Building of Pediatric COVID-19 Module Clinic: A Novel Operation Model in Response to COVID-19 Pandemic

In mid-2022, as the wave of pediatric coronavirus disease 2019 (COVID-19) cases escalated in South Korea, a public-private partnership was made to establish a Pediatric COVID-19 Module Clinic (PMC). We describe the utilization of the first prototype children’s modular clinic in Korea University Anam Hospital functioning as the COVID-19 PMC. Between August 1 and September 30, 2022, a total of 766 children visited COVID-19 PMC. Daily number of patient visits to the COVID-19 PMC ranged between 10 and 47 in August; and less than 13 patients per day in September 2022. Not only the model provided timely care for the COVID-19 pediatric patients, but it also enabled safe and efficacious care for the non-COVID-19 patients in the main hospital building while minimizing exposure risk to severe acute respiratory syndrome coronavirus 2 transmission. Current description highlights the importance of spatial measures for mitigating in-hospital transmission of COVID-19, in specifically on pediatric care.

Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. @*Materials and Methods@#From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. @*Results@#Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). @*Conclusion@#The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

Kikuchi-Fujimoto Disease Mimicking Mesenteric Lymphadenitis in Children:A Case Report and Systematic Review

Kikuchi-Fujimoto disease (KFD) is an acute febrile disease that mainly involves histiocytic necrotizing lymphadenitis in children and young adults. Diagnosis of KFD is even more difficult if image-guided percutaneous biopsy is technically challenging. We present a case of clinically diagnosed KFD in an 11-year-old boy who presented with fever, abdominal pain, and mesenteric lymphadenopathy, resulting in a diagnostic challenge. Additionally, we conducted a systematic review, and our goal was to describe the spectrum of disease, therapy, and outcomes. We identified 15 cases of KFD with symptoms that mimicked mesenteric lymphadenitis. Reports from the Americas, Europe, and Asia were also included. Most patients were male, exhibited leukopenia and elevated inflammatory markers, and recovered without significant sequelae or complications. A high index of suspicion of KFD should be maintained in children presenting with prolonged fever and unusual manifestations, such as mesenteric lymphadenitis.

Neonatal Risk Factors for Growth Retardation in Infants With Congenital Heart Disease

Background@#While the association of congenital heart disease (CHD) and growth retardation (GR) is known, data remain limited. This study investigated the incidence of GR and its neonatal risk factors in patients with CHD using nationwide population-based claims data.Method: The study population was extracted from Korean National Health Insurance Service claims data from January 2002 to December 2020. We included patients diagnosed with CHD under one year of age. GR was defined as an idiopathic growth hormone deficiency or short stature on the claims data. We investigated the neonatal risk factors for GR. @*Results@#The number of patients diagnosed with CHD within the first year of birth was 133,739. Of these, 2,921 newborns were diagnosed with GR. The cumulative incidence of GR was 4.8% at 19 years of age for individuals diagnosed with CHD at infancy. In the multivariable analysis, the significant risk factors for GR were preterm birth, small for gestational age, low birth weight, respiratory distress, bronchopulmonary dysplasia, bacterial sepsis, necrotizing enterocolitis, feeding problems and cardiac procedure. @*Conclusion@#Several neonatal conditions were significant risk factors for GR in CHD patients, and appropriate monitoring and treatment programs are required in CHD neonates with these factors. Considering this study is limited to claims data, further studies are warranted, including genetic and environmental factors affecting GR in CHD patients.

Treatment Experience for a Child with Ewing Sarcoma who Refused to Receive Blood Transfusions for Religious Reasons as a Jehovah’s Witness / 임상소아혈액종양

Ewing sarcoma is a solid tumor involving the bone and/or surrounding soft tissue, which requires multidisciplinary treatment, primarily through a combination of surgery and chemotherapy and, in some cases, radiation therapy. We present the case of a patient who was diagnosed with stage I Ewing sarcoma without metastasis who had a lesion in one of the left 3rd metacarpal bones. The patient’s family members were active Jehovah’s Witnesses; therefore, the patient’s guardian refused blood transfusions for religious reasons. The medical staff explained the fatal side effects of pancytopenia after chemotherapy, and when life-threatening anemia, thrombocytopenia, and neutropenia occurred, the patient’s guardian agreed that the intensity of chemotherapy needed to be either reduced or stopped. The patient’s treatment regimen was as follows: VDC (vincristine, doxorubicin, cyclophosphamide) and IE (ifosfamide, etoposide) were alternately administered as neoadjuvant chemotherapy, followed by complete tumor excision and more chemotherapy. As red blood cell transfusion was impossible, only erythropoietin (EPO; EpokineⓇ, HK inno.N, Seoul, Korea) and granulocyte colony-stimulating factor (G-CSF; GrasinⓇ , Kyowa Kirin, Seoul, Korea) were administered as adjuvant therapy. During the course of treatment, hemoglobin levels decreased to as low as 6.7 g/dL; however, treatment without transfusion was possible with the administration of EPO and G-CSF, with minimal changes in the chemotherapy schedule. With 6 months follow-up post-treatment, the patient is still alive with no relapses observed on positron emission tomography-CT and bone scans.

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.

Loss of Heterozygosity at Chromosome 16q Is a Negative Prognostic Factorin Korean Pediatric Patients with Favorable Histology Wilms Tumor:A Report of the Korean Pediatric Hematology Oncology Group (K-PHOG) / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Loss of heterozygosity (LOH) at chromosomes 1p and 16q is a poor prognostic factor infavorable histology Wilms tumor (FHWT). This study investigated the prevalence of LOH at1p and 16q and evaluated its prognostic value in Korean children with FHWT. @*Materials and Methods@#We analyzed 101 FHWT patients who were diagnosed between 1996 and 2016 in KoreanSociety of Pediatric Hematology Oncology Group hospitals. Using paraffin-embedded kidneytissue samples sent from each center, we reviewed LOH at 1p and 16q in each patient andassessed the prognostic value of LOH status for clinical parameters affecting event-freesurvival (EFS). @*Results@#Of the 101 patients, 12 (11.9%) experienced recurrence; the 3-year EFS was 87.6%. LOHat 1p or 16q was detected in 19 patients (18.8%), with five having LOH at both 1q and 16q.The frequency of LOH at 1p was higher among younger patients (p=0.049), but there wasno difference in LOH prevalence according to tumor stage. In the multivariate analysis, LOHat 16q was a significant negative prognostic factor affecting EFS (3-year EFS, 73.7% vs.91.1%; hazard ratio, 3.95; p=0.037), whereas LOH at 1p was not (p=0.786). @*Conclusion@#LOH at 16q was a significant negative prognostic factor affecting outcome in Korean pediatricFHWT patients. Due to the small sample size of this study, large-scale multicenter trialsare warranted to investigate the prognostic value of LOH at 1p and 16q in Korean childrenwith FHWT.

A Case of Recurrent Rosai-Dorfman Disease Successfully Treated with 2-Chlorodeoxyadenosine (Cladribine) / 임상소아혈액종양

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.

Successful engraftment after infusion of multiple low doses of CD34+ cells from a poorly matched sibling donor in a patient with severe aplastic anemia / 영남의대학술지

The dose of CD34+ cells is known to influence the outcome of allogeneic peripheral blood stem cell (PBSC) and/or T-cell-depleted transplantation. A previous study proposed that 2×10⁶ CD34+ cells/kg is the ideal minimum dose for allogeneic transplantation, although lower doses did not preclude successful therapy. In the case we present here, CD34+ cells were collected from a matched sibling donor on the day of allogeneic hematopoietic stem cell transplantation; however, the number of cells was not sufficient for transplantation. Consequently, PBSCs were collected three additional times and were infused along with cord blood cells from the donor that were cryopreserved at birth. The cumulative dose of total nuclear cells and CD34+ cells was 15.9×10⁸ cells/kg and 0.95×10⁶ cells/kg, respectively. White blood cells from this patient were engrafted on day 12. In summary, we report successful engraftment after infusion of multiple low doses of CD34+ cells in a patient with severe aplastic anemia.

Successful engraftment after infusion of multiple low doses of CD34+ cells from a poorly matched sibling donor in a patient with severe aplastic anemia / 영남의대학술지

The dose of CD34+ cells is known to influence the outcome of allogeneic peripheral blood stem cell (PBSC) and/or T-cell-depleted transplantation. A previous study proposed that 2×10⁶ CD34+ cells/kg is the ideal minimum dose for allogeneic transplantation, although lower doses did not preclude successful therapy. In the case we present here, CD34+ cells were collected from a matched sibling donor on the day of allogeneic hematopoietic stem cell transplantation; however, the number of cells was not sufficient for transplantation. Consequently, PBSCs were collected three additional times and were infused along with cord blood cells from the donor that were cryopreserved at birth. The cumulative dose of total nuclear cells and CD34+ cells was 15.9×10⁸ cells/kg and 0.95×10⁶ cells/kg, respectively. White blood cells from this patient were engrafted on day 12. In summary, we report successful engraftment after infusion of multiple low doses of CD34+ cells in a patient with severe aplastic anemia.

Rare Concurrence of Congenital Muscular Torticollis and a Malignant Tumor in the Same Sternocleidomastoid Muscle

While congenital muscular torticollis (CMT) can occur along with other conditions, such as clavicle fracture or brachial plexus injury, these conditions exist outside the sternocleidomastoid muscle (SCM). We present a rare case with concurrence of CMT and a malignant tumor inside the same SCM, along with serial clinical and radiological findings of the atypical features of CMT. The malignant tumor was in fact a low-grade fibromyxoid sarcoma. To the best of our knowledge, the current case is the first of a concurrent condition of CMT inside the SCM. This case suggests that concurrent conditions could exist either inside or outside the SCM with CMT. Therefore, a thorough evaluation of SCM is required when subjects with CMT display atypical features, such as the increase of mass or poor response to conservative therapy. In that case, appropriate imaging modalities, such as ultrasonogram or magnetic resonance imaging, are useful for differential diagnosis.

A Study of Central Diabetes Insipidus in Head and Neck Langerhans Cell Histiocytosis: A Single Center Experience / 임상소아혈액종양

BACKGROUND: Langerhans cell histiocytosis (LCH) frequently involves the head and neck and increases the risk of central nervous system (CNS) involvement of LCH, such as central diabetes insipidus (CDI), when the craniofacial bones are involved. We analyzed risk factors and clinical features of CDI among patients with LCH involving the head and neck. METHODS: From January 1, 2000 to May 1, 2018, 63 patients with histologically confirmed LCH in the Department of Pediatrics, Ajou University Hospital were retrospectively analyzed. RESULTS: Forty eight cases (76.2%) of patients had head and neck involvement, and 9 cases (14.3%) in craniofacial bones at the time of initial diagnosis of LCH. CDI was found in 6 cases (9.5%) among all LCH patients, 6 cases (12.2%) among patients with head and neck involvement, and 3 cases (33.3%) among patients with craniofacial bone involvement. Three cases of CDI occurred at the time of initial LCH diagnosis, and another 3 cases occurred at the time of 2, 4, and 8 years after initial LCH diagnosis. Of the 6 CDI patients, 3 had CNS risk lesions and 3 had no CNS risk lesions, but all had multi-system involvement of LCH. CONCLUSION: CDI can occur even in patients with head and neck LCH without CNS risk lesions, if there are multisystem involvement of LCH. Patients with head and neck LCH may develop CDI over time, so continuous observations should be done while considering the occurrence of CDI.

Association Kikuchi disease with Hashimoto thyroiditis: a case report and literature review

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disease characterized by fever and lymphadenopathy. The etiology of KFD is unknown, but an autoimmune cause has been suggested. Hashimoto thyroiditis is the most common autoimmune thyroid disorder in children and is known to be associated with other autoimmune diseases. Only a few cases of Hashimoto thyroiditis associated with KFD have been documented. We report a case of a 16-year-old girl who was first diagnosed with KFD and developed Hashimoto thyroiditis 2 years and 6 months later during her follow-up period. Physicians of patients with KFD should consider the possibility of autoimmune diseases like Hashimoto’s thyroiditis.

Clinical Characteristics of Severe Neutropenia in Children / 임상소아혈액종양

BACKGROUND: Severe neutropenia is defined as an absolute neutrophil count (ANC) less than 0.5×109/L, which is known to increase the risk of serious bacterial infections. The aim of this study was to investigate characteristics, etiology and differences between transient and chronic severe neutropenia in children.METHODS: 204 children, who were diagnosed with severe neutropenia at the Ajou University Hospital during a 5-year period, were included in the study. Clinical and laboratory features were analyzed. The patients were classified as having transient severe neutropenia (TSN) if recovery occurred within 6 months of diagnosis, and chronic severe neutropenia (CSN) if the neutropenia persisted for 6 months or more.RESULTS: 184 (90.2%) patients with TSN and 20 (9.8%) patients with CSN were identified. Most of the TSN occurred in patients less than 2 year of age (75.5%) and rarely occurred in patients 5 years or older (5.4%). The most common cause of TSN was infection-related neutropenia (82.6%), and most of the associated infections were respiratory infections (44.6%). Compared to TSN, CSN patients were younger at diagnosis (1.00 vs. 0.71, P < 0.001), had a lower ANC at diagnosis (364.8 vs. 214.9, P < 0.001), lower ANC at nadir (356.0 vs. 50.0, P < 0.001), and higher platelet count (188×10⁹ vs. 308×10⁹, P < 0.001), monocyte count (491.5×10⁶ vs. 832.9×10⁶, P=0.010) and CRP (0.22 vs. 0.85, P=0.036).CONCLUSION: Most of the severe neutropenia occurred in children younger than 2 years of age, and virus infection was the most common cause of TSN.

Differences in Clinical Characteristics of Transient Myeloproliferative Disease and Leukemia in Down Syndrome: A Single University Hospital Study / 임상소아혈액종양

BACKGROUND: Children with Down syndrome (DS) have a 10- to 20-fold increased risk of developing leukemia. However, in some patients, leukemia does not become apparent despite significant number of blast cells in the peripheral blood. This condition is called Transient myeloproliferative disorder (TMD), and is a disease entity unique to DS newborns and defined as the morphologic detection of blasts in DS less than three months of age. The present study investigated whether there was a difference between leukemia and TMD, and determined prognostic and risk factors.METHODS: We collected blood samples from 317 patients of 433 DS confirmed patients. We found 18 patients who had blast cells in their peripheral blood.RESULTS: Twelve patients were positive for blasts during the neonate period, and only one patient progressed to leukemia. The other 11 patients were later diagnosed with TMD. Six more patients were later diagnosed with leukemia, therefore, 7 patients were diagnosed with leukemia in total. All patients diagnosed with leukemia had anemia at the time of diagnosis, which was not found in TMD patients. All leukemia patients developed their disease after three months of life. Acute Myeloid Leukemia (AML) patients had additional chromosome mutation to trisomy 21 when they were diagnosed.CONCLUSION: In patients with Down Syndrome, anemia at diagnosis and age of onset could be helpful in distinguishing TMD from acute leukemia. Cancerous mutations in the chromosomes of peripheral and marrow blast cells of Down syndrome patients may foreshadow acute leukemia.

Clinical Characteristics of Severe Neutropenia in Children / 임상소아혈액종양

BACKGROUND: Severe neutropenia is defined as an absolute neutrophil count (ANC) less than 0.5×109/L, which is known to increase the risk of serious bacterial infections. The aim of this study was to investigate characteristics, etiology and differences between transient and chronic severe neutropenia in children. METHODS: 204 children, who were diagnosed with severe neutropenia at the Ajou University Hospital during a 5-year period, were included in the study. Clinical and laboratory features were analyzed. The patients were classified as having transient severe neutropenia (TSN) if recovery occurred within 6 months of diagnosis, and chronic severe neutropenia (CSN) if the neutropenia persisted for 6 months or more. RESULTS: 184 (90.2%) patients with TSN and 20 (9.8%) patients with CSN were identified. Most of the TSN occurred in patients less than 2 year of age (75.5%) and rarely occurred in patients 5 years or older (5.4%). The most common cause of TSN was infection-related neutropenia (82.6%), and most of the associated infections were respiratory infections (44.6%). Compared to TSN, CSN patients were younger at diagnosis (1.00 vs. 0.71, P < 0.001), had a lower ANC at diagnosis (364.8 vs. 214.9, P < 0.001), lower ANC at nadir (356.0 vs. 50.0, P < 0.001), and higher platelet count (188×10⁹ vs. 308×10⁹, P < 0.001), monocyte count (491.5×10⁶ vs. 832.9×10⁶, P=0.010) and CRP (0.22 vs. 0.85, P=0.036). CONCLUSION: Most of the severe neutropenia occurred in children younger than 2 years of age, and virus infection was the most common cause of TSN.

Differences in Clinical Characteristics of Transient Myeloproliferative Disease and Leukemia in Down Syndrome: A Single University Hospital Study / 임상소아혈액종양

BACKGROUND: Children with Down syndrome (DS) have a 10- to 20-fold increased risk of developing leukemia. However, in some patients, leukemia does not become apparent despite significant number of blast cells in the peripheral blood. This condition is called Transient myeloproliferative disorder (TMD), and is a disease entity unique to DS newborns and defined as the morphologic detection of blasts in DS less than three months of age. The present study investigated whether there was a difference between leukemia and TMD, and determined prognostic and risk factors. METHODS: We collected blood samples from 317 patients of 433 DS confirmed patients. We found 18 patients who had blast cells in their peripheral blood. RESULTS: Twelve patients were positive for blasts during the neonate period, and only one patient progressed to leukemia. The other 11 patients were later diagnosed with TMD. Six more patients were later diagnosed with leukemia, therefore, 7 patients were diagnosed with leukemia in total. All patients diagnosed with leukemia had anemia at the time of diagnosis, which was not found in TMD patients. All leukemia patients developed their disease after three months of life. Acute Myeloid Leukemia (AML) patients had additional chromosome mutation to trisomy 21 when they were diagnosed. CONCLUSION: In patients with Down Syndrome, anemia at diagnosis and age of onset could be helpful in distinguishing TMD from acute leukemia. Cancerous mutations in the chromosomes of peripheral and marrow blast cells of Down syndrome patients may foreshadow acute leukemia.

Allogenic Peripheral Blood Stem Cell Transplant-Induced Membranous Glomerulonephropathy as Chronic Graft-Versus-Host Disease / 대한내과학회지

Heavy proteinuria in the nephrotic range is an uncommon, often unrecognized manifestation of graft-versus-host disease (GVHD) following hematopoietic stem cell transplantation. A few isolated case reports have been published in the Korean literature involving a small number of patients who developed membranous nephropathy as GVHD after peripheral blood stem cell transplantation (PBSCT). A 17-year-old female was diagnosed with non-Hodgkin's lymphoma. Following remission, she underwent allogeneic PBSCT. Shortly thereafter, she developed acute GVHD, which was managed by medical therapy with prednisolone and cyclosporine. Approximately 13 months following PBSCT, the patient developed proteinuria without peripheral edema. Pulsed steroid therapy was initiated three times, but her condition did not improve. Twenty months after PBSCT, she developed nephrotic range proteinuria. A renal biopsy was performed, and the diagnosis was histologically consistent with membranous nephropathy. Because the response to steroids was not satisfactory, the dose of cyclosporine was increased. Approximately 3 months after renal biopsy, the proteinuria disappeared. Given the recent increase in the incidence of GVHD-mediated renal disease, in particular, renal biopsy is indispensable to the diagnosis of nephropathy and to the prevention of disease progression.

Outcome of Reinduction Chemotherapy with a Modified Dose of Idarubicin for Children with Marrow-Relapsed Acute Lymphoblastic Leukemia: Results of the Childhood Acute Lymphoblastic Leukemia (CALL)-0603 Study

This multicenter, prospective trial was conducted to develop an effective and safe reinduction regimen for marrow-relapsed pediatric acute lymphoblastic leukemia (ALL) by modifying the dose of idarubicin. Between 2006 and 2009, the trial accrued 44 patients, 1 to 21 years old with first marrow-relapsed ALL. The reinduction regimen comprised prednisolone, vincristine, L-asparaginase, and idarubicin (10 mg/m²/week). The idarubicin dose was adjusted according to the degree of myelosuppression. The second complete remission (CR2) rate was 72.7%, obtained by 54.2% of patients with early relapse < 24 months after initial diagnosis and 95.0% of those with late relapse (P = 0.002). Five patients entered remission with extended treatment, resulting in a final CR2 rate of 84.1%. The CR2 rate was not significantly different according to the idarubicin dose. The induction death rate was 2.3% (1/44). The 5-year event-free and overall survival rates were 22.2% ± 6.4% and 27.3% ± 6.7% for all patients, 4.2% ± 4.1% and 8.3% ± 5.6% for early relapsers, and 43.8% ± 11.4% and 50.0% ± 11.2% for late relapsers, respectively. Early relapse and slow response to reinduction chemotherapy were predictors of poor outcomes. In conclusion, a modified dose of idarubicin was effectively incorporated into the reinduction regimen for late marrow-relapsed ALL with a low toxic death rate. However, the CR2 rate for early relapsers was suboptimal, and the second remission was not durable in most patients.