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Clinical Course of Neutropenia in Previously Healthy Children.

Clinical Pediatric Hematology-Oncology.2018;25(2):87-96. doi:10.15264/cpho.2018.25.2.87

BACKGROUND: Neutropenia can be easily found in previously healthy children associated with various medical conditions, and the clinical course ranges from transient benign to life threatening. This study aimed to investigate the etiology, clinical characteristics, and clinical courses of neutropenia in previously healthy children. METHODS: We evaluated 215 previously healthy children under aged 18 years who diagnosed with neutropenia in two hospitals. Clinical and laboratory features were analyzed retrospectively based on the medical records. RESULTS: Transient infectious neutropenia (TIN) accounted for 97.7% of cases and chronic neutropenia (CN), for 2.3%. An infectious agent was identified in 128/210 (61%) patients with TIN, and the most frequent agents were viruses (46.5%). The most common viral agent was respiratory syncytial virus (RSV) (29%). TIN subgroups exhibited no differences in severity according to infectious agent (virus, bacteria, Mycoplasma); however, neutropenia severity differed among viral agents [mild-to-moderate neutropenia in the RSV group (857.3±293.3/µL) and moderate-to-severe neutropenia in the parainfluenza group (567.3±198.1/µL); P=0.017]. All patients with CN had anti-neutrophil antibody positivity (autoimmune neutropenia, AIN), and moderate-to-severe neutropenia predominated. The median duration of TIN was 8 days (range, 3–286 days), and it was significantly longer for AIN at 330 days (range, 217–730 days) (P=0.000). The median duration of neutropenia was also different according to each viral agent, with 4 days (range, 3–11 days) for the RSV group and longer durations for 3 other groups (influenza, parainfluenza, other respiratory viruses) (P=0.015). CONCLUSION: Neutropenia in previously healthy children is usually of transient infectious origin, with mild-to-moderate severity, and it resolves spontaneously without complications.
Autoimmune Diseases ; Bacteria ; Child* ; Humans ; Medical Records ; Neutropenia* ; Paramyxoviridae Infections ; Respiratory Syncytial Viruses ; Retrospective Studies ; Tin

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Predictive Value of Endometrial Thickness for Anemia in Adolescent Girls with Abnormal Uterine Bleeding.

Ji Sung LEE ; Young Hwan CHO ; In Sang JEON

Clinical Pediatric Hematology-Oncology.2018;25(2):81-86. doi:10.15264/cpho.2018.25.2.81

BACKGROUND: Immaturity of the endocrine system that controls the normal menstrual cycle frequently results in abnormal uterine bleeding (AUB) and elicits anemia in adolescent girls. This study was conducted to assess the predictive value of endometrial thickness (ET) for anemia in adolescent girls with AUB. METHODS: A retrospective chart review was performed for a cohort of adolescents (12–18 years old) with AUB who presented over a 10-year period. Complete blood count and ultrasonographic data of 115 adolescent girls with AUB were analyzed. Subjects were classified according to ET as group I (ET < 11 mm) and group II (≥11 mm), and the incidence of anemia was compared. Subjects were also classified according to age as group Y (12–15 years old of age) and group O (16–18 yr), and ET, hemoglobin (Hb), and incidence of anemia were compared. RESULTS: The incidence of anemia in all subjects was 67.8% and was significantly higher in group II than in group I (P < 0.001). The incidence of severe anemia was 56.9% in group II, which was higher than in group I (P=0.039). The incidence of anemia was not significantly different between groups Y and O. However, the incidence of severe anemia was significantly higher in group Y than in group O (P=0.001). CONCLUSION: AUB can result in severe anemia in adolescent girls particularly those who are close to menarche or have a thick endometrium. Early supervision of AUB is required in order to avoid anemia in adolescent girls with AUB.
Adolescent* ; Anemia* ; Blood Cell Count ; Cohort Studies ; Endocrine System ; Endometrium ; Female* ; Humans ; Incidence ; Menarche ; Menstrual Cycle ; Organization and Administration ; Retrospective Studies ; Uterine Hemorrhage*

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Two Cases of Adjuvant Immunotherapy with Cytokine-Induced Killer Cells for Relapsed or Refractory Neuroblastoma.

Jung Yoon CHOI ; Hong Yul AN ; Kyung Taek HONG ; Chery HONG ; Hyoung Jin KANG ; Hee Young SHIN

Clinical Pediatric Hematology-Oncology.2018;25(2):202-207. doi:10.15264/cpho.2018.25.2.202

The treatment outcomes of relapsed or refractory neuroblastoma have been unsatisfactory till date. We reported two cases of adoptive immunotherapy using cytokine-induced killer (CIK) cells against relapsed or refractory neuroblastoma. CIK cell production was attempted in three patients, out of which two patients exhibited adequate levels of CIK cell production. Two patients completed full term of CIK cell infusions (weekly for 6 weeks and then biweekly for 8 wk) without serious adverse events. The progression-free survivals for the two patients were 1.9 and 4.1 months. Their overall survivals were 16.7 and 28.7 months. Although the efficacy was unclear, CIK cell infusion combined with other treatment strategies may have prolonged overall survival in refractory neuroblastoma patients. Further studies are needed to determine the exact role of CIK cell-based immunotherapy in relapsed or refractory neuroblastoma patients.
Cytokine-Induced Killer Cells* ; Disease-Free Survival ; Humans ; Immunotherapy* ; Immunotherapy, Adoptive ; Neuroblastoma*

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Two Pediatric Cases of Spontaneous Ruptured Solid Tumors Successfully Treated with Transcutaneous Arterial Embolization.

Kyo Jin JO ; Eu Jeen YANG ; Kyung Mi PARK ; Jin Heyok KIM ; Ung Bae JEON ; Joo Yeon JANG ; Young Tak LIM

Clinical Pediatric Hematology-Oncology.2018;25(2):197-201. doi:10.15264/cpho.2018.25.2.197

Spontaneous rupture with internal bleeding of solid tumors has rarely been described at the time of diagnosis or during chemotherapy. This rare event must be regarded as a life threatening condition. In these emergency situations, control of hemorrhage, which is life-saving, can be achieved by transcatheter arterial embolization (TAE) and/or surgical resection. This report describes two infants presenting with acute hemorrhagic shock due to spontaneous tumor rupture of hepatoblastoma and neuroblastoma during chemotherapy. TAE successfully arrested the tumor bleeding and a visibly reduced the tumor size in both children. Spontaneous rupture of solid tumors occur infrequently in children, but is a life threatening situation. Careful monitoring for the occurrence of this rare event especially in very young children presenting with a large tumor mass.
Child ; Diagnosis ; Drug Therapy ; Emergencies ; Hemorrhage ; Hepatoblastoma ; Humans ; Infant ; Neuroblastoma ; Rupture ; Rupture, Spontaneous ; Shock, Hemorrhagic

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A Case of Transient Myeloproliferative Disorder Associated with Clonal Trisomy 21 in a Chromosomally Normal Newborn.

Jihyun PARK ; Yoo Rha HONG ; Seom Gim KONG

Clinical Pediatric Hematology-Oncology.2018;25(2):191-196. doi:10.15264/cpho.2018.25.2.191

Transient myeloproliferative disorder (TMD) is similar to congenital leukemia, with leukocytosis, increased peripheral blast cells, and hepatomegaly in the neonatal period. Although TMD occurs only in patients with Down syndrome and trisomy 21 mosaicism, there have been reports of congenital leukemia with trisomy 21 limited to hematopoietic cells showing spontaneous resolution. We identified trisomy 21 in the leukemic cells in a patient with congenital leukemia. As there was no other gene abnormality, we stopped chemotherapy, and the disease resolved spontaneously. We reviewed the cases of clonal trisomy 21 TMD and found that their clinical features were similar to those of TDM associated with Down syndrome. In conclusion, in a phenotypically normal patient with suspected congenital leukemia, it is necessary to confirm the presence of 21 trisomy. If the neonate has only trisomy 21 without other gene abnormalities, intensive chemotherapy may not be required.
Chromosomes, Human, Pair 21 ; Down Syndrome* ; Drug Therapy ; GATA1 Transcription Factor ; Hepatomegaly ; Humans ; Infant ; Infant, Newborn* ; Leukemia ; Leukocytosis ; Mosaicism ; Myeloproliferative Disorders* ; Trisomy*

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A Case of Pediatric Unprovoked Deep Vein Thrombosis due to Combined Hereditary Thrombophilia of Antithrombin III and Protein S Deficiency.

Jeong Yeon KIM ; I Seok KANG ; Hee Jin KIM

Clinical Pediatric Hematology-Oncology.2018;25(2):185-190. doi:10.15264/cpho.2018.25.2.185

Unprovoked deep vein thrombosis (DVT) is uncommon in pediatric patients and, among those, combined hereditary thrombophilia is particularly rare. We present a 9-year-old Korean boy who developed lower extremity pain with swelling, and was diagnosed with unprovoked DVT due to hereditary (combined hereditary thrombophilia). Coagulation test revealed antithrombin III and protein S deficiency. The genetic work up confirmed the first case of combined antithrombin III deficiency and protein S deficiency by SERPINC1 heterozygous termination mutation [c.685C>T (p.Arg229*)] and PROS1 heterozygous missense mutation [c.1597G>A (p.Val533Met)]. He was treated with continuous heparin and catheter intervention but those were ineffective or transiently effective. His DVT gradually improved only after prolonged anticoagulation.
Antithrombin III Deficiency ; Antithrombin III* ; Catheters ; Child ; Heparin ; Humans ; Lower Extremity ; Male ; Mutation, Missense ; Protein S Deficiency* ; Protein S* ; Thrombophilia* ; Venous Thrombosis*

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Management of Spontaneous Spinal Epidural Hematoma Developed in Children with Hemophilia.

Han Na JANG ; Hoi Soo YOON ; Young Shil PARK

Clinical Pediatric Hematology-Oncology.2018;25(2):180-184. doi:10.15264/cpho.2018.25.2.180

Spinal epidural hematoma (SEH) is a rare neurosurgical emergency in which pressure on the spinal cord leads to acute neurological deficits, and is a rare complication in children with hemophilia. We report three cases of SEH in severe hemophilia A. An 8-month-old boy who presented with non-traumatic acute-onset irritability was found to have SEH and was later diagnosed with hemophilia. The two other patients presented with neck pain and magnetic resonance imaging confirmed the diagnosis of SEH. Two patients who received conservative management fully recovered, however the patient who presented with progressive neurological abnormalities at the time of diagnosis, received surgery but later developed breathing difficulties and quadriplegia. Early diagnosis and immediate, aggressive, clotting factor replacement therapy are crucial when managing SEH in children with hemophilia. Immediate and aggressive factor replacement, accompanied by both neurological monitoring and early imaging, are essential for hemophiliac with suspected SEH.
Child* ; Diagnosis ; Early Diagnosis ; Emergencies ; Hematoma ; Hematoma, Epidural, Spinal* ; Hemophilia A* ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Neck Pain ; Quadriplegia ; Respiration ; Spinal Cord

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A Case of Ethosuximide-Induced Aplastic Anemia Successfully Treated with Methylprednisolone Pulse Therapy.

Hyun Sik KANG ; Sun Hyung KIM

Clinical Pediatric Hematology-Oncology.2018;25(2):175-179. doi:10.15264/cpho.2018.25.2.175

Aplastic anemia may develop secondary to environmental exposure to entities such as chemicals, medical drugs, and infectious agents. Fatal complications from antiepileptic medications may occur despite careful and appropriate use. We report the case of a 9-year-old girl with a presenting diagnosis of aplastic anemia following treatment with ethosuximide for absence seizures. Aplastic anemia can now be cured with stem cell transplantation or immunosuppressive therapy. In this case, however, because of the impossibility of bone marrow transplantation and the specific needs of the patient's parents, three courses of methylprednisolone pulse therapy were administered. Following the therapy, there was improvement in pancytopenia and complete remission in the bone marrow. No adverse side effects of therapy were observed. The authors suggest that methylprednisolone pulse therapy may be a treatment for acquired aplastic anemia.
Anemia, Aplastic* ; Anticonvulsants ; Bone Marrow ; Bone Marrow Transplantation ; Child ; Diagnosis ; Environmental Exposure ; Epilepsy, Absence ; Ethosuximide ; Female ; Humans ; Methylprednisolone* ; Pancytopenia ; Parents ; Stem Cell Transplantation

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Cytomegalovirus-Associated Severe Direct Antiglobulin Test Negative Hemolytic Anemia: A Case Report.

Hyun Jung HONG ; Young Hye CHO ; Jae Young LIM ; Jung Sook YEOM ; Ji Sook PARK ; Eun Sil PARK ; Ji Hyun SEO ; Hyang Ok WOO ; Hee Shang YOUN

Clinical Pediatric Hematology-Oncology.2018;25(2):170-174. doi:10.15264/cpho.2018.25.2.170

Cytomegalovirus is a common virus that is mostly asymptomatic when infected, but rarely causes life-threatening hemolysis especially in immunocompromised children. We report a case of antiglobulin test negative severe hemolytic anemia caused by cytomegalovirus infection developed in an immune competent 9-year-old girl. The patient's hemoglobin level was 4.8 g/dL on the day of admission. The diagnosis was achieved by exclusion of other causes of hemolytic anemia and serological evidence of recent CMV infection. The patient was successfully treated with anti-viral agents and steroids resulting in recovery from anemia. Clinicians should consider cytomegalovirus infection in the differential diagnosis of hemolytic anemia in pediatric patients.
Anemia ; Anemia, Hemolytic* ; Child ; Coombs Test* ; Cytomegalovirus ; Cytomegalovirus Infections ; Diagnosis ; Diagnosis, Differential ; Female ; Hemolysis ; Humans ; Steroids

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Disulfram Treatment of NUP98-PHF23 AML Is Not Effective In Vivo: Potential Role for Hematopoietic Stem Cells Niche.

Eun Sil PARK ; Yang Jo CHUNG ; Peter D APLAN

Clinical Pediatric Hematology-Oncology.2018;25(2):162-169. doi:10.15264/cpho.2018.25.2.162

BACKGROUND: NUP98 has numerous partner genes of which plant homeodomain (PHD) finger protein 23 (PHF23) fusion with NUP98 (NP23) can be detected by RT-PCR in patients with cytogenetically normal acute myelogenous leukemia (AML). In this fusion transcript of NP23 PHD of PHF23 is known to specifically bind H3K4me3 residues and act as a chromatic modifier. Disulfiram (DSF) which inhibits the binding of PHD to H3K4me3 residues selectively killed NP23 myeloblasts in vitro and therefore, we planned to evaluate the efficacy of DSF in vivo. METHODS: Cultured 961C cells (CD45.2), NP23 myeloblast cells were transplanted into B57BL/6 mice (CD45.1). Using limit dilution assay the number of leukemic stem cells (LSCs) could be calculated. A certain amount of 961C cells were transplanted into B57BL/6 mice and DSF was treated after 1 week. The engraftment level was monitored with CD45.2. Kaplan Meier survival curve was used to compare the survival between therapeutic and control group. RESULTS: 961C cells could be transplanted without radiation in recipient mice. Calculated LSC was estimated to be 1 out of 184 cells (95% CI range, 56–609). When treated with DSF of different doses and administration routes in 961C recipient mice no survival advantage of DSF was observed in 961C transplanted immunocompetent mouse, however it was evident that engraftment level was consistent in both groups. CONCLUSION: No survival advantage of DSF in 961C transplanted immunocompetent mouse was observed, however it was evident that 961C cells shared niche with normal hematopoietic stem cells (HSCs). We expect that 961C cells and transplanted recipient mice have the potential to be used as in vivo system for new drugs development as well as for research dealing with niche for normal HSCs and LSCs.
Animals ; Disulfiram ; Fingers ; Granulocyte Precursor Cells ; Hematopoietic Stem Cells* ; Humans ; In Vitro Techniques ; Leukemia, Myeloid, Acute ; Mice ; Plants ; Stem Cells

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