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.

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.