Journal Detail Information

1

Cite

Share

Primary Cardiac Hemangioendothelioma in an Infant: A Case Report

Jeong wook SEO ; Mi Kyoung SONG ; Sung Hye PARK ; Hye Eun PARK ; Sin Ae PARK

Clinical Pediatric Hematology-Oncology.2019;26(1):60-65. doi:10.15264/cpho.2019.26.1.60

Primary cardiac tumors are rare, with a prevalence of 0.001–0.2%. Among such tumors, cardiac hemangioendotheliomas are some of the most uncommon. In Korea, there have been no reports of hemangioendothelioma occurring in the heart of infants. We herein report a case of an infant that was admitted to our medical center and presented with cough and a runny nose. The initial diagnosis was acute bronchiolitis. Cardiomegaly was observed on chest radiography. Echocardiography revealed a tumor measuring 3.5×4.0 cm in the right atrium. The infant was transferred to a tertiary medical center for tumor excision. The excised lesion was 3.8×3×3.2 cm in size, and biopsy confirmed a diagnosis of hemangioendothelioma. In this case report, we describe our experience with a rare case involving cardiac tumor in an infant with an upper respiratory tract infection.
Biopsy ; Bronchiolitis ; Cardiomegaly ; Cough ; Diagnosis ; Echocardiography ; Heart ; Heart Atria ; Heart Neoplasms ; Hemangioendothelioma ; Humans ; Infant ; Korea ; Nose ; Prevalence ; Radiography ; Respiratory Tract Infections ; Thorax ; Twins

2

Cite

Share

Various Complications after a Vascular Procedure in Patients with Hemophilia

Yeon Soo HA ; Young Shil PARK

Clinical Pediatric Hematology-Oncology.2019;26(1):55-59. doi:10.15264/cpho.2019.26.1.55

Hemophilia, an inherited bleeding disorder, is caused by a deficiency of coagulation factor VIII or IX. Most of patients with hemophilia need vascular procedure, which can lead to complications. Even though these complications can also occur in normal people, hemophilia and coagulopathy are particular risk factors. We reviewed medical records of patients with hemophilia who underwent vascular procedures and investigated its complications. Vessel-related complications occurred in five patients. Three patients had pseudoaneurysms after radial arterial puncture. All patients underwent coagulation factor replacement or ultrasound-guided compression and showed improvement. Neuropathy developed in one patient due to a hematoma that occurred after blood sampling. The hematoma improved, but motor and sensory deficits remained and neuropathy was confirmed. One patient died of uncontrolled bleeding after angiography. Vascular procedures require more attention in patients with hemophilia. Caution and prevention of complications is essential, even before the patient is diagnosed with hemophilia.
Aneurysm, False ; Angiography ; Blood Coagulation Factors ; Factor VIII ; Hematoma ; Hemophilia A ; Hemorrhage ; Humans ; Medical Records ; Punctures ; Risk Factors

3

Cite

Share

A Multicenter Study on von Willebrand Disease Realities in Yeungnam Region

Hyun Ju KIM ; Ye Jee SHIM ; Jae Min LEE ; Young Tak LIM ; Eu Jeen YANG ; Kyung Mi PARK ; Hee Won CHUEH ; Eun Sil PARK ; Hyo Sun KIM ; Ji Kyoung PARK ; Eun Jin CHOI ; Seom Gim KONG ; Ji Yoon KIM ; Sang Kyu PARK

Clinical Pediatric Hematology-Oncology.2019;26(1):46-54. doi:10.15264/cpho.2019.26.1.46

BACKGROUND: von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. METHODS: Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. RESULTS: Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). CONCLUSION: A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.
Adult ; Child ; Diagnosis ; Hemophilia A ; Hemorrhage ; Hospitals, University ; Humans ; Korea ; Medical Records ; Prevalence ; Retrospective Studies ; von Willebrand Diseases

4

Cite

Share

Current Assessment and Management of Retinoblastoma

Hee Won CHUEH

Clinical Pediatric Hematology-Oncology.2019;26(1):35-45. doi:10.15264/cpho.2019.26.1.35

Retinoblastoma is the most common intraocular malignancy in childhood. Diagnosis is currently made by ophthalmologists under general anesthesia as it is the gold standard for intraocular assessment. However, evaluations for extraocular disease are also necessary. Treatment strategies vary according to the disease status. If a single eye is involved, the treatment goal is oriented to the removal of the tumor and prevention of relapse. In bilateral retinoblastoma, the main treatment goal is to save monocular vision and save life. This article will explore the available treatment options for retinoblastoma including enucleation, radiotherapy, local therapy, intravenous chemotherapy, intra-arterial injection and intra-vitreal injections. There were recent advances in our understanding on the genetic pathophysiology of the retinoblastoma protein gene in tumorigenesis, which may help developing future treatment. Early detection of retinoblastoma is important for prolonging survival and improving quality of life.
Anesthesia, General ; Carcinogenesis ; Diagnosis ; Drug Therapy ; Injections, Intra-Arterial ; Quality of Life ; Radiotherapy ; Recurrence ; Retinoblastoma Protein ; Retinoblastoma ; Stem Cell Transplantation ; Vision, Monocular

5

Cite

Share

Ewing Sarcoma

Hee Young JU

Clinical Pediatric Hematology-Oncology.2019;26(1):27-34. doi:10.15264/cpho.2019.26.1.27

Ewing sarcoma is the second most frequently occurring malignant tumor of the bone and soft tissue in adolescents and young adults. Genetically, Ewing sarcoma is characterized by balanced chromosomal translocation in which a member of FET gene family is fused with an ETS transcription factor, with the most common fusion being EWSR1-FLI1 (85% of cases). Treatment of Ewing sarcoma is based on multidisciplinary approach (local surgery, radiotherapy and multiagent chemotherapy), which are associated with chronic late effects that may compromise quality of life of survivors. First line treatment includes combination of drugs incorporating doxorubicin, vincristine, cyclophosphamide, ifosfamide, etoposide, and dactinomycin. The beneficial role of high dose chemotherapy has been suggested in high-risk localized Ewing sarcoma patients, and the studies are being performed to investigate the role in metastatic disease. The 5-year overall survival for localized Ewing sarcoma has improved to reach 65% to 75%. But patients with metastatic disease have a 5-year survival rate of <30%, except for those with isolated pulmonary metastasis (approximately 50%). Patients with recurrent tumor have a dismal prognosis. Novel therapeutic strategies based on understanding of molecular mechanisms are needed to improve the outcome of Ewing sarcoma and to lessen the treatment-related late effects.
Adolescent ; Cyclophosphamide ; Dactinomycin ; Doxorubicin ; Drug Therapy ; Etoposide ; Humans ; Ifosfamide ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive, Peripheral ; Prognosis ; Quality of Life ; Radiotherapy ; Sarcoma, Ewing ; Survival Rate ; Survivors ; Transcription Factors ; Translocation, Genetic ; Vincristine ; Young Adult

6

Cite

Share

Advances in the Treatment of Childhood Acute Lymphoblastic Leukemia

Hyery KIM

Clinical Pediatric Hematology-Oncology.2019;26(1):12-26. doi:10.15264/cpho.2019.26.1.12

In recent decades, survival rates for childhood acute lymphoblastic leukemia have improved remarkably, as demonstrated by risk-stratified, prospective multicenter studies. Treatment protocols have evolved and become better matched to both prognostic factors and treatment responses. Recently, new molecular prognostic factors have been discovered in leukemia genomic studies. New tumor subtypes with independent gene expression profiles have also been characterized. Furthermore, therapies targeted to specific candidate mutations are being identified to broaden therapeutic options for patients with poor prognoses. Many new drugs are in clinical trials and immunotherapy is attracting significant interest for the treatment of recurrent or refractory disease in childhood acute lymphoblastic leukemia.
Clinical Protocols ; Humans ; Immunotherapy ; Leukemia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prognosis ; Prospective Studies ; Survival Rate ; Transcriptome

7

Cite

Share

Vascular Anomaly: An Updated Review

Meerim PARK

Clinical Pediatric Hematology-Oncology.2019;26(1):6-11. doi:10.15264/cpho.2019.26.1.6

Vascular anomalies comprise a heterogeneous group of disorders characterized by abnormal growth or development of blood vessels. Diagnosis of vascular anomalies is often challenging due to the large variety of conditions, which exhibit phenotypic heterogeneity and a wide range of symptomology and severity. Accurate diagnosis is crucial for appropriate evaluation and management, often requiring multidisciplinary specialists. Recent interdisciplinary collaboration has led to collaborative studies and their outcomes are being prospectively evaluated. While there is still a role for surgical intervention in various vascular anomalies, discoveries of pharmacologic agents effective in treating vascular anomalies have broadened our medical therapeutic options. This paper focuses on vascular anomaly issues often seen by the pediatricians and reviews the clinical pearls on infantile hemangiomas, lymphatic malformations, venous malformations, and arteriovenous malformations.
Arteriovenous Malformations ; Blood Vessels ; Cooperative Behavior ; Diagnosis ; Hemangioma ; Population Characteristics ; Prospective Studies ; Specialization

8

Cite

Share

What Should We Consider Carefully When Performing Survival Analysis?

Sang Gyu KWAK ; Eun Jin CHOI

Clinical Pediatric Hematology-Oncology.2019;26(1):1-5. doi:10.15264/cpho.2019.26.1.1

The survival data and the survival analysis are the data and analysis methods used to study the probability of survival. The survival data consist of a period from the juncture of a start event to the juncture of the end event (occurrence event). The period is called the survival period or survival time. In this way, the method of analysing the survival time of subjects and appropriately summarizing the degree of survival is called survival analysis. To understand and analyse survival analysis methods, researchers must be aware of some concepts. Concepts to be aware of in the survival analysis include events, censored data, survival period, survival function, survival curve and so on. This review focuses on the terms and concepts used in the survival analysis. It will also cover the types of survival data that should be collected and prepared when using actual survival analysis method and how to prepare them.
Methods ; Survival Analysis

9

Cite

Share

Two Cases of Cytomegalovirus Infection Developed in Pediatric Acute Lymphoblastic Leukemia Patients

Nayoung JUNG ; Donghyun KIM ; Hee Seung CHIN ; Soon Ki KIM

Clinical Pediatric Hematology-Oncology.2019;26(2):115-118. doi:10.15264/cpho.2019.26.2.115

A 14 year-old boy with acute lymphoblastic leukemia (ALL) on maintenance chemotherapy presented with vision-threatening cytomegalovirus (CMV) retinitis. Treatment with intavitreal ganciclovir injection (2 mg/0.1 mL) followed by oral ganciclovir resulted in successful resolution of CMV retinitis. Another 13 year-old boy with ALL on maintenance chemotherapy presented with prolonged fever with no response to antibiotics administration. CMV and real-time PCR revealed positive result and a titer of 2,618,700 copies/mL, respectively. Ganciclovir was used for more than the approved duration of treatment, but viral titer frequently recurred with elevated liver enzymes and fever. In these 2 cases of CMV infection, a high index of suspicion and prompt management is important in children receiving ALL chemotherapy.
Anti-Bacterial Agents ; Child ; Cytomegalovirus Infections ; Cytomegalovirus ; Drug Therapy ; Fever ; Ganciclovir ; Humans ; Liver ; Maintenance Chemotherapy ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Real-Time Polymerase Chain Reaction ; Retinitis

10

Cite

Share

A Case of Congenital Hepatoblastoma Presented with Hepatic Osteodystrophy Complicated by Multiple Bone Fractures

Yoon Heui SO ; Dae Sung KIM ; Bo Ae YOON ; Yoo Duk CHOI ; Hee Jo BAEK ; Hoon KOOK

Clinical Pediatric Hematology-Oncology.2019;26(2):110-114. doi:10.15264/cpho.2019.26.2.110

Hepatic osteodystrophy is frequent complication in patients with chronic liver disease, particularly with chronic cholestasis. We report a male infant with congenital hepatoblastoma, who had osteodystrophy complicated by multiple bone fractures despite adequate supplementation of fat-soluble vitamins including vitamin D. He was born by Caesarean section because of a 7 cm–sized abdominal mass detected by prenatal ultrasonography. The pathologic diagnosis was hepatoblastoma, PRETEXT staging III or IV. Whole body bone scan at the time of diagnosis showed no abnormal uptake. Oral vitamin D3 of 2,000 IU/day was administered with other fat-soluble vitamins. Serum direct bilirubin level gradually increased up to 28.9 mg/dL at postnatal 6 days and was above 5 mg/dL until 110 days of age. Bony changes consistent with rickets became apparent in left proximal humerus since 48 days of age, and multiple bone fractures developed thereafter. With resolving cholestasis by chemotherapy, his bony lesions improved gradually after add-on treatment of bisphosphonate and parenteral administration of vitamin D with calcium. High level of suspicion and prevention of osteodystrophy is needed in patients with hepatoblastoma, especially when cholestasis persists.
Bilirubin ; Calcium ; Cesarean Section ; Cholecalciferol ; Cholestasis ; Diagnosis ; Drug Therapy ; Female ; Fractures, Bone ; Hepatoblastoma ; Humans ; Humerus ; Infant ; Liver Diseases ; Male ; Pregnancy ; Rickets ; Ultrasonography, Prenatal ; Vitamin D ; Vitamins

DISPLAY OPTIONS

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share

Cite

Share