI-based regimen and 15 received a non-TBI regimen. RESULTS: The overall survival and event-free survival rates in the TBI group were not significantly different from those in the non-TBI group (overall survival rate 71% vs. 73%, respectively; P=0.906; event-free survival rate 71% vs. 73%, respectively P=0.923). CONCLUSION: Our results indicate that non-TBI conditioning regimens can be an alternative treatment option of the treatment of pediatric acute lymphoblastic leukemia undergoing HSCT.

Myeloablative Hematopoietic Stem Cell Transplantation with a Non-total Body Irradiation Regimen for Treating Pediatric Acute Lymphoblastic Leukemia / 임상소아혈액종양

BACKGROUND: Total body irradiation (TBI) has been traditionally used as a conditioning regimen prior to hematopoietic stem cell transplantation (HSCT) in patients with pediatric leukemia. However, TBI can cause late sequelae such as growth impairment, cataract, hormone abnormalities, infertility, neurocognitive effects, and secondary malignancy in pediatric patients.METHODS: This single center retrospective study included 22 patients with acute lymphoblastic leukemia who were aged <18 years and underwent HSCT between May 1999 and December 2014; seven patients received a TBI-based regimen and 15 received a non-TBI regimen.RESULTS: The overall survival and event-free survival rates in the TBI group were not significantly different from those in the non-TBI group (overall survival rate 71% vs. 73%, respectively; P=0.906; event-free survival rate 71% vs. 73%, respectively P=0.923).CONCLUSION: Our results indicate that non-TBI conditioning regimens can be an alternative treatment option of the treatment of pediatric acute lymphoblastic leukemia undergoing HSCT.

A Case of Dasatinib-Induced Nephrotic Syndrome in a Child with Philadelphia Chromosome Positive Acute Lymphoblastic Leukemia

No abstract available.

Kikuchi-Fujimoto disease mimicking malignant lymphoma with 2-18Ffluoro-2-deoxy-D-glucose PET/CT in children / 소아과

PURPOSE: Kikuchi-Fujimoto disease (KFD) is a benign disease, which is characterized by a cervical lymphadenopathy with fever, and it often mimics malignant lymphoma (ML). 2-[18F]fluoro-2-deoxy-D-glucose positron emission tomography/computed tomography (18F-FDG PET/CT) is a powerful imaging modality for the diagnosis, staging and monitoring of ML, with the limitations including the nonspecific FDG uptake in infectious or inflammatory processes. This study compared clinical manifestations and PET/CT findings between KFD and ML patients. METHODS: We retrospectively reviewed the medical records of 23 patients with KFD and 33 patients with ML, diagnosed histopathologically, between January 2000 and May 2013 at the Department of Pediatrics, Yeungnam University Medical Center. Among them, we analyzed the clinical manifestations, laboratory findings and characteristics, and the amount of 18F-FDG uptake between 8 KFD and 9 ML patients who had 18F-FDG PET/CT. RESULTS: The 18F-FDG PET/CT maximum standardized uptake values (SUVmax) ranged from 8.3 to 22.5 (mean, 12.0) in KFDs, and from 5.8 to 34.3 (mean, 15.9) in MLs. There were no significant differences in SUVmax between KFDs and MLs. 18F-FDG PET/CT with ML patients showed hot uptakes in the extranodal organs, such as bone marrow, small bowel, thymus, kidney, orbit and pleura. However, none of the KFD cases showed extranodal uptake (P<0.001). 18F-FDG PET/CT findings of KFD with nodal involvement only were indistinguishable from those of ML. CONCLUSION: Patients who had extranodal involvement on PET/CT were more likely to have malignancy than KFD.

Hereditary hemolytic anemia in Korea from 2007 to 2011: A study by the Korean Hereditary Hemolytic Anemia Working Party of the Korean Society of Hematology

BACKGROUND: The number of patients diagnosed with hereditary hemolytic anemia (HHA) has increased since the advent of novel diagnostic techniques that accurately identify this disorder. Here, we report data from a survey on the prevalence and characteristics of patients diagnosed with HHA in Korea from 2007 to 2011. METHODS: Information on patients diagnosed with HHA in Korea and their clinical and laboratory results were collected using a survey questionnaire. Globin gene and red blood cell (RBC) enzyme analyses were performed. In addition, we analyzed data collected by pediatricians. RESULTS: In total, 195 cases of HHA were identified. Etiologies identified for HHA were RBC membranopathies, hemoglobinopathies, and RBC enzymopathies, which accounted for 127 (64%), 39 (19.9%), and 26 (13.3%) cases, respectively. Of the 39 patients with hemoglobinopathies, 26 were confirmed by globin gene analysis, including 20 patients with beta-thalassemia minor, 5 patients with alpha-thalassemia minor, and 1 patient with unstable hemoglobin disease. CONCLUSION: The number of patients diagnosed with hemoglobinopathies and RBC enzymopathies has increased considerably since the previous survey on HHA in Korea, dated from 1997 to 2006. This is likely the result of improved diagnostic techniques. Nevertheless, there is still a need for more sensitive diagnostic tests utilizing flow cytometry and for better standardization of test results to improve the accuracy of diagnosis of RBC membranopathies in Korea. Additionally, more accurate assays for the identification of RBC enzymopathies are warranted.

Efficacy of pamidronate in children with low bone mineral density during and after chemotherapy for acute lymphoblastic leukemia and non-Hodgkin lymphoma

BACKGROUND: Reduced bone mineral density (BMD) is a significant sequelae in children receiving chemotherapy for acute lymphoblastic leukemia (ALL) and non-Hodgkin lymphoma (NHL). Reduced BMD is associated with an increased risk for fractures. Pamidronate, a second-generation bisphosphonate, has been used to treat osteoporosis in children. This study evaluated the safety and efficacy of pamidronate in children with low BMD during and after chemotherapy for ALL and NHL. METHODS: Between April 2007 and October 2011, 24 children with ALL and NHL were treated with pamidronate. The indication was a decreased BMD Z-score less than -2.0 or bone pain with a BMD Z-score less than 0. Pamidronate was infused at 1 mg/kg/day for 3 days at 1-4 month intervals (pamidronate group, cases). The BMD Z-scores of the cases were compared with those of 10 untreated patients (control group). Lumbar spine BMDs were measured every 6 cycles using dual energy X-ray absorptiometry and Z-scores were calculated. Bone turnover parameters (25-hydroxyvitamin D, alkaline phosphatase, parathyroid hormone, osteocalcin, and type I collagen c-terminal telopeptide) were analyzed. RESULTS: The median cycle of pamidronate treatment was 12. Increases in BMD Z-scores were significantly higher in the pamidronate group than in the control group (P<0.001). BMD (mg/cm2) increased in all pamidronate-treated cases. Twenty patients who complained of bone pain reported pain relief after therapy. The treatment was well tolerated. CONCLUSION: Pamidronate appears to be safe and effective for the treatment of children with low BMD during and after chemotherapy for ALL and NHL.

Red Blood Cell Enzymopathies Causing Hereditary Hemolytic Anemia / 임상소아혈액종양

The RBC enzyme deficiencies causing hereditary hemolytic anemia (HHA) can be divided into three groups: those participating in the glycolytic (E-M) pathway; those involved with the maintenance of a high ratio of reduced to oxidized glutathione; one enzyme in the nucleotide degradation and salvage pathway. Although RBC enzyme deficiencies causing HHA are rare, 3 of the 15 kinds of important and relatively frequently reported enzyme deficiencies such as pyruvate kinase, glucose-6-phosphate-dehydrogenase and pyrimidine-5'-nucleotidase deficiencies are briefly reviewed. The molecular genetics, clinical symptoms, diagnosis and therapeutic approaches of each enzyme deficiencies are summerized. As these enzyme deficiencies are reported throughout the world as well as in Korea with the identification of the mutations, considering a broad spectrum of etiologies for the diagnosis of HHA seems to be warranted.

Red Blood Cell Enzymopathies Causing Hereditary Hemolytic Anemia / 임상소아혈액종양

The RBC enzyme deficiencies causing hereditary hemolytic anemia (HHA) can be divided into three groups: those participating in the glycolytic (E-M) pathway; those involved with the maintenance of a high ratio of reduced to oxidized glutathione; one enzyme in the nucleotide degradation and salvage pathway. Although RBC enzyme deficiencies causing HHA are rare, 3 of the 15 kinds of important and relatively frequently reported enzyme deficiencies such as pyruvate kinase, glucose-6-phosphate-dehydrogenase and pyrimidine-5'-nucleotidase deficiencies are briefly reviewed. The molecular genetics, clinical symptoms, diagnosis and therapeutic approaches of each enzyme deficiencies are summerized. As these enzyme deficiencies are reported throughout the world as well as in Korea with the identification of the mutations, considering a broad spectrum of etiologies for the diagnosis of HHA seems to be warranted.

Novel influenza A (H1N1) 2009 infection in the pediatric patients with hematologic and oncologic diseases in the Yeungnam region / 소아과

PURPOSE: Natural history and consequences of the novel 2009 influenza A H1N1 (2009 H1N1) infection in immunocompromised pediatric patients are not yet fully understood. In this study, we investigated the clinical features and outcomes of the 2009 H1N1 infection in pediatric patients with hematological and oncological diseases. METHODS: We retrospectively reviewed the medical records of 528 patients who had hematological and oncological diseases and who were treated at 7 referral centers located in the Yeungnam region. Among the 528 patients, 27 with definite diagnosis of 2009 H1N1 infection were the subjects of this study. All patients were divided into the following 3 groups: patients who were receiving chemotherapy (group 1), patients who were immunosuppressed due to a non-malignant hematological disease (group 2), and patients who were off chemotherapy and had undergone their last chemotherapy course within 2 years from the influenza A pandemic (group 3). RESULTS: All 28 episodes of 2009 H1N1 infection were treated with the antiviral agent oseltamivir (Tamiflu(R)), and 20 episodes were treated after hospitalization. Group 1 patients had higher frequencies of lower respiratory tract infection and longer durations of fever and hospitalization as compared to those in group 2. Ultimately, all episodes resolved completely with no complications. CONCLUSION: These results suggest that early antiviral therapy did not influence the morbidity or mortality of pediatric patients with hematological and oncological diseases in the Yeungnam region of Korea after the 2009 H1N1 infection. However, no definite conclusions can be drawn because of the small sample size.

Transfusion-Related Acute Lung Injury with Hemophagocytic Lymphohistiocytosis in Aplastic Anemia: Case Report / 임상소아혈액종양

Transfusion-related acute lung injury (TRALI) is a serious clinical syndrome associated with transfusion and has come to be recognized as the leading cause of transfusion-related death recently. TRALI occurs more often in critically ill patients (sepsis, surgery, massive transfusion, cytokine administration) than in a general hospital population, possibly due to the presence of underlying inflammatory conditions that may prime pulmonary neutrophils. We report a case of TRALI developed in a female aplastic anemia patient who presented with a persisting fever for several days. Serologic tests of the patient were consistent with acute EBV infection. As hemophagocytic lymphohistiocytosis developed under septic condition, bicytopenia persisted and the patient needed repeated transfusions. Following transfusion of the blood components, the patient experienced hypotension and a significant change in respiratory status within 6 hours. A chest computed tomography showed newly developed diffuse ground-glass opacities on both lungs. The finding was a non-cardiogenic effect and there was no volume overloading. Anti-neutrophil antibody was detected in serum, and the patient was diagnosed as TRALI. Recurrent lung injury with prolonged pancytopenia caused pulmonary hemorrhage. The patient was managed with mechanical ventilation prior to death.

Excellent Outcomes of Preoperative Chemotherapy and Surgery for Initially Unresectable Hepatoblastoma in Children / 임상소아혈액종양

BACKGROUND: Hepatoblastoma is the most common primary malignant tumor of the liver in children. Complete surgical resection is the treatment of choice for cure. However, only 50% are eligible for resection at diagnosis. Recently combination of preoperative chemotherapy and surgery had led improved resectability and survival rate.METHODS: Between May, 2001 and November, 2010, 9 patients were diagnosed with initially unresectable hepatoblastoma at the department of pediatrics, Yeungnam University Hospital. Medical records were reviewed retrospectively. Initial evaluation included complete blood counts, liver function, serum AFP, cholesterol level and abdominal-CT scan. Preoperative chemotherapy was consisted of cisplatin and doxorubicin every 3-4 weeks. Second-line chemotherapy was cisplatin, vincristine and fluorouracil. The treatment response was analyzed by the Response Evaluation Criteria In Solid Tumors (RECIST) criteria.RESULTS: Among 9 patients, male:female was 4:5. Median age at diagnosis was 12 months (4-59 months). The most common presenting symptom was the abdominal mass. Laboratory findings revealed: median AFP 216,841 ng/dL (3,535-1,036,404 ng/dL), anemia: 4, thrombocytosis: 5, elevated AST/ALT: 8, hyperbilirubinemia: 1 and hypercholesterolemia: 5. The median tumor size was 11 cm (8-15 cm). No patient had metastasis. After median 4 (3-5) cycles of preoperative chemotherapy, all patients(100%) showed a partial response and underwent complete surgical resection. Postoperative chemotherapy was given for median 4 (3-5) cycles. The median follow up was 34 months (6-117 months) and all patients are surviving without events.CONCLUSION: Although this study includes limited number of cases, preoperative intensive chemotherapy and surgery for initially unresectable hepatoblastoma in children resulted in excellent outcomes.

Excellent Outcomes of Preoperative Chemotherapy and Surgery for Initially Unresectable Hepatoblastoma in Children / 임상소아혈액종양

BACKGROUND: Hepatoblastoma is the most common primary malignant tumor of the liver in children. Complete surgical resection is the treatment of choice for cure. However, only 50% are eligible for resection at diagnosis. Recently combination of preoperative chemotherapy and surgery had led improved resectability and survival rate. METHODS: Between May, 2001 and November, 2010, 9 patients were diagnosed with initially unresectable hepatoblastoma at the department of pediatrics, Yeungnam University Hospital. Medical records were reviewed retrospectively. Initial evaluation included complete blood counts, liver function, serum AFP, cholesterol level and abdominal-CT scan. Preoperative chemotherapy was consisted of cisplatin and doxorubicin every 3-4 weeks. Second-line chemotherapy was cisplatin, vincristine and fluorouracil. The treatment response was analyzed by the Response Evaluation Criteria In Solid Tumors (RECIST) criteria. RESULTS: Among 9 patients, male:female was 4:5. Median age at diagnosis was 12 months (4-59 months). The most common presenting symptom was the abdominal mass. Laboratory findings revealed: median AFP 216,841 ng/dL (3,535-1,036,404 ng/dL), anemia: 4, thrombocytosis: 5, elevated AST/ALT: 8, hyperbilirubinemia: 1 and hypercholesterolemia: 5. The median tumor size was 11 cm (8-15 cm). No patient had metastasis. After median 4 (3-5) cycles of preoperative chemotherapy, all patients(100%) showed a partial response and underwent complete surgical resection. Postoperative chemotherapy was given for median 4 (3-5) cycles. The median follow up was 34 months (6-117 months) and all patients are surviving without events. CONCLUSION: Although this study includes limited number of cases, preoperative intensive chemotherapy and surgery for initially unresectable hepatoblastoma in children resulted in excellent outcomes.

Transfusion-Related Acute Lung Injury with Hemophagocytic Lymphohistiocytosis in Aplastic Anemia: Case Report / 임상소아혈액종양

Transfusion-related acute lung injury (TRALI) is a serious clinical syndrome associated with transfusion and has come to be recognized as the leading cause of transfusion-related death recently. TRALI occurs more often in critically ill patients (sepsis, surgery, massive transfusion, cytokine administration) than in a general hospital population, possibly due to the presence of underlying inflammatory conditions that may prime pulmonary neutrophils. We report a case of TRALI developed in a female aplastic anemia patient who presented with a persisting fever for several days. Serologic tests of the patient were consistent with acute EBV infection. As hemophagocytic lymphohistiocytosis developed under septic condition, bicytopenia persisted and the patient needed repeated transfusions. Following transfusion of the blood components, the patient experienced hypotension and a significant change in respiratory status within 6 hours. A chest computed tomography showed newly developed diffuse ground-glass opacities on both lungs. The finding was a non-cardiogenic effect and there was no volume overloading. Anti-neutrophil antibody was detected in serum, and the patient was diagnosed as TRALI. Recurrent lung injury with prolonged pancytopenia caused pulmonary hemorrhage. The patient was managed with mechanical ventilation prior to death.

Reduced-dose craniospinal radiotherapy followed by high-dose chemotherapy and autologous stem cell rescue for children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuroectodermal tumor

BACKGROUND: In this study, we investigated the effects of reduced-dose craniospinal radiotherapy (CSRT) followed by tandem high-dose chemotherapy (HDCT) with autologous stem cell rescue (ASCR) in children with a newly diagnosed high-risk medulloblastoma (MB) or supratentorial primitive neuroectodermal tumor (sPNET). METHODS: Between March 2005 and April 2007, patients older than 3 years with a newly diagnosed high-risk MB or sPNET were enrolled. The patients received two cycles of pre-RT chemotherapy consisting of cisplatin, etoposide, vincristine, and cyclophosphamide (cycle A), and carboplatin, etoposide, vincristine, and ifosphamide (cycle B), followed by CSRT with 23.4 Gy and local RT with 30.6 Gy. After four cycles of post-RT chemotherapy (cycles A, B, A, and B), tandem double HDCT with ASCR was performed. RESULTS: A total of 13 patients (MB=11, sPNET=2) were enrolled. Of these, one patient progressed, one patient died of septic shock after the second cycle of B, and one patient relapsed after the third cycle of B. The 3-year event-free survival (EFS) rate of the patients intended for HDCT was 76.9%, whereas the 3-year EFS rate of the patients who received HDCT was 100%. No treatment-related mortality occurred during HDCT. CONCLUSION: Although the follow-up period was short and the patient cohort was small in size, the results of this study are encouraging. The limited toxicity and favorable EFS rate observed in children treated with reduced-dose CSRT followed by HDCT and ASCR warrant further exploration in a larger study population.