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Tumors of the Pleura and Lung Developed 17 Years after Allogeneic Bone Marrow Transplantation for Childhood Acute Myelomonocytic Leukemia:Synovial Sarcoma Mimicking Malignant Mesothelioma

Jun Ah LEE ; Bin CHO ; Sun Ah SHIN ; Seog Yun PARK ; Meerim PARK ; Hyeon Jin PARK

Clinical Pediatric Hematology-Oncology.2021;28(1):63-66. doi:10.15264/cpho.2021.28.1.63

Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body irradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational asbestos exposure. Five months later, the patient presented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma.To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.

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Cytomegalovirus Infection Mimicking Recurrence of Malignant Lymphoma: A Case Report

Sae-Mee PARK ; Young Bae CHOI ; Joon Kee LEE

Clinical Pediatric Hematology-Oncology.2021;28(1):58-62. doi:10.15264/cpho.2021.28.1.58

When a patient with malignant lymphoma develops new lymph node enlargement, a recurrence of lymphoma is usually suspected first. However, painless and rapid lymph node enlargement, a manifestation of non-Hodgkin’s lymphoma, could also be due to other causes. A 3-year-old boy who was previously diagnosed with Burkitt lymphoma was admitted for routine tumor evaluation one year following completion of treatment. Abdominal computed tomography showed several enlarged lymph nodes in the right lower quadrant, and 18 F-fluoro-2-deoxy-D-glucose positron emission tomography revealed hypermetabolic enlarged lymph nodes in the corresponding lesion. The patient underwent ileocecal lymph node biopsy for pathologic confirmation, which revealed reactive hyperplasia without lymphoma recurrence. Serologic test results for cytomegalovirus immunoglobulin G and immunoglobulin M were positive. Additionally, the polymerase chain reaction test performed using a urine sample was positive for cytomegalovirus. After several outpatient follow-ups, we concluded cytomegalovirus infection that mimicked a recurrence of lymphoma on imaging as the cause for lymph node enlargements. This case highlights the importance of using prompt and multiple approaches after detecting a possible tumor recurrence through imaging studies.

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Recombinant von Willebrand Factor for Pediatric Patient with von Willebrand Disease: First Report in Korea

Seoin KIM ; Young Shil PARK

Clinical Pediatric Hematology-Oncology.2021;28(1):54-57. doi:10.15264/cpho.2021.28.1.54

von Willebrand disease (VWD) is the most common hereditary bleeding disorder. The treatment of VWD consists mainly of desmopressin and plasma-derived von Willebrand factor (pd-VWF) concentrate. We report on the first patient with VWD to be treated with recombinant VWF (rVWF) concentrate in Korea. Our patient was diagnosed with type 2 VWD at 10 months of age and suffered persistent severe epistaxis despite therapeutic levels of VWF activity and factor VIII (FVIII). At 34 months of age, rVWF was initiated and administered a total of 15 times in the following 8 months. No drug-related adverse events were observed and the patient was neither admitted nor given any transfusions during this period. Unlike pd-VWF/FVIII concentrates, rVWF did not increase FVIII to the excessively high levels that constitute a risk factor for thromboembolism, and was also preferable to pd-VWF/FVIII concentrates in that it contains ultra-large multimers of VWF. This is the first reported case in Korea in which rVWF was used to treat VWD. rVWF may be well tolerated and effective in VWD patients, especially those with refractory bleeding.

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Successful Treatment of Severe Hemolytic Disease of the Newborn Caused by Anti-Jk b

Goo Lyeon KIM ; Yoonsoo KIM ; Young Pyo CHANG ; Ju-Hee SEO ; Mee Jeong LEE

Clinical Pediatric Hematology-Oncology.2021;28(1):49-53. doi:10.15264/cpho.2021.28.1.49

Hemolytic disease of the newborn (HDN) is a condition in which maternal antibodies cross the placenta and cause hemolytic reactions. Anti-RhD was the most common cause, but with the introduction of immunoglobulin, the frequency has decreased sig nificantly, making hemolytic disease caused by other minor blood g roups more important. Kidd antigen is also known to cause hemolytic transfusion reactions. Only 13 cases have been reported so far, because Kidd antig en dose not usually cause HDN. Most cases have a good outcome, and only two fatal cases have been reported.A four-day-old male patient was hospitalized for jaundice, and hemolysis was confirmed by blood test. The mother’s blood was Jkb antibody positive. The patient did not improve with phototherapy, so an exchange transfusion was performed. Additional hemolysis occurred, so we undertook transfusion of red blood cells, resulting in cessation of hemolysis. We report HDN caused by Jkb antibody that responded to exchange blood transfusion.

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A Case of Severe Iron Deficiency Anemia Due to Pediculus Capitis

Andrew Van HERSH ; Samantha WIRKOWSKI ; Benjamin MCMILLON ; Amelia BALDERSTON ; Alexis HAMELINK ; Kerry MCGOWAN

Clinical Pediatric Hematology-Oncology.2021;28(1):46-48. doi:10.15264/cpho.2021.28.1.46

Infestation with Pediculus capitis, or head lice, is a common occurrence in the pediatric population. These ectoparasites survive by feeding on human blood. While a nuisance, lice are typically considered harmless and the amount of blood imbibed is not usually considered to be of clinical significance. We present a case of a 10-year-old female with severe, prolonged head lice infestation with concomitant iron deficiency anemia that was unable to be attributed to other causes.

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Significance of Evaluation for von Willebrand Disease in Premenopausal Women with Iron Deficiency Anemia and Menorrhagia

Ji Hyun KIM ; Ji Yeon LEE ; Bo Lyun LEE ; Kwang Hoon KIM ; Jeong Eun LEE ; Ji Kyoung PARK

Clinical Pediatric Hematology-Oncology.2021;28(1):39-45. doi:10.15264/cpho.2021.28.1.39

Background: In Korea, in the case of patients with significant bleeding symptoms due to unknown causes, there are very few studies that evaluate bleeding disorders, including von Willebrand disease (VWD ). VWD should be considered as an important causative factor in patients with iron deficiency anemia (IDA) and unexplained menorrhagia. This study aimed to understand the clinical characteristics of VWD and the significance of evaluation for VWD in premenopausal women in Korea with menorrhagia and ID A. Methods: Premenopausal women who were diagnosed with IDA and menorrhagia from January 2009 to March 2020 were included. IDA was diagnosed by either low ferritin or transferrin saturation with microcytic anemia. Menorrhagia was evaluated based on the medical records obtained from a gynecologist. VWD diagnosis was defined as von Willebrand factor antigen ＜50% and von Willebrand factor ristocetin cofactor activity ＜50%, which were low according to the Hospital for Sick Children criteria. Results: Out of a total of 120 patients, only 12 were tested for VWD, all of whom were pediatric patients. Four of the 12 pediatric patients tested were diagnosed with VWD and 4 of the 120 (3.3%) patients with IDA and menorrhagia were diagnosed with VWD. Three out of the 4 patients was diagnosed with VWD by repeat screening test. Although all parameters are not statistically significant, VWD patients tended to have ID A at a younger age (13.25 vs. 15.00 years) and were more likely to have recurrent IDA than patients without VWD. Conclusion Clinical doctors should consider VWD if patients have menorrhagia with ID A. If VWD is suspected, repeated VWD screening tests are necessary to increase the diagnosis rate. Accurate diagnosis of VWD in patients with significant bleeding may facilitate decisions for appropriate treatment.

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Treosulfan-Based Conditioning Regimen for Hematopoietic Stem Cell Transplantation in Pediatric Patients with Hemophagocytic Lymphohistiocytosis

Ho Joon IM ; Sung Han KANG

Clinical Pediatric Hematology-Oncology.2021;28(1):28-38. doi:10.15264/cpho.2021.28.1.28

Hemophagocytic lymphohistiocytosis (HLH) is a fatal disease unless timely and effective treatment is given. Immunochemotherapy including etoposide, with or without hematopoietic stem cell transplantation (HCT), has improved the outcomes for patients with HLH. In patients with familial or refractory HLH, HCT is now routinely performed and is the only curative treatment. Conditioning regimens play an important role in the success of HCT for pediatric patients with HLH and other nonmalignant diseases and have improved dramatically in recent decades. The initial HCT approach using myeloablative conditioning significantly improved the outcomes of patients with HLH but was associated with considerable transplant-related mortality. A subsequent strategy using reduced-intensity conditioning (RIC) remarkably reduced the incidence of T RM. However, the high level of mixed chimerism associated with RIC has prompted the search for improved conditioning regimens. Recently, treosulfan has replaced busulfan as a component of a reduced toxicity conditioning regimen. Both its myeloablative and immunosuppressive properties, as well as a favorable toxicity profile, make treosulfan a potential candidate for use as part of conditioning regimen prior to HCT. Indeed, treosulfan-based conditioning regimens are being increasingly used in pediatric patients with various non-malignant diseases. We here review the recent progress in HCT for pediatric HLH with a focus on treosulfan-based conditioning regimens, including our own experience.

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EBV-Associated Lymphoproliferative Disorders

Young Hyeh KO

Clinical Pediatric Hematology-Oncology.2021;28(1):14-27. doi:10.15264/cpho.2021.28.1.14

Epstein–Barr virus (EBV) is associated with a wide range of human lymphoproliferative disorders (LPD) of B, T, and natural killer (NK)-cell lineage. In children, abnormal immune response to primary EBV infection can cause peculiar forms of T/NKcell LPD of childhood, such as the systemic form of chronic active EBV infection, hydroa vacciniforme-like LPD, severe mosquito bite allergy and systemic T cell lymphoma of childhood. In adults, dysregulation of the immune response to EBV infection, immunosenescence caused by aging, chronic inflammation in a closed space, and iatrogenic immune suppression can lead to EBV-positive LPD of diverse types involving B, T, and NK cells with unique clinical and pathological presentations. This review describes the clinical, pathological, and genetic findings of EBV-positive LPD listed in the revised 2016 WHO Classification of Tumours of Haematopoietic and Lymphoid Tissue.

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Management of Malignancies Developing in AYA

Alex WK. LEUNG ; Herbert HF. LOONG ; Teresa TSE ; Chi-kong LI

Clinical Pediatric Hematology-Oncology.2021;28(1):1-13. doi:10.15264/cpho.2021.28.1.1

Adolescent and young adult (AYA) with cancers have distinct spectrum of cancers as compared to younger and older age groups. The definition of age limits of AYA varies among countries, from 15-25 years to 12-39 years. The differences in age definition lead to variation in report of incidence, types of cancers and survival. In younger AYA patients, hematological malignancies are leading cause of cancers. In older AYA patients, testicular cancers are common in males while breast cancers and cervical cancers are predominant types in females. There is increasing incidence of AYA cancers worldwide in the past two decades. Overall survival and treatment outcome of AYA cancer has been improving in the last few decades. Specialized centers for AYA with cancers provide more comprehensive care and have been reported to have superior outcome. About 80% of AYA with cancers survive at 5 years after diagnosis but they are higher risk of developing second malignancies. Barriers to AYA cancer treatment included social economic status, insurance system and accessibility to clinical trials. Survivors of AYA cancers are also at higher risk dying from cardiovascular diseases and respiratory diseases. Survivorship program should be in place to enhance education and surveillance.

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Use of Recombinant Activated Factor VII for Pulmonary Hemorrhage in Premature Infants: A Single-Center Experience

Hye Jin PARK ; Eun Jin CHOI

Clinical Pediatric Hematology-Oncology.2016;23(1):42-45. doi:10.15264/cpho.2016.23.1.42

BACKGROUND: Pulmonary hemorrhage in prematurity is a life-threatening complication and associated with a high mortality. Recombinant activated factor VII (rFVIIa) has been reported as hemostatic treatment in sick neonates with refractory bleeding events in many studies. We evaluated the efficacy and safety of rFVIIa in prematurity with pulmonary hemorrhage in our institution.METHODS: From the prematurities who were treated with rFVIIa to pulmonary hemorrhage from January 2010 to December 2015, we retrospectively analyzed the results of rFVIIa.RESULTS: Of the 29 prematurities who were treated with rFVIIa for pulmonary hemorrhage, fifteen were male and fourteen were female. The median gestational age was 27 1/7 weeks (range, 22 1/7-34 1/7 weeks) and median birth weight was 870 g (range, 470-2,070 g). One to eight doses of rFVIIa (median dose 115.6 µg/kg/dose) were administered, with 16 (55%) patients receiving a single dose. Hemostatic effect was achieved in 21 (72.4%) cases, but 6 of 21 patients died of unrelated cause, and overall mortality was 14 of 29 (48.3%). Thrombotic adverse event was not observed in any of our patients.CONCLUSION: Although the number of patients included in this study was small and the fact that this was a retrospective non-randomized control study, rFVIIa could be considered as a therapeutic option for pulmonary hemorrhage in prematurity.
Birth Weight ; Factor VIIa ; Female ; Gestational Age ; Hemorrhage ; Humans ; Infant, Newborn ; Infant, Premature ; Male ; Mortality ; Retrospective Studies

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