[Show abstract][Hide abstract] ABSTRACT: Transformation of MDS into ALL during childhood is extremely rare. We report a rare case of an 8-yr-old girl who presented with refractory cytopenia of childhood (RCC) that transformed into ALL only 3 months after the diagnosis of childhood MDS. Although no cytogenetic abnormalities were observed in conventional karyotype and FISH analysis, we found several deletions on chromosomes 5q, 12q, 13q, and 22q. Partial homozygous deletion of the RB1 gene was observed on microarray analysis, with the bone marrow specimen diagnosed as ALL. This is the first case report of transformation of ALL from childhood MDS in Korea. We also compared the clinical, cytological, and cytogenetic features of 4 previously reported childhood MDS cases that transformed into ALL.
Annals of Laboratory Medicine 03/2013; 33(2):130-135. DOI:10.3343/alm.2013.33.2.130 · 1.48 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Although osteomyelitis is a very rare complication of Bacillus Calmette-Guérin (BCG) vaccination, sternal osteomyelitis as a late complication of BCG vaccination diagnosed by polymerase chain reaction (PCR) in a child is described.We might consider BCG osteomyelitis in the case of osteomyelitis without bacterial isolation within a year after BCG vaccination, the absence of pulmonary foci, and a contact to the patient with tuberculosis.
The Thoracic and Cardiovascular Surgeon 01/2013; 62(3). DOI:10.1055/s-0032-1331897 · 0.98 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Knowledge of the roles of tacrolimus and minidose methotrexate (MTX) in the prevention of acute graft-versus-host disease (aGVHD) in pediatric allogeneic hematopoietic stem cell transplantation (HSCT) is limited. We retrospectively evaluated the engraftment status, incidence of aGVHD and chronic GVHD (cGVHD), and toxicities of tacrolimus and minidose MTX in aGVHD prophylaxis in children undergoing allogeneic HSCT.
Seventeen children, who underwent allogeneic HSCT and received tacrolimus and minidose MTX as GVHD prophylaxis from March 2003 to February 2011, were reviewed retrospectively. All the patients received tacrolimus since the day before transplantation at a dose of 0.03 mg/kg/day and MTX at a dose of 5 mg/m(2) on days 1, 3, 6, and 11.
Of the 17 patients, 9 received human leukocyte antigen (HLA)-matched related donor transplants, and 8 received HLA-matched, or partially mismatched unrelated donor transplants. The median time for follow-up was 55 months. The incidence of aGVHD in the related and unrelated donor groups was 22.2% and 42.9%, respectively. cGVHD was not observed. To maintain therapeutic blood levels of tacrolimus, the younger group (<8 years of age) required an increased mean dose compared to the older group (≥8 years) (P=0.0075). The adverse events commonly associated with tacrolimus included hypomagnesemia (88%), nephrotoxicity (23%), and hyperglycemia (23%).
Tacrolimus and minidose MTX were well tolerated and effective in GVHD prophylaxis in pediatric patients undergoing allogeneic HSCT. Children <8 years of age undergoing HSCT required increased doses of tacrolimus to achieve therapeutic levels.
The Korean journal of hematology 06/2012; 47(2):113-8. DOI:10.5045/kjh.2012.47.2.113
[Show abstract][Hide abstract] ABSTRACT: We report the outcome of 236 pediatric umbilical cord blood transplantations (UCBT) performed in Korea. Given that the sources of the grafts were mostly unrelated donors (n = 226; 95.8%), only the results of unrelated UCBT were included for all statistics. The most frequent primary disease was acute leukemia (n = 167). In total, 91.7% of recipients were seropositive for cytomegalovirus (CMV). The median doses of nucleated cells and CD34+ cells were 4.84 × 10(7)/kg and 2.00 × 10(5)/kg, respectively. The median times to neutrophil (>0.5 × 10(9)/L) and platelet recovery (>20 × 10(9)/L) were 18 and 45 days, respectively. Grade 2-4 acute graft-versus-host-disease (GVHD) and chronic GVHD developed in 41.1 and 36.1% of cases, respectively. Forty-five patients developed CMV disease. The 5-year overall and event-free survival were 47.5 and 36.9%, respectively. Multivariate analysis revealed that adverse factors for survival of the whole cohort were total body irradiation-based conditioning (P = 0.007), salvage transplant (P = 0.001), failure to achieve early complete chimerism (P < 0.0005), and CMV disease (P = 0.001). The outcomes of the single- and double-unit UCBT (n = 64) were similar, while double-unit recipients were heavier (P < 0.0005) and older (P < 0.0005). We conclude that double-unit UCBT is a reasonable option for older or heavier children and that the thorough surveillance of CMV infection and the development of an effective CMV therapeutic strategy may be especially important for Korean children, whose CMV seroprevalence exceeds 90%.
American Journal of Hematology 01/2011; 86(1):12-7. DOI:10.1002/ajh.21886 · 3.80 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Apert syndrome is a rare congenital anomaly characterized by craniofacial malformations and severe symmetrical syndactyly of fingers and toes. This syndrome is caused by a genetic mutation; the S253 mutation is common, though the P253R mutation is not as frequent. Common symptoms include skeletal malformations, poor joint mobility, eye and ear problems, cleft palate, and orthodontic and other dental problems. We report a case of an infant with the common morphological features of Apert syndrome. Interestingly, she was found to have the P253R mutation in FGFR2 exon VIII, which has been less commonly observed in Korea. A brief review of the literature is included.
[Show abstract][Hide abstract] ABSTRACT: A paradoxical tuberculosis (TB) reaction is defined as the clinical or radiological worsening of pre-existing TB lesions or the development of new lesions during treatment. We treated a case of such a paradoxical reaction in a 21-day-old female infant who was diagnosed with congenital TB and was being treated with antituberculous drugs. The paradoxical reaction improved after additional treatment with corticosteroids. Therefore, corticosteroids might be useful to control paradoxical reactions in patients with congenital tuberculosis.
International journal of infectious diseases: IJID: official publication of the International Society for Infectious Diseases 02/2009; 13(5):e279-81. DOI:10.1016/j.ijid.2008.11.005 · 1.86 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: There have recently been some reports suggesting that once-daily intravenous busulfan (IV Bu) as a conditioning regime for hematopoietic stem cell transplantation (HSCT) possibly reduces the toxicities without influencing the clinical outcome as compared with the traditional 4 times daily dosage schedule. We report here on the clinical outcome of once-daily IV Bu as a conditioning regime for HSCT in children with AML at a single treatment center.
The Korean journal of hematology 01/2009; 44(1). DOI:10.5045/kjh.2009.44.1.1
[Show abstract][Hide abstract] ABSTRACT: The aim of this study was to investigate the incidence and course of neutropenia following intravenous immunoglobulin (IVIG) therapy in children with idiopathic thrombocytopenic purpura (ITP).
Korean Journal of Pediatrics 01/2008; 51(2). DOI:10.3345/kjp.2008.51.2.204
[Show abstract][Hide abstract] ABSTRACT: Thymic hyperplasia results from thymic regrowth after atrophy during stressful conditions such as burns, surgery, infection, and chemotherapy. Although thymic lesions are relatively common causes of anterior mediastinal masses, they also can develop in other mediastinal compartments on rare occasions. It is well known that thymic tissue can develop in ectopic intrathoracic lesions. Few cases of ectopic thymus associated with disturbance during thymus embryogenesis have been reported as incidental findings. We report the case of a 4-year-old boy with true thymic hyperplasia from an ectopic thymus after successful treatment for Burkitt lymphoma. This is a rare finding in the differential diagnosis of a middle mediastinal mass in a child following chemotherapy for lymphoma. The diagnosis of thymic hyperplasia from an ectopic thymus can be confirmed only histologically.