Immune thrombocytopenia (ITP) is a disease in which thrombocytopenia occurs because of immune-mediated platelet destruction and decreased platelet production. Although many pediatric patients with ITP experience spontaneous remission or reach remission within 12 months of first-line therapy, approximately 20% progress to chronic ITP. Patients who do not respond to first-line treatment or experience frequent relapses are of great concern to physicians. This review summarizes recent treatments for second-line treatment of pediatric chronic ITP.
Citations
Citations to this article as recorded by
Beta-Thalassemia with Initial Presentation as Immune Thrombocytopenia: A Case Report Hyun Sik Kang Clinical Pediatric Hematology-Oncology.2023; 30(1): 42. CrossRef
Background Interest in digital medical information has increased because it allows doctors to easily access a patient’s medical records and provide appropriate medical care. Blockchain technology ensures data safety, reliability, integrity, and transparency by distributing medical data to all users over a peer-to-peer network. This study attempted to assess pediatricians’ thoughts and attitudes toward introducing blockchain technology into the medical field.
Methods This study used a questionnaire survey to examine the thoughts and attitudes of 30- to 60-year-old pediatricians regarding the introduction of blockchain technology into the medical field. Responses to each item were recorded on a scale ranging from 1 (never agree) to 7 (completely agree).
Results The scores for the intentions and expectations of using blockchain technology were 4.0 to 4.6. Pediatricians from tertiary hospitals responded more positively (4.5–4.9) to the idea of using blockchain technology for hospital work relative to the general population (4.3–4.7). However, pediatricians working in primary and secondary hospitals had a slightly negative view of the application of blockchain technology to hospital work (p=0.018).
Conclusion When introducing the medical records of related pediatric and adolescent patients using blockchain technology in the future, it would be better to conduct a pilot project that prioritizes pediatricians in tertiary hospitals. The cost, policy, and market participants’ perceptions are essential factors to consider when introducing technology in the medical field.
Background Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe, life-threatening inflammatory condition if untreated. We aimed to investigate the etiologies, outcomes, and risk factors for death in children and adults with HLH.
Methods The medical records of patients who met the HLH criteria of two regional university hospitals in Korea between January 2001 and December 2019 were retrospectively investigated.
Results Sixty patients with HLH (35 children and 25 adults) were included. The median age at diagnosis was 7.0 years (range, 0.1–83 years), and the median follow-up duration was 8.5 months (range, 0–204 months). Four patients had primary HLH, 48 patients had secondary HLH (20 infection-associated, 18 neoplasm-associated, and 10 autoimmune-associated HLH), and eight patients had HLH of unknown cause. Infection was the most common cause in children (14/35, 40.0%), whereas neoplasia was the most common cause in adults (13/25, 52.0%). Twenty-eight patients were treated with HLH-2004/94 immunochemotherapy. The 5-year overall survival (OS) rate for all HLH patients was 59.9%. The 5-year OS rates for patients with primary, infection-associated, neoplasm-associated, autoimmune-associated, and unknown cause HLH were 25.0%, 85.0%, 26.7%, 87.5%, and 62.5%, respectively. Using multivariate analysis, neoplasm-induced HLH (p=0.001) and a platelet count <50×109/L (p=0.008) were identified as independent risk factors for poor prognosis in patients with HLH.
Conclusion Infection was the most common cause of HLH in children, while it was neoplasia in adults. The 5-year OS rate for all HLH patients was 59.9%. HLH caused by an underlying neoplasm or a low platelet count at the time of diagnosis were risk factors for poor prognosis.
Citations
Citations to this article as recorded by
Prevalence and mortality of haemophagocytic lymphohistiocytosis in dengue fever: a systematic review and meta-analysis Leong Tung Ong, Roovam Balasubramaniam Transactions of The Royal Society of Tropical Medicine and Hygiene.2024; 118(11): 711. CrossRef
Predicting relapsed/refractory disease in childhood hemophagocytic lymphohistiocytosis based on clinical features at diagnosis: A 13-year single-institute retrospective study in Thailand Pattranan Kusontammarat, Chane Choed-Amphai, Lalita Sathitsamitphong, Watchareewan Sontichai, Rungrote Natesirinilkul, Pimlak Charoenkwan Annals of Hematology.2024; 103(10): 3963. CrossRef
Autoimmune encephalitis followed by hemophagocytic lymph histiocytosis: a case report Li Huang, Jie Tan, Peihao Lin, Zixuan Chen, Qihua Huang, Haiyan Yao, Lihong Jiang, Baoyi Long, Youming Long Frontiers in Immunology.2024;[Epub] CrossRef
Hemophagocytic Lymphohistiocytosis Triggered by Herpes Simplex Virus 1 and 2: A Narrative Review Andria Papazachariou, Petros Ioannou Hematology Reports.2024; 16(3): 487. CrossRef
Spontaneous Resolution of Hemophagocytic Lymphohistiocytosis in a Child Infected with Epstein–Barr Virus Rita Alfattal, Hussain Sadeq, Abdullah Ali Journal of Applied Hematology.2023; 14(1): 57. CrossRef
Pediatric inborn errors of immunity causing hemophagocytic lymphohistiocytosis: Case report and review of the literature María Soledad Caldirola, Andrea Gómez Raccio, Daniela Di Giovanni, María Isabel Gaillard, María Victoria Preciado Journal of Leukocyte Biology.2022; 112(4): 607. CrossRef
Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature Bruno Fattizzo, Marta Ferraresi, Juri Giannotta, Wilma Barcellini Journal of Clinical Medicine.2021; 10(4): 870. CrossRef
HLH-Like Syndrome and Rhabdomyolysis in an Adolescent Patient Lauren T. Maloney, Bronwyn Baz, Dia Hazra Pediatrics.2021;[Epub] CrossRef
Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a self-limiting lymphadenitis. It is a benign disease mainly characterized by high fever, lymph node swelling, and leukopenia. Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease with clinical symptoms similar to those of KFD, but it requires a significantly more aggressive treatment. A 19-year-old Korean male patient was hospitalized for fever and cervical lymphadenopathy. Variable-sized lymph node enlargements with slightly necrotic lesions were detected on computed tomography. Biopsy specimen from a cervical lymph node showed necrotizing lymphadenitis with HLH. Bone marrow aspiration showed hemophagocytic histiocytosis. The clinical symptoms and the results of the laboratory test and bone marrow aspiration met the diagnostic criteria for HLH. The patient was diagnosed with macrophage activation syndrome—HLH, a secondary HLH associated with KFD. He was treated with dexamethasone (10 mg/m2/day) without immunosuppressive therapy or etoposide-based chemotherapy. The fever disappeared within a day, and other symptoms such as lymphadenopathy, ascites, and pleural effusion improved. Dexamethasone was reduced from day 2 of hospitalization and was tapered over 8 weeks. The patient was discharged on day 6 with continuation of dexamethasone. The patient had no recurrence at the 18-month follow-up.
Citations
Citations to this article as recorded by
Histiocytic necrotizing lymphadenitis with hemophagocytic lymphohistiocytosis in adults: A single‐center analysis of 5 cases Qingqing Chen, Jing Zhang, Huijun Huang, Tonglu Qiu, Ze Jin, Yu Shi, Huayuan Zhu, Lei Fan, Jianyong Li, Wenyu Shi, Yi Miao Immunity, Inflammation and Disease.2024;[Epub] CrossRef
A young Saudi female with combined hemophagocytic lympho-histiocytosis and Kikuchi’s disease: A case report Kamal Al-Zahrani, Batol Gasmelseed, Hesham Waaer Shadi, Rehab Y AL-Ansari SAGE Open Medical Case Reports.2023;[Epub] CrossRef
Cefalea y fiebre: no todo es lo que parece María Pilar Iranzo-Alcolea, Carmen Ariño-Palao, Grisell Starita-Fajardo, Andrés González-García, Cecilia Suárez-Carantoña Revista Española de Casos Clínicos en Medicina Interna.2023; 8(2): 105. CrossRef
Kikuchi–Fujimoto disease: literature review and report of four cases V. G. Potapenko, V. V. Baykov, А. Yu. Markova, N. B. Mikhailova, A. S. Ter‑Grigoryan, Yu. А. Krivolapov Oncohematology.2022; 17(4): 48. CrossRef
We report the case of a 16-month-old patient with chronic immune thrombocytopenia (ITP) patient who experienced delayed treatment-free response (TFR) after romiplostim treatment. He received intravenous immunoglobulin every month to maintain a platelet count above 20,000/μL for 2 years. Thereafter, he received rituximab and cyclosporine as second-line therapy, with no response, followed by romiplostim. After 4 weeks of treatment, the platelet count was maintained above 50,000/μL. Following 7 months of treatment, he discontinued romiplostim, and the platelet count decreased. His platelet counts remained above 50,000/μL, without any bleeding symptoms, 2 years after romiplostim discontinuation. This is the first report of TFR after romiplostim treatment in pediatric chronic ITP.
Citations
Citations to this article as recorded by
Tapering and Sustained Remission of Thrombopoietin Receptor Agonists (TPO-RAs): Is it Time for Paediatric ITP? Susana Marcos-Peña, Beatriz Fernández-Pernia, Drew Provan, Tomás José González-López Advances in Therapy.2024; 41(10): 3771. CrossRef
A cost–utility analysis of thrombopoietin receptor agonists for treating pediatric immune thrombocytopenia purpura after failure of first‐line therapies Huimin Du, Jiamin Wang, Joel Livingston, Ziyad Alrajhi, Melanie Kirby‐Allen, Brian Chan, Rebecca Hancock‐Howard, Peter C. Coyte Pediatric Blood & Cancer.2023;[Epub] CrossRef
Generic romiplostim for children with persistent or chronic immune thrombocytopenia: Experience from a tertiary care centre in North India Chandana Mareddy, Manas Kalra, Anupam Sachdeva British Journal of Haematology.2022; 197(5): 618. CrossRef
Tapering of the thrombopoietin receptor agonist in paediatric patients with chronic immune thrombocytopenia: Is it possible? María Solsona, Rubén Berrueco, Elena Sebastián, Áurea Cervera, Ana Sastre, Itziar Astigarraga, Bienvenida Argilés, María Ángeles Dasí, José Luís Dapena, Emilio Monteagudo British Journal of Clinical Pharmacology.2022; 88(9): 4220. CrossRef
BACKGROUND Neutropenic fever is one of the most common and potentially severe complications of chemotherapy in pediatric oncology patients, while urinary tract infection (UTI) is one of the most prevalent bacterial infections in these patients. Therefore, this study was conducted to investigate features of UTI with neutropenic fever in pediatric oncology patients. METHODS: We retrospectively reviewed and analyzed the medical records, laboratory results and image findings of cases of neutropenic fever in the Department of Pediatrics of Yeungnam University Medical Center, South Korea between November 2013 and May 2015. Episodes were divided into two groups, UTI vs. non-UTI group according to the results of urine culture. The results were then compared between groups. The analysis was performed using IBM SPSS 23.0. A p-value <0.05 was considered to indicate a significant difference between groups. RESULTS: Overall, 112 episodes of neutropenic fever were analyzed, among which 22 episodes (19.6%) showed organisms on urine culture and were classified as UTI. The remaining 90 episodes were classified as non-UTI. Only four episodes (18.2%) of the UTI group showed pyuria on urine analysis. In the UTI group, 76.5% were sensitive to the first line antibiotics and showed higher clinical response than the non-UTI group. Among hematologic malignancy patients, the UTI group revealed higher serum β 2-microglobulin levels than the non-UTI group (1.56±0.43 mg/L vs. 1.2±0.43 mg/L, p<0.028). CONCLUSION: UTI in pediatric neutropenic fever responds well to antibiotics. Hematologic malignancy cases with UTI reveal increased serum β2-microglobulin level. These results will be helpful to early phase diagnosis of UTI.
Central nervous system (CNS) prophylaxis is an essential component of the treatment in childhood acute lymphoblastic leukemia (ALL). Methotrexate (MTX) is an dispensable antimetabolite for treatment of ALL. High-dose (HD) MTX and intrathecal (IT) MTX have improved the prognosis and reduced the rate of CNS relapse. However, the drug also has a significant toxic effect on the CNS and can potentially lead to severe neurologic morbidity. The overall incidence of acute MTX induced neurotoxicity has been estimated to be 0.8∼10% of treated children, depending on the amounts of MTX and leucovorin in the treatment protocol. Acute neurotoxicity generally develops within 5∼14 days after IT MTX or HD MTX and may include headache, nausea, emesis, lethargy, altered mental status, blurred vision, aphasia, hemiparesis, and seizure. Diffusion weighted MRI shows restricted diffusion of water in brains of patients with ALL who experienced stroke-like event after IT MTX. We report the cinical and imaging findings of acute neurotoxicity in two patients after intrathecal administration of MTX for CNS prophylaxes of ALL.