Seung Yeon Kwon

Posterior reversible encephalopathy syndrome in childhood with hematologic/oncologic diseases.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-neuroradiologic disease entity represented by characteristic magnetic resonance image (MRI) findings of subcortical/cortical hyperintensity in T2-weighted sequences, more often observed in parieto-occipital lobes, accompanied by clinical neurologic alterations. PRES is a rare central nervous system complication in childhood hematologic-oncologic patients and shows very different neurologic symptoms between patients, from numbness on extremities to generalized seizure. The etiology of PRES was not well known until these days. In this study, 8 patients with PRES were reviewed, retrospectively. There were 4 patients with acute lymphocytic leukemia, 1 with aplastic anemia, and 3 with solid tumors (1 patient each for neuroblastoma, Ewing sarcoma, and osteosarcoma). Allogeneic stem cell transplantation was performed in 2 patients. Immunosuppressive agents such as tacrolimus and cyclosporine A were used in 3 patients. One neuroblastoma patient was in immediate postoperative status. All patients experienced seizure attacks of different types and showed typical MRI findings. Follow-up MRIs revealed significant improvements. From this review, we might consider chemotherapy and surgery as additive causes for PRES other than immunosuppressive agents. Therefore, careful examination of the patients receiving chemotherapy and surgery was needed to find out this uncommon but good prognostic complication.

Autologous peripheral blood stem cell transplantation in children with non-Hodgkin’s lymphoma: a report from the Korean society of pediatric hematology-oncology

Recent development of stratified chemotherapeutic regimens has rapidly improved the survival rate of non-Hodgkin’s lymphoma (NHL) of childhood. Despite these improvements, the outcome for children with recurrent or refractory NHL remains dismal. We explored the use of high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (HDC/PBSCT) for children with either refractory or recurrent NHL, and we evaluated various factors influencing outcome of HDC/PBSCT. Thirty-three patients underwent HDC/PBSCT in 11 institutes were enrolled. All patients had refractory or recurrent NHL. Sex, stage at diagnosis, histologic subtype (lymphoblastic, Burkitt’s, and large-cell lymphoma), LDH level at diagnosis, disease status at transplantation, and preparative regimens for HDC/PBSCT were explored. In regard to the patients, six had Burkitt’s lymphoma, 13 had lymphoblastic lymphoma, and 14 had large-cell lymphoma. The 2-year event-free survival (EFS) was 59.1±9.3%. The EFS for Burkitt’s, lymphoblastic, and large-cell lymphoma was 66.7±27.2, 50.5±14.8, and 82.1±11.7%, respectively. In comparison with lymphoblastic and non-lymphoblastic lymphoma, the relative risk for lymphoblastic lymphoma was higher than the others (P=0.037). EFS between anaplastic large-cell and diffuse large-cell lymphoma was 100 and 55.6±24.9%, respectively (P=0.106). Status at transplantation was the most predictive factor for the survival after HDC/PBSCT (EFS for CR 70.8±9.5% vs non-CR 20.0±17.9%, P=0.008). Transplantation-related complications were minimal, and infection was the most prevalent complication. HDC/PBSCT is considered applicable to recurrent or refractory pediatric NHL patients safely and it could replace conventional chemotherapy. In this study, children with CR status at the time of HDC/PBSCT showed higher survival rate. However, refractory or recurrent lymphoblastic lymphoma patients showed dismal results. Therefore, new therapeutic modalities may be needed for this group of NHL patients.

Two Cases of Wernicke's Encephalopathy in Young Age Patients Receiving Allogeneic Hematopoietic Stem Cell Transplantation

Wernickes encephalopathy is an acute neurolopsychiatric syndrome caused by thiamine deficiency, and classically presents with the triad of opthalmopathy, ataxia and altered mentality. Both prolonged total parenteral nutrition and reduced oral intake can induce Wernickes encephalopathy during hematopoietic stem cell transplantation (HSCT). Although early treatment is important for recovery from Wernickes encephalopathy, the vague symptoms and characteristics hinder early diagnosis. Furthermore, Wernickes encephalopathy is not infrequent and can develop at any age during HSCT. Herein, we present two young patients developing Wernickes encephalopathy during HSCT.

FEASIBILITY OF SEQUENTIAL HIGH-DOSE CHEMOTHERAPY IN ADVANCED PEDIATRIC SOLID TUMORS

The purpose of this study was to evaluate the feasibility and tumor response of 3 cycles of sequential high-dose chemotherapy (HDCT) in advanced pediatric solid tumor patients. Medical records of 11 children who underwent 2 consequent courses of reduced conditioning HDCT followed by final HDCT with autologous HSC infusion were reviewed in a retrospective manner. Each median time to an absolute neutrophil count > 0.5 × 109/L was 12, 13, and 12 days. Major toxic reactions were fever, infection, and vomiting. One patient experienced transplantation-related mortality. Nine patients showed complete and partial responses to the therapy at 6 months follow-up after final HDCT. Finally, 6 patients are alive without evidence of disease at median follow-up of 24 months. Even though it is a preliminary result, the authors think that this treatment could be a feasible treatment option for advanced pediatric solid tumor patients.

Acute interstitial pneumonia in siblings: a case report.

Acute interstitial pneumonia (AIP) is a rapidly progressive condition of unknown cause that occurs in a previously healthy individual and produces the histologic findings of diffuse alveolar damage. Since the term AIP was first introduced in 1986, there have been very few case reports of AIP in children. Here we present a case of AIP in a 3-yr-old girl whose other two siblings showed similar radiologic findings. The patient was confirmed to have AIP from autopsy showing histological findings of diffuse alveolar damage and proliferation of fibroblasts. Her 3-yr-old brother was also clinically and radiologically highly suspected as having AIP, and the other asymptomatic 8-yr-old sister was radiologically suspected as having AIP.

A Good Outcome for a Case of Chronic Pneumonitis of Infancy

Chronic pneumonitis of infancy (CPI) is a very rare interstitial lung disease. Its pathological features differ from other types of interstitial pneumonia that occur in adults and children. The mortality rate of CPI is high, even with treatment. We report a case of a 3 month old girl diagnosed with CPI after an open lung biopsy who improved after proper treatment.