Survival Benefit of Hematopoietic Stem Cell Transplantation in Infant Acute Lymphoblastic Leukemia, According to Classification of High Risk Group / 임상소아혈액종양

BACKGROUND: In comparison to older children, acute leukemia in infants has a dismal outcome, despite introduction of intensive multi-agent chemotherapy. Patients with age under 1 year and mixed-lineage leukemia (MLL) gene rearrangements are the most high risk group. In this study, we investigate the outcome of more intensive chemotherapy and the role of hematopoietic stem cell transplantation (HSCT) in high risk group of infant leukemia.METHODS: This study analyzed on 9 infants with acute lymphoblastic leukemia (ALL) who were diagnosed and treated between 1998 and 2008 at Severance hospital. We classified high risk group with age at diagnosis is under 6 months or white blood cell at diagnosis are over 50,000/microL or presence of MLL gene rearrangements. Patients in high risk group were treated on a protocol of intensive chemotherapy followed by HSCT.RESULTS: In this study, the 3-years EFS rate for 9 infant ALL patients was 66.7+/-15.7%. Both of 2 patients younger than 6 months of age at diagnosis did not survive. 3yr-EFS rate for the 7 patients categorized in high risk group out of 9 infant ALL patients was 57.1+/-18.7%, and 3 yr EFS of the 6 patients in the high risk group who received HSCT was 60+/-21.9%. We did not experience relapse in 6 patients treated with HSCT.CONCLUSION: This study showed promising results and lesser risks in high risk group infant ALL treated with intensive chemotherapy and HSCT. We suggest early HSCT with intensive chemotherapy for infant leukemia patients in high risk group.

Survival Benefit of Hematopoietic Stem Cell Transplantation in Infant Acute Lymphoblastic Leukemia, According to Classification of High Risk Group / 임상소아혈액종양

BACKGROUND: In comparison to older children, acute leukemia in infants has a dismal outcome, despite introduction of intensive multi-agent chemotherapy. Patients with age under 1 year and mixed-lineage leukemia (MLL) gene rearrangements are the most high risk group. In this study, we investigate the outcome of more intensive chemotherapy and the role of hematopoietic stem cell transplantation (HSCT) in high risk group of infant leukemia. METHODS: This study analyzed on 9 infants with acute lymphoblastic leukemia (ALL) who were diagnosed and treated between 1998 and 2008 at Severance hospital. We classified high risk group with age at diagnosis is under 6 months or white blood cell at diagnosis are over 50,000/microL or presence of MLL gene rearrangements. Patients in high risk group were treated on a protocol of intensive chemotherapy followed by HSCT. RESULTS: In this study, the 3-years EFS rate for 9 infant ALL patients was 66.7+/-15.7%. Both of 2 patients younger than 6 months of age at diagnosis did not survive. 3yr-EFS rate for the 7 patients categorized in high risk group out of 9 infant ALL patients was 57.1+/-18.7%, and 3 yr EFS of the 6 patients in the high risk group who received HSCT was 60+/-21.9%. We did not experience relapse in 6 patients treated with HSCT. CONCLUSION: This study showed promising results and lesser risks in high risk group infant ALL treated with intensive chemotherapy and HSCT. We suggest early HSCT with intensive chemotherapy for infant leukemia patients in high risk group.

Acute Epididymitis in Two Children with Henoch-Schonlein Purpura

Scrotal involvement has been reported from 2% to 38% of males with Henoch-Schonlein purpura (HSP). It may present before the rash occurs or even long time after it has disappeared. Scrotal involvement of HSP usually results in pain, tenderness, swelling or discoloration of scrotum. Scrotal pain sometimes mimics testicular torsion to various degrees, which can be followed by unnecessary operation. In our 2 cases, one was a 5-year and 11-month-old boy who came to our emergency department due to scrotal pain before the diagnosis of HSP, and the other was a 5-year and 1-month-old boy who came to the outpatient clinic due to scrotal pain after the resolution of HSP about 1 month before the visit. We performed Doppler ultrasonography (USG) to evaluate the acute scrotal pain in the two boys. On Doppler USG, epididymis showed increased blood flow, and testis showed normal blood flow. These findings enabled the diagnosis of acute epididymitis and excluded testicular torsion. Epididymitis was improved by conservative management including shortterm steroid therapy within 5 days. It is important to perform adequate evaluation with tools such as Doppler USG in the early course of acute scrotum of HSP, to avoid unnecessary scrotal exploration.