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1. Risk factors for neurologic sequelae in children and adolescents with hemophilia after intracranial hemorrhage

Author

Melike Sezgin Evim, Ayşegül Ünüvar, Canan Albayrak, Emine Zengin, Ebru Yılmaz, Zühre Kaya, Nihal Karadaş, Mehtap Ertekin, Hülya Üzel, Gül Nihal Özdemir, Davut Albayrak, Funda Tayfun Küpesiz, Ayşenur Bahadır, Hüseyin Tokgöz, Kamuran Karaman, Barış Yılmaz, Sinan Akbayram, Burçak Tatlı Güneş, Burcu Belen Apak, Can Acıpayam, Yusuf Ziya Aral, Serap Karaman, and Hale Ören

Subjects
adolescents, children, hemophilia, intracranial hemorrhage, outcome, Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Background: Intracranial hemorrhage (ICH) is reportedly rare but has high morbidity and mortality risk in persons with hemophilia. Although the risk factors that facilitate bleeding are known, the factors affecting the sequelae are not well known. Objectives: We planned to investigate the risk factors for neurologic sequelae in children and adolescents with hemophilia suffering from ICH. Methods: An invitation was sent to pediatric hematology centers via email. Clinical and laboratory findings, neurologic sequelae, and recurrence of bleeding in persons with hemophilia who developed ICH were questioned. Results: Eighty-six patients from 21 centers were evaluated. All patients were less than 18 years of age at the time of ICH. Thirteen patients had ICH in the neonatal period, while 40 patients had a known diagnosis of hemophilia before ICH, and 33 patients were undiagnosed before ICH. Five patients died, 2 of whom died in the neonatal period. The rate of neurologic sequelae was 25 of 81 (30%). The most common neurologic sequela was epilepsy (n = 11/25), followed by hemiparesis (n = 5/25). Cerebral shift (odds ratio, 3.48) and development of ICH in the neonatal period (odds ratio, 4.67) were significant for the development of neurologic sequelae in multivariate analysis. On follow-up, recurrence of ICH occurred in 8 of 81 (10%). Conclusion: ICH in the neonatal period and cerebral shift were the two main risk factors for the development of neurologic sequelae. Neonatal departments must be alert to the signs of bleeding. It is important for healthcare professionals to overcome the barriers to primary prophylaxis and to take trauma-related precautions.

Published
2024
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2. Retrospective analysis of hemophilia B in Turkey: identifying main characteristics and treatment options

Author

Bülent Zülfikar, Başak Koç, Fahri Şahin, Hatice İlgen Şaşmaz, Kaan Kavaklı, Can Balkan, Ali Bülent Antmen, Sinan Akbayram, Birol Güvenç, Vahap Okan, Emine Türkkan, Canan Albayrak, Davut Albayrak, Nazan Sarper, Tülin Tiraje Celkan, Orhan Ayyıldız, Salih Aksu, Türkan Patıroğlu, Zafer Şalcıoğlu, Adalet Meral Güneş, Yasemin Altuner Torun, Ümran Çalışkan, Hüseyin Tokgöz, Yılmaz Ay, Gül Nihal Özdemir, Mehmet Sönmez, Ekrem Ünal, Ahmet Fayik Öner, Nil Güler, Osman Alphan Küpesiz, Hale Ören, Serap Karaman, Ayşegül Ünüvar, Mehmet Dağlı, Ahmet Muzaffer Demir, Murat Söker, Bülent Alioğlu, Zühre Kaya, Aylin Canbolat Ayhan, Zafer Bıçakçı, Yusuf Ziya Aral, and Muhlis Cem Ar

Subjects
factor IX, hemophilia B, joint diseases, prophylaxis, retrospective studies, surgery, Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Background: Hemophilia B (HB), an X-linked recessive inherited bleeding disorder, exhibits a high prevalence among males. Objectives: To present the first national cohort of persons with HB to define the demographics, clinical characteristics, and treatment patterns in Turkey. Methods: This multicenter, retrospective study included 433 alive persons with HB registered in 35 centers between 1961 and 2018. Analyses were performed by age subgroups (0-17 years, 18-64 years, and ≥65 years), disease severity by factor levels (severe, 5 IU/dL). Additionally, patients were stratified based on the initiation year of follow-up at the relevant study center, creating 2 periods: 1993-2006 (referred to as period A) and 2007-2018 (referred to as period B). Results: Predominantly male (98.6%), the median age at data entry was 22.1 years (n = 429). The majority (49.0%) had moderate HB, followed by severe (30.0%) and mild (15.7%) disease. Of the 377 patients with complete treatment details, 209 (55.4%) were under prophylaxis from their diagnosis onwards, while 79 patients (21.0%) only received on-demand treatment. Additionally, 89 patients (23.6%) initially underwent on-demand treatment and later were switched to prophylaxis. Knees were the primary site of bleeding and the most frequently intervened joints. Most of the major (47.5%) and minor (53.3%) orthopedic procedures were carried out in persons with severe HB, while half of radioactive synovectomy procedures were performed on persons with moderate HB. Conclusion: This paper describes the demographics, clinical characteristics, and treatments patterns of a large cohort of alive persons with HB on a national scale.

Published
2024
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3. Castleman Disease: A Multicenter Case Series from Turkey

Author

Eren Gündüz, Hakkı Onur Kırkızlar, Elif Gülsüm Ümit, Sedanur Karaman Gülsaran, Vildan Özkocaman, Fahir Özkalemkaş, Ömer Candar, Tugrul Elverdi, Selin Küçükyurt, Semra Paydaş, Özcan Çeneli, Sema Karakuş, Senem Maral, Ömer Ekinci, Yıldız İpek, Cem Kis, Zeynep Tuğba Güven, Aydan Akdeniz, Tiraje Celkan, Ayşe Hilal Eroğlu Küçükdiler, Gülsüm Akgün Çağlıyan, Ceyda Özçelik Şengöz, Ayşe Karataş, Tuba Bulduk, Alper Özcan, Fatma Burcu Belen Apak, Aylin Canbolat, İbrahim Kartal, Hale Ören, Ersin Töret, Gül Nihal Özdemir, and Şule Mine Bakanay Öztürk

Subjects
castleman disease, unicentric, multicentric, treatment, Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Objective: Castleman disease (CD) is a rare disease also known as angiofollicular lymph node hyperplasia. The two main histological subtypes are the hyaline vascular and plasma cell variants. It is further classified as unicentric CD (UCD) or multicentric CD (MCD) according to the anatomical distribution of the disease and the number of lymph nodes involved. The aim of this multicenter study was to evaluate all cases of CD identified to date in Turkey to set up a national registry to improve the early recognition, treatment, and follow-up of CD. Materials and Methods: Both adult (n=130) and pediatric (n=10) patients with lymph node or involved field biopsy results reported as CD were included in the study. Patients' demographic information, clinical and laboratory characteristics, imaging study results, treatment strategies, and clinical outcomes were evaluated retrospectively. Results: A total of 140 patients (69 male and 71 female) with a diagnosis of UCD (n=73) or MCD (n=67) were included. The mean age was 39 years in the UCD group and 47 years in the MCD group. Female patients were more common in the UCD group. The most common histological subtype was hyaline vascular for both UCD and MCD patients. Asymptomatic patients were more common in the UCD group. Anemia, elevations of acute phase reactants, and hypoalbuminemia were more common in the MCD group. The most commonly used treatment strategies for UCD were surgical excision, rituximab, and radiotherapy, respectively. All UCD patients were alive at a median of 19.5 months of follow-up. The most commonly used treatment strategies for MCD were methyl prednisolone, R-CHOP, R-CVP, and rituximab. Thirteen MCD patients had died at a median of 34 months of follow-up. Conclusion: This study is important in presenting the patient characteristics and treatment strategies for CD from Turkey, with the potential of increasing awareness about CD. Treatment data may help in making decisions, particularly in countries that do not have access to siltuximab. However, larger prospective studies are needed to make definitive conclusions.

Published
2022
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4. Gaucher Disease for Hematologists

Author

Gül Nihal Özdemir and Eren Gündüz

Subjects
gaucher disease, anemia, thrombocytopenia, splenomegaly, Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Gaucher disease (GD) is a rare hereditary lysosomal storage disease that arises due to deficiency of glucocerebrosidase. Early diagnosis is very important for starting proper treatment and preventing complications. Splenomegaly, anemia, and thrombocytopenia are the most common findings in GD and so most patients are initially referred to hematologists. The Turkish Society of Hematology established its Rare Hematological Diseases Subcommittee in 2015. One of the main topics of this subcommittee was to increase and improve awareness and education of rare diseases among hematologists in Turkey. This review presents GD with an overview of its clinical features, pathophysiology, and treatment options for hematologists.

Published
2022
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5. Twelve-month observational study of children with cancer in 41 countries during the COVID-19 pandemic

Author

Md Hasanuzzaman, Mohamed Ahmed, Ahmed Samir, Charlotte Smith, Lubna Samad, Vaishnavi Govind, Fakher Rahim, Ahmed Moussa, Adesoji O Ademuyiwa, Bobby John, Augusto Zani, Vivek Singh, Muhammad Arshad, Sadaf Altaf, Chan Hon Chui, Pooja Kumari, Thomas Smith, Ayesha Saleem, Matthew HV Byrne, Madhivanan Karthigeyan, Pravin Salunke, Darica Au, Kate Cross, Kokila Lakhoo, Vishal Kumar, Anna Maria Testi, Robyn Brown, Noel Peter, Georgios Tsoulfas, Francesco Pata, Adesoji Ademuyiwa, Tahmina Banu, Bruce Bvulani, Milind Chitnis, Maryam Ghavami Adel, Vrisha Madhuri, Pierfrancesco Lapolla, Andrea Mingoli, Hamidah Alias, Simone de Campos Vieira Abib, Ibukunolu Olufemi Ogundele, Felix M Alakaloko, Emmanuel A Ameh, Laila Hessissen, Kareem O Musa, Georgios Karagiannidis, Manoj Gupta, Maricarmen Olivos, Daniel Rhee, Maryam Khan, Christine Nitschke, Alexandra Valetopoulou, Ashrarur Rahman Mitul, Sabbir Karim, Mahmoud M Saad, Francis Abantanga, Gaetano Gallo, Mohamedraed Elshami, Mahmoud Elfiky, Soham Bandyopadhyay, Muath Alser, Elliott H Taylor, Duha Jasim, Somy Charuvila, Nazmul Islam, William B Lo, Uttam Kumar Nath, Robin Simpson, Zarina Abdul Latiff, Bruno Cirillo, Gioia Brachini, Megan Murphy, Zineb Bentounsi, Anette S Jacobsen, Anna Casey, Mohammed Alhendy, Taiwo Akeem Lawal, Samson Olori, Michael Boettcher, Muhammed Elhadi, Shaun Wilson, Dragana Janić, Amit Sehrawat, Patricia Shinondo, Shireen Anne Nah, Alhassan Abdul-Mumin, Karl-Heinz Frosch, Poorvaprabha Patil, Sarah Muma, Md Asaduzzaman, Athanasios Tragiannidis, Vijayendra Kumar, Mahan Salehi, Sara Ali, Renu Madan, Hafeez Abdelhafeez, Max Pachl, Benjamin Martin, Sonal Nagras, Mihir Sheth, Catherine Dominic, Suraj Gandhi, Divya Parwani, Rhea Raj, Diella Munezero, Rohini Dutta, Nsimire Mulanga Roseline, Kellie McClafferty, Armin Nazari, Smrithi Sriram, Sai Pillarisetti, King-David Nweze, Aishwarya Ashwinee, Gul Kalra, Priyansh Nathani, Khushman Kaur Bhullar, Nehal Rahim, Shweta Madhusudanan, Joshua Erhabor, Manasi Shirke, Aishah Mughal, Sravani Royyuru, Syeda Namayah Fatima Hussain, Daniel Robinson, Mehdi Khan, Alexandre Dukundane, Kwizera Festus, Rohan Pancharatnam, Lorraine Ochieng, Hritik Nautiyal, Leanne Gentle, Ehab Hanafy, Catherine Yang, Gideon Karplus, John Mathew, Olumide Abiodun Elebute, Oluwaseun Ladipo-Ajayi, Okechukwu Hyginus Ekwunife, Sherief Ghozy, Emily Hamilton, Dhruva Ghosh, Ahmed Sherif, Hajar Moujtahid, Ariana Axiaq, Amir Labib, Eman Abdulwahed, Kemal Tolga Saracoglu, Yasin Kara, Ahmed Y Azzam, Omar Elmandouh, Manjul Tripathi, Abdelrahman Azzam, Anfel Bouderbala, Aouabed Nesrine, Ammar Ayman, Mohamed Bonna, Safia Lorabi, Hira Zuberi, Iyad Sultan, Reto M Baertschiger, Kefas John Bwala, AM Umar, Abdurahaman Aremu, Dauda E Suleiman, Tybat Aliyu, Kashaf Turk, Oluseyi Oyebode Ogunsua, Tunde Talib Sholadoye, Musliu Adetola Tolani, Yakubu Alfa, Keffi Mubarak Musa, Ken Muma, Mitchelle Obat, Youssef Sameh Badran, Abdulrahman Ghassan Qasem, Faris Ayasra, Reema Alnajjar, Mohamed Abdel-Maboud, Abdelrahman Bahaa, Ayat M Saadeldin, Mohamed Adwi, Mahmoud Adly, Abdallah Elshenawy, Amer Harky, Kirstie Wright, Jessica Luyt, Olivia White, Nathan Thompson, Imogen Harrison, Sara Kader Alsaeiti, Fatma Saleh Benkhial, Hend Mohammed Masoud, Mabroukah Saeid Alshamikh, Fatma Mohammed Masoud, Nyararai Togarepi, Elaine Carrolan, Ahmed Saleh, Mahmoud Bassiony, Mostafa Qatora, Mohamed Bahaaeldin, Shady Fadel, Yasmine El Chazli, Kamel Hamizi, Mehdi Anouar Zekkour, Rima Rahmoun, Boutheyna Drid, Salma Naje Abu Teir, Mohamed Yazid Kadir, Yassine Zerizer, Nacer Khernane, Brahim Saada, Imane Ammouze, Yahya Elkaoune, Ghita Chaoui, Hajar Benaouda, Meryem Gounni, Narjiss Aji, Joana Mafalda Monteiro, Susana Nunes, Maria do Bom-Sucesso, Kerri Becktell, Md Afruzul Alam, Orindom Shing Pulock, Tasmiah Tahera Aziz, Rosanda Ilic, Danica Grujicic, Tijana Nastasovic, Igor Lazic, Mihailo Milicevic, Vladimir Bascarevic, Radovan Mijalcic, Vuk Scepanovic, Aleksandar Stanimirovic, Aleksandra Paunovic, Ivan Bogdanovic, Shahnoor Islam, AKM Amirul Morshed, Mehnaz Akter, Zannat Ara, Mohammed Tanvir Ahammed, Tania Akter, Kamrun Nahar, Fatema Sayed, Ashfaque Nabi, Elif Akova, Evren Aydogmus, Bekir Can Kendirlioglu, Tufan Hicdonmez, Asim Noor Rana, Mohammed A Azab, Alzhraa Salah Abbas, Olanrewaju Moses, Ibiyeye Taiye Taibat, Taiwo Jones, Kalu Ukoha, Olagundoye Goke, Okorie Ikechukwu, Abiodun Idowu Okunlola, Helga Nauhaus, Danelle Erwee, Agata Chylinska, Prasanna Gomes, Elvercio Pereira de Oliveira Junior, Fabiola Leonelli Diz, Mohamed El Kassas, Usama Eldaly, Ahmed Tawheed, Mohamed Abdelwahab, Oudrhiri Mohammed Yassaad, Bechri Hajar, El Ouahabi Abdessamad, Arkha Yasser, Hessissen Laila, Farah Sameer Yahya, Maria Teresa Peña Gallardo, Jacqueline Elizabeth Montoya Vásquez, Juan Luis García León, Sebastián Shu Yip, Mariam Lami, Harmit Ghattaura, Eric W Etchill, Stacy Cooper, Kevin Crow, Morgan Drucker, Benjamin Shou, Alan Siegel, Gül Nihal Özdemir, Ehab El Refaee, John George Massoud, Ayah Bassam Ibrahim, Ruaa Bassam Ibrahim, Faris Abu Za'nouneh, Toqa Fahmawee, Ghazwani Salman, Ehab Alameer, Al-Mudeer Ali, Ghazwani Yahia, Khozairi Waleed, Khalil Ghandour, Shaima' Al-Dabaibeh, Ammar Al-Basiti, Hazim Ababneh, Omaima El-Qurneh, Yousef Alalawi, Ahmad Al Ayed, Naif Al Bolowi, Heidi Barola, Aubrey L Pagaduan, Jingdan Fan, Olufemi Oni, Janita Zarrish, Ramsha Saleem, Soha Zahid, Atiqa Amirali, Ahsan Nadeem, Sameer Saleem Tebha, Zonaira Qayyum, Sana Tahir, Anneqa Tahir, Rabbey Raza Khan, Ayesha Mehmood, Taimur Iftikhar Qureshi, Victor Calvagna, Nathalie Galea, Matthew R Schuelke, Kirk David Wyatt, Agnes Vojcek, Seham M Ragab, Abdallah R Allam, Eman Ibrahim Hager, Kıvılcım Karadeniz Cerit, Adnan Dağçınar, Tümay Umuroğlu, Ayten Saraçoğlu, Mustafa Sakar, Can Kıvrak, Gül Çakmak, Ibrahim Sallam, Gamal Amira, Mohamed Sherief, Arissa Ikeda, Licia Portela, Marianne Monteiro Garrigo, Fernanda Lobo, Sima Ester Ferman, Andrew Nwankwo Osuigwe, Chisom Adaobi Nri-Ezedi, Eric Okechukwu Umeh, Abiodun Folashade Adekanmbi, Olubunmi Motunrayo Fatungase, Olubunmi Obafemi Obadaini, Sarah Al-Furais, Humaida Hemlae, Sreylis Nay, Fabianne Altruda de Moraes Costa Carlesse, Denis Cozzi, Paolo Musiu, Paolo Sapienza, Martina Zambon, Simona Meneghini, Pierfranco Cicerchia, Abdulrahman Omar Taha, Bouaoud Souad, Mebarki Malika, Bioud Belkacem, Fayza Haider, Halwani Yaninga Fuseini, Peter Gyamfi Kwarteng, Abubakari Bawa Abdulai, Sheba Mary Pognaa Kunfah, Stephanie Ajinkpang, Mary Joan Kpiniong, Kingsley Aseye Hattor, Kingsley Appiah Bimpong, Mohamed Elbahnasawy, Sherief Abdelsalam, Amanpreet Brar, Andreea C Matei, Hira Khalid Zuberi, Kishwer Nadeem, Naema Khayyam, Fatima Ambreen Imran, Nida Zia, Sadia Muhammad, Muhammad Rafie Raza, Muhammad Rahil Khan, Alaa Hamdan, Abdeljawad Mazloum, Ali Abodest, Nisreen Ali, Ammar Omran, Alaa Ahmed, Munawar Hraib, Victor Khoury, Abdulrahman Almjersah, Mohammad Ali Deeb, Akram Ahmed, Ahmad Bouhuwaish, Alqasim Abdulkarim, Marwa Biala, Reem Ghamgh, Amani Alamre, Marwa Shelft, Hoda Tawel, Emmanuel Hatzipantelis, Eleni Tsotridou, Assimina Galli-Tsinopoulou, C-Khai Loh, Doris Lau, Kelvin Ifeanyichukwu Egbuchulem, Olakayode Olaolu Ogundoyin, Isaac Dare Olulana, Oluwasegun Joshua Afolaranmi, AbdulBasit Fehintola, Annika Heuer, Matthias Priemel, Lennart Viezens, Martin Stangenberg, Marc Dreimann, Alonja Reiter, Jasmin Meyer, Leon Köpke, Uduak Offiong, Philip Mari Mshelbwala, Fashie Andrew Patrick, Aminu Muhammed Umar, N Otene ThankGod, Yuki Julius Ng, Syukri Ahmad Zubaidi, Murad Almasri, Rasaq Olaosebikan, Akila Muthukumar, Amon Ngongola, Azad Patel, Abdullahi Nuhu-Koko, Baba Jibrin, Gabriela Guillén, Sergio López, José Andrés Molino, Pablo Velasco, Omar Hamam, Rim Elmandouh, Nensi Melissa Ruzgar, Rachel Levinson, Shashwat Kala, Sarah Ullrich, Emily Christison-Lagay, Janice Hui Ling Wong, Reto Baertschiger, Essam Elhalaby, Guido Seitz, Judith Lindbert, Asimina Galli-Tsinopoulou, Calogero Virgone, Eric Mwangi Irungu, Outani Oumaima, Lily Saldana, Jan Godzinsky, Abdelbasit Ali, Mohamed Bella Jalloh, Nellie Bell, Annette Jacobsen, Israel Fernandez Pineda, Lucas Krauel, Waha Rahama, Hazim Elfatih, Arda Isik, Andrea Hayes-Jordan, Roshni Dasgupta, Krishna Kumar Govindarajan, Marta deAndres Crespo, Nitin James Peters, Santosh Kumar Mahalik, Rajat Piplani, Enono Yhoshu, K S Rajkumar, Sadi A Abukhalaf, Mohammed Miftah Faraj Almihashhish, Eman Salem Muftah Burzeiza, Raja Mari Mohammed Nasef, Benjamin J O'Sullivan, Mohamed Hassanin, Dave R Lal, Brian T Craig, Vishal Michael, M Joseph John, William Bhatti, Swati Daniel, Jyoti Dhiman, Hunar Mahal, Atul Suroy, Shruti Kakkar, Shaina Kamboj, Suraj Singh, AKM Khairul Basher, SM Rezanur Rahman, Md Asif Iqbal, Md Masud Rana, Monica Dobs, Mohamed Atef Mohamed Ghamry, Joana Monteiro, Marco Aurelio Ciriaco Padilha, Lucas Garschagen deCarvalho, Sandip Kumar Rahul, Digamber Chaubey, Rejin Kebudi, Sema Bay Buyukkapu, Kumaravel Sambandan, Smita Kayal, Gunaseelan Karunanithi, Bikash Kumar Naredi, Bibekanand Jindal, Ranya M Baddourah, Ayah Al Shraideh, Ahmad Ozair, Ankur Bajaj, Bal Krishna Ojha, Kaushal Kishor Singh, Atique Anwar, Vinay Suresh, Mohamad K Abou Chaar, Christopher O Bode, Justina O Seyi-Olajide, George C Ihediwa, Edamisan O Temiye, Adeseye M Akinsete, Iqra Effendi, Khaled Mamdouh, Mohamed Atef, Mohamed Faried, Jake A Kloeber, Robert L Owen, Alexander S Roth, J Hudson Barnett, Lucien P Jay, Paul J Galardy, Bernard Mbwele, Irene Nguma, Moshi Moshi Shabani, Amani Twaha, Bilal Matola, Mahmoud Maher Abdelnaby Alrahawy, Simone deOliveira Coelho, Ricardo Vianna deCarvalho, FernandaFerreira daSilva Lima, Moawia Mohammed AliElhassan, Nada Osman Yousif Elhaj, Hytham KS Hamid, Vincent E Nwatah, Adewumi B Oyesakin, RM Jeffri Ismail, Simone deCamposVieira Abib, Mayara Caroline Amorim Fanelli, Fernanda Kelly Marques de Souza, Sandeep Mohindra, Ninad R Patil, Richa Jain, Gopal Nambi, Norehan Johari, Anas Shikha, Win SabaiPhyu Han, Zahidah Ahmad, Yen Yan Lim, Roserahayu Idros, Noorainun Mohd Yusof, David Nelson Jaisingh, Fatema Naser AlFayez, Elana Kleinman, Taylor Ibelli, Rochelle Fayngor, Tzvi Najman, Etai Adam, Daniella Melamed, Cecilia Paasche, Farman Ali Laghari, Zainab Al Balushi, Abdulhakim Awadh SalimAl-Rawas, Ali Al Sharqi, Ammar Saif AlShabibi, Ismail Al Bulushi, Muna Alshahri, Abdulrahman AlMirza, Ola Al Hamadani, Jawaher Al Sharqi, Anisa Al Shamsi, Bashar Dawud, Sareya Al Sibai, Gilbert B Bonsaana, Edmund M Der, Francis A Abantanga, Bardisan Gawrieh, Hassan Salloum, Mohammad Ahmad Almahmod Alkhalil, Waseem Shater, Ali Farid Alelayan, Alaa Guzlan, Asmaa AM Albanna, Dayang AnitaAbdul Aziz, Azrina Syarizad Khutubul Zaman, Biobele J Brown, Ajiboye L Olalekan, Christopher S Lukong, Ezekiel I Ajayi, Luca Pio, Nitin James Peter, Ravi Kishore, Mohammad K Abou Chaar, Dayang Anita Abdul Aziz, Dhruva Nath Ghosh, and Raphael N Vuille-dit-Bille

Subjects
Medicine (General), R5-920, Infectious and parasitic diseases, RC109-216
Abstract

Introduction Childhood cancer is a leading cause of death. It is unclear whether the COVID-19 pandemic has impacted childhood cancer mortality. In this study, we aimed to establish all-cause mortality rates for childhood cancers during the COVID-19 pandemic and determine the factors associated with mortality.Methods Prospective cohort study in 109 institutions in 41 countries. Inclusion criteria: children

Published
2022
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6. Outcomes of Eltrombopag Treatment and Development of Iron Deficiency in Children with Immune Thrombocytopenia in Turkey

Author

Ayça Koca Yozgat, Göksel Leblebisatan, Sinan Akbayram, Simge Çınar Özel, Zeynep Karakaş, Erol Erduran, Şebnem Yılmaz, Ülker Koçak, Şule Ünal, Gül Nihal Özdemir, Meryem Albayrak, Emine Zengin, Yeşim Oymak, Özcan Bör, Hasan Fatih Çakmaklı, Murat Söker, Dilek Gürlek Gökçebay, Hüseyin Tokgöz, Barış Malbora, Serap Karaman, Tiraje Celkan, İlgen Şaşmaz, Neşe Yaralı, Hale Ören, and Ayşegül Ünüvar

Subjects
immune thrombocytopenia, eltrombopag, iron deficiency, Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Objective: Immune thrombocytopenia (ITP) is a rare autoimmune disease and hematologic disorder characterized by reduced platelet counts that can result in significant symptoms, such as bleeding, bruising, epistaxis, or petechiae. The thrombopoietin receptor agonist eltrombopag (EPAG) is a second-line agent used to treat chronic ITP purpura in adults and children. Materials and Methods: The present retrospective study evaluated the efficacy, safety, and side effects of EPAG treatment in pediatric patients with acute refractory and chronic immune thrombocytopenia, particularly focusing on iron-deficiency anemia. Results: The diagnosis was chronic ITP in 89 patients and acute refractory ITP in 16 patients. The mean age of patients was 9.5+-4.5 years (minimum-maximum: 1.2-18 years) at the beginning of EPAG treatment. The overall response rate was 74.3% (n=78). The mean time for platelet count of ≥50x109/L was 11.6+-8 weeks (range: 1-34 weeks). The treatment was stopped for 27 patients (25.7%) at an average of 6.8+-9 months (range: 1-38 months). The reason for discontinuation was lack of response in 18 patients, nonadherence in 4 patients, and hepatotoxicity in 2 patients. Response to treatment continued for an average of 4 months after cessation of EPAG in 3 patients. Conclusion: Results of the current study imply that EPAG is an effective therapeutic option in pediatric patients with acute refractory and chronic ITP. However, patients must be closely monitored for response and side effects during treatment, and especially for iron deficiency.

Published
2020
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7. Impact of the COVID-19 pandemic on patients with paediatric cancer in low-income, middle-income and high-income countries: a multicentre, international, observational cohort study

Author

Mohamed Ahmed, Ahmad Mansour, Ahmed Samir, Charlotte Smith, Lubna Samad, Vaishnavi Govind, Fakher Rahim, Augusto Zani, Muhammad Arshad, Sadaf Altaf, Chan Hon Chui, Pooja Kumari, Thomas Smith, Ayesha Saleem, Darica Au, Kate Cross, Kokila Lakhoo, Anna Maria Testi, Robyn Brown, Noel Peter, Francesco Pata, Adesoji Ademuyiwa, Tahmina Banu, Bruce Bvulani, Milind Chitnis, Maryam Ghavami Adel, Matthew H V Byrne, Pierfrancesco Lapolla, Andrea Mingoli, Lucy Davies, Dennis Mazingi, Hamidah Alias, Simone de Campos Vieira Abib, Ibukunolu Olufemi Ogundele, Laila Hessissen, Mohammad Ahmad, Maricarmen Olivos, Daniel Rhee, Maryam Khan, Christine Nitschke, Alexandra Valetopoulou, Ashrarur Rahman Mitul, Sabbir Karim, Gaetano Gallo, Mohamedraed Elshami, Mahmoud Elfiky, Soham Bandyopadhyay, Muath Alser, Elliott H Taylor, Duha Jasim, Somy Charuvila, Nazmul Islam, William B Lo, Robin Simpson, Zarina Abdul Latiff, Bruno Cirillo, Gioia Brachini, Megan Murphy, Zineb Bentounsi, Anette S Jacobsen, Anna Casey, Abdulrasheed A Nasir, Taiwo Akeem Lawal, Samson Olori, Michael Boettcher, Muhammed Elhadi, Shaun Wilson, Dragana Janić, Patricia Shinondo, Shireen Anne Nah, Alhassan Abdul-Mumin, Dayang Anita Abdul Aziz, J Benjamin, Karl-Heinz Frosch, Poorvaprabha Patil, Sarah Muma, Emmanuel Uwiringiyimana, Athanasios Tragiannidis, Mahan Salehi, Sara Ali, Hafeez Abdelhafeez, Max Pachl, Benjamin Martin, Sonal Nagras, Mihir Sheth, Catherine Dominic, Suraj Gandhi, Divya Parwani, Rhea Raj, Diella Munezero, Rohini Dutta, Nsimire Mulanga Roseline, Kellie McClafferty, Armin Nazari, Smrithi Sriram, Sai Pillarisetti, Aishwarya Ashwinee, Gul Kalra, Priyansh Nathani, Khushman Kaur Bhullar, Nehal Rahim, Shweta Madhusudanan, Joshua Erhabor, Manasi Shirke, Aishah Mughal, Sravani Royyuru, Daniel Robinson, Mehdi Khan, Alexandre Dukundane, Kwizera Festus, Rohan Pancharatnam, Lorraine Ochieng, Hritik Nautiyal, Leanne Gentle, Ehab Hanafy, Catherine Yang, John Mathew, Olumide Abiodun Elebute, Oluwaseun Ladipo-Ajayi, Okechukwu Hyginus Ekwunife, Sherief Ghozy, Aya Zazo, Salah Eddine Oussama Kacimi, Ahmed Sherif, Krithi Ravi, Nermin Badwi, Hajar Moujtahid, Ariana Axiaq, Eman Abdulwahed, Kemal Tolga Saracoglu, Yasin Kara, Ahmed Y Azzam, Omar Elmandouh, KingDavid Nweze, Syeda Namayah Fatima Hussain, Marta de Andres Crespo, Fatumata Jalloh, Shahnur Shah, Rohini Rajpal, Masooma Rana, Muskaan Abdul Qadir, Abdelrahman Azzam, Mayara Fanelli, Gustavo Mendonça Ataíde Gomes, Igor Lima Buarque, Isadora Schwaab Guerini, Anfel Bouderbala, Sarah Alfurais, Mohamed Gamal, Yara Hijazi, Shatha Tailakh, Hamza AlNaggar, Zain Douba, Sewar Elejla, Abdullah Eldaly, Ekram Sharashi, Tamara Elyan, Aouabed Nesrine, Ammar Ayman, Mohamed Bonna, Safia Lorabi, Hassan Alalami, Rawan Yasser Emam, Mohamad K Abou Chaar, Hira Zuberi, Iyad Sultan, Dhruv Nath Ghosh, Nitin James Peters, Reto M Baertschiger, Kefas John Bwala, AM Umar, Abdurahaman Aremu, Dauda E Suleiman, Tybat Aliyu, Kashaf Turk, Oluseyi Oyebode Ogunsua, Tunde Talib Sholadoye, Musliu Adetola Tolani, Yakubu Alfa, Keffi Mubarak Musa, Mwangi Irungu, Ken Muma, Mitchelle Obat, Youssef Sameh Badran, Abdulrahman Ghassan Qasem, Faris Ayasra, Reema Alnajjar, Mohamed Abdel-Maboud, Abdelrahman Bahaa, Ayat M Saadeldin, Mohamed Adwi, Mahmoud Adly, Abdallah Elshenawy, Amer Harky, Kirstie Wright, Jessica Luyt, Olivia White, Nathan Thompson, Imogen Harrison, Sara Kader Alsaeiti, Fatma Saleh Benkhial, Mohammed Miftah Faraj Almihashhish, Eman Salem Muftah Burzeiza, Raja Mari Mohammed Nasef, Hend Mohammed Masoud, Mabroukah Saeid Alshamikh, Fatma Mohammed Masoud, Nyararai Togarepi, Elaine Carrolan, Mohamed Hassanin O'Sullivan, Ahmed Saleh, Mahmoud Bassiony, Mostafa Qatora, Mohamed Bahaaeldin, Shady Fadel, Yasmine El Chazli, Kamel Hamizi, Mehdi Anouar Zekkour, Rima Rahmoun, Boutheyna Drid, Salma Naje Abu Teir, Mohamed Yazid Kadir, Yassine Zerizer, Nacer Khernane, Brahim Saada, Imane Ammouze, Yahya Elkaoune, Ghita Chaoui, Hajar Benaouda, Meryem Gounni, Narjiss Aji, Joana Mafalda Monteiro, Susana Nunes, Maria do Bom-Sucesso, Dave R. Lal, Brian T. Craig, Kerri Becktell, Md Afruzul Alam, Orindom Shing Pulock, Tasmiah Tahera Aziz, Rosanda Ilic, Danica Grujicic, Tijana Nastasovic, Igor Lazic, Mihailo Milicevic, Vladimir Bascarevic, Radovan Mijalcic, Vuk Scepanovic, Aleksandar Stanimirovic, Aleksandra Paunovic, Ivan Bogdanovic, Shahnoor Islam, AKM Amirul Morshed, A. K. M. Khairul Basher, Mehnaz Akter, S. M. Rezanur Rahman, Zannat Ara, Mohammed Tanvir Ahammed, Tania Akter, Kamrun Nahar, Fatema Sayed, Ashfaque Nabi, Md. Asif Iqbal, Md. Masud Rana, Md. Asaduzzaman, Md. Hasanuzzaman, Elif Akova, Evren Aydogmus, Bekir Can Kendirlioglu, Tufan Hicdonmez, Arshiya Adhnon, Asim Noor Rana, Hani Humad, Anjan Madasu, Mohammed A Azab, Alzhraa Salah Abbas, Olanrewaju Moses, Ibiyeye Taiye Taibat, Taiwo Jones, Kalu Ukoha, Olagundoye Goke, Okorie Ikechukwu, Abiodun Idowu Okunlola, Helga Nauhaus, Danelle Erwee, Agata Chylinska, Prasanna Gomes, Marco Aurelio Ciriaco Padilha, Elvercio Pereira de Oliveira Junior, Lucas Garschagen de Carvalho, Fabiola Leonelli Diz, Mohamed El Kassas, Usama Eldaly, Ahmed Tawheed, Mohamed Abdelwahab, Oudrhiri Mohammed Yassaad, Bechri Hajar, El Ouahabi Abdessamad, Arkha Yasser, Hessissen Laila, Farah Sameer Yahya, Yasir Al-Agele, Maria Teresa Peña Gallardo, Jacqueline Elizabeth Montoya Vásquez, Juan Luis García León, Sebastián Shu Yip, Mariam Lami, Harmit Ghattaura, Eric W Etchill, Stacy Cooper, Kevin Crow, Morgan Drucker, Benjamin Shou, Alan Siegel, Gül Nihal Özdemir, Ehab El Refaee, John George Massoud, Ayah Bassam Ibrahim, Ruaa Bassam Ibrahim, Faris Abu Za'nouneh, Ranya M. Baddourah, Toqa Fahmawee, Ayah Al_Shraideh, Ghazwani Salman, Ehab Alameer, Al-Mudeer Ali, Ghazwani Yahia, Khozairi Waleed, Mohamad K. Abou Chaar, Khalil Ghandour, Shaima' Al-Dabaibeh, Ammar Al-Basiti, Hazim Ababneh, Omaima El-Qurneh, Yousef Alalawi, Ahmad Al Ayed, Naif Al Bolowi, Amos HP Loh, Heidi Barola, Aubrey L Pagaduan, Jingdan Fan, Adesoji O. Ademuyiwa, Christopher O. Bode, Justina O. Seyi-Olajide, Felix M. Alakaloko, George C. Ihediwa, Kareem O. Musa, Edamisan O. Temiye, Olufemi Oni, Adeseye M. Akinsete, Janita Zarrish, Ramsha Saleem, Soha Zahid, Atiqa Amirali, Ahsan Nadeem, Sameer Saleem Tebha, Zonaira Qayyum, Sana Tahir, Anneqa Tahir, Rabbey Raza Khan, Ayesha Mehmood, Taimur Iftikhar Qureshi, Victor Calvagna, Nathalie Galea, Matthew R Schuelke, Jake A. Kloebe, Robert L. Owen, Alexander S. Roth, J. Hudson Barnett, Lucien P. Jay, Kirk David Wyatt, Paul J. Galardy, Agnes Vojcek, Mahmoud Maher Abdelnaby Alrahawy, Seham M Ragab, Abdallah R Allam, Eman Ibrahim Hager, Kıvılcım Karadeniz Cerit, Adnan Dağçınar, Tümay Umuroğlu, Ayten Saraçoğlu, Mustafa Sakar, Can Kıvrak, Gül Çakmak, Ibrahim Sallam, Gamal Amira, Mohamed Sherief, Simone de Oliveira Coelho, Arissa Ikeda, Licia Portela, Marianne Monteiro Garrigo, Ricardo Vianna de Carvalho, Fernanda Lobo, Sima Ester Ferman, Fernanda Ferreira da Silva Lima, Moawia Mohammed Ali Elhassan, Nada Osman Yousif Elhaj, Hytham K. S. Hamid, Emmanuel A. Ameh, Vincent E. Nwatah, Adewumi B. Oyesakin, Andrew Nwankwo Osuigwe, Chisom Adaobi Nri-Ezedi, Eric Okechukwu Umeh, Nellie Patiala, Abiodun Folashade Adekanmbi, Olubunmi Motunrayo Fatungase, Olubunmi Obafemi Obadaini, Sarah Al-Furais, Humaida Hemlae, Sreylis Nay, R M Jeffri Ismail, Simonede Campos Vieira Abib, Fabianne Altruda de Moraes Costa Carlesse, Mayara Caroline Amorim Fanelli, Fernanda Kelly Marques de Souza, Denis Cozzi, Paolo Musiu, Paolo Sapienza, Martina Zambon, Simona Meneghini, Pierfranco Cicerchia, Abdulrahman Omar Taha, Bouaoud Souad, Mebarki Malika, Bioud Belkacem, Ayman Meelad, Hajier Salim Alrashed, Fayza Haider, Fatema Naser Al Fayez, Halwani Yaninga Fuseini, Peter Gyamfi Kwarteng, Abubakari Bawa Abdulai, Sheba Mary Pognaa Kunfah, Gilbert B. Bonsaana, Stephanie Ajinkpang, Edmund M. Der, Francis A. Abantanga, Mary Joan Kpiniong, Kingsley Aseye Hattor, Kingsley Appiah Bimpong, Mohamed Elbahnasawy, Sherief Abdelsalam, Reto M. Baertschiger, Amanpreet Brar, Andreea C Matei, Hira Khalid Zuberi, Kishwer Nadeem, Naema Khayyam, Fatima Ambreen Imran, Nida Zia, Sadia Muhammad, Muhammad Rafie Raza, Muhammad Rahil Khan, Alaa Hamdan, Abdeljawad Mazloum, Ali Abodest, Nisreen Ali, Bardisan Gawarieh, Ammar Omran, Almed Moussa, Alaa Ahmed, Munawar Hraib, Victor Khoury, Abdulrahman Almjersah, Mohammad Ali Deeb, Almahmod Alkhalil, Akram Ahmed, Ali Alelayan, Ali Hammed, Wassem Shater, Ahmad Bouhuwaish, Alqasim Abdulkarim, Marwa Biala, Reem Ghamgh, Amani Alamre, Marwa Shelft, Asmaa A. M. Albanna, Hoda Tawel, Emmanuel Hatzipantelis, Eleni Tsotridou, Assimina Galli-Tsinopoulou, C-Khai Loh, Doris Lau, Azrina Syarizad Khutubul, Raphael N. Vuille-dit-Bille, Stefan G. Holland-Cunz, Nima Allafi, Kelvin Ifeanyichukwu Egbuchulem, Olakayode Olaolu Ogundoyin, Isaac Dare Olulana, Biobele J. Brown, Oluwasegun Joshua Afolaranmi, AbdulBasit Fehintola, Annika Heuer, Matthias Priemel, Lennart Viezens, Martin Stangenberg, Marc Dreimann, Alonja Reiter, Jasmin Meyer, Leon Köpke, Uduak Offiong, Philip Mari Mshelbwala, Fashie Andrew Patrick, Aminu Muhammed Umar, N Otene ThankGod, Kazeem O. O. Ibrahim, Dupe S. Ademola-Popoola, Olayinka T. Sayomi, Alege Abdurrzzaq, Ademola A. Adeyeye, Khadijah O. Omokanye, Lukman O Abdur-Rahman, Olubisi Olutosin Bamidele, Shakirullah AbdulAzeez, Aminat Akinoso, Michael O. Adegboye, Yuki Julius Ng, Syukri Ahmad Zubaidi, Murad Almasri, Rasaq Olaosebikan, Akila Muthukumar, Amon Ngongola, Azad Patel, Abdullahi Nuhu-Koko, Baba Jibrin, Ajiboye L. Olalekan, Christopher S. Lukong, Ezekiel I. Ajayi, Gabriela Guillén, Sergio López, José Andrés Molino, Pablo Velasco, Omar Hamam, Rim Elmandouh, Nensi Melissa Ruzgar, Rachel Levinson, Shashwat Kala, Sarah Ullrich, Emily Christison-Lagay, Aya Sabry Mortada, Mahmoud Ahmed Ebada, Eman Seif Alnaser Solimam, Khaled Abualkher, Amr Mohammed Elsayed Yousf, Mohamed Mohamed Holail, Reem Mohamed Almowafy, Janice Hui Ling Wong, Reto Baertschiger, Essam Elhalaby, Mahmoud M. Saad, Guido Seitz, Judith Lindbert, Francis Abantanga Georgios Tsoulfas, Asimina Galli-Tsinopoulou, Calogero Virgone, Mohammad K. Abou Chaar, Eric Mwangi Irungu, Outani Oumaima, Lily Saldana, Jan Godzinsky, Abdelbasit Ali, Mohamed Bella Jalloh, Nellie Bell, Annette Jacobsen, Israel Fernandez Pineda, Lucas Krauel, Waha Rahama, Hazim Elfatih, Arda Isik, Andrea Hayes-Jordan, and Roshni Dasgupta

Subjects
Medicine
Abstract

Objectives Paediatric cancer is a leading cause of death for children. Children in low-income and middle-income countries (LMICs) were four times more likely to die than children in high-income countries (HICs). This study aimed to test the hypothesis that the COVID-19 pandemic had affected the delivery of healthcare services worldwide, and exacerbated the disparity in paediatric cancer outcomes between LMICs and HICs.Design A multicentre, international, collaborative cohort study.Setting 91 hospitals and cancer centres in 39 countries providing cancer treatment to paediatric patients between March and December 2020.Participants Patients were included if they were under the age of 18 years, and newly diagnosed with or undergoing active cancer treatment for Acute lymphoblastic leukaemia, non-Hodgkin’s lymphoma, Hodgkin lymphoma, Wilms’ tumour, sarcoma, retinoblastoma, gliomas, medulloblastomas or neuroblastomas, in keeping with the WHO Global Initiative for Childhood Cancer.Main outcome measure All-cause mortality at 30 days and 90 days.Results 1660 patients were recruited. 219 children had changes to their treatment due to the pandemic. Patients in LMICs were primarily affected (n=182/219, 83.1%). Relative to patients with paediatric cancer in HICs, patients with paediatric cancer in LMICs had 12.1 (95% CI 2.93 to 50.3) and 7.9 (95% CI 3.2 to 19.7) times the odds of death at 30 days and 90 days, respectively, after presentation during the COVID-19 pandemic (p

Published
2022
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8. Juvenile Myelomonocytic Leukemia in Turkey: A Retrospective Analysis of Sixty-five Patients

Author

Özlem Tüfekçi, Ülker Koçak, Zühre Kaya, İdil Yenicesu, Canan Albayrak, Davut Albayrak, Şebnem Yılmaz Bengoa, Türkan Patıroğlu, Musa Karakükçü, Ekrem Ünal, Elif Ünal İnce, Tali İleri, Mehmet Ertem, Tiraje Celkan, Gül Nihal Özdemir, Nazan Sarper, Dilek Kaçar, Neşe Yaralı, Namık Yaşar Özbek, Alphan Küpesiz, Tuba Karapınar, Canan Vergin, Ümran Çalışkan, Hüseyin Tokgöz, Melike Sezgin Evim, Birol Baytan, Adalet Meral Güneş, Deniz Yılmaz Karapınar, Serap Karaman, Vedat Uygun, Gülsun Karasu, Mehmet Akif Yeşilipek, Ahmet Koç, Erol Erduran, Berna Atabay, Haldun Öniz, and Hale Ören

Subjects
hematopoietic stem cell transplantation, juvenile myelomonocytic leukemia, turkey, Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Objective: This study aimed to define the status of juvenile myelomonocytic leukemia (JMML) patients in Turkey in terms of time of diagnosis, clinical characteristics, mutational studies, clinical course, and treatment strategies. Materials and Methods: Data including clinical and laboratory characteristics and treatment strategies of JMML patients were collected retrospectively from pediatric hematology-oncology centers in Turkey. Results: Sixty-five children with JMML diagnosed between 2002 and 2016 in 18 institutions throughout Turkey were enrolled in the study. The median age at diagnosis was 17 months (min-max: 2-117 months). Splenomegaly was present in 92% of patients at the time of diagnosis. The median white blood cell, monocyte, and platelet counts were 32.9x109/L, 5.4x109/L, and 58.3x109/L, respectively. Monosomy 7 was present in 18% of patients. JMML mutational analysis was performed in 32 of 65 patients (49%) and PTPN11 was the most common mutation. Hematopoietic stem cell transplantation (HSCT) could only be performed in 28 patients (44%), the majority being after the year 2012. The most frequent reason for not performing HSCT was the inability to find a suitable donor. The median time from diagnosis to HSCT was 9 months (min-max: 2-63 months). The 5-year cumulative survival rate was 33% and median estimated survival time was 30+-17.4 months (95% CI: 0-64.1) for all patients. Survival time was significantly better in the HSCT group (log-rank p=0.019). Older age at diagnosis (>2 years), platelet count of less than 40x109/L, and PTPN11 mutation were the factors significantly associated with shorter survival time. Conclusion: Although there has recently been improvement in terms of definitive diagnosis and HSCT in JMML patients, the overall results are not satisfactory and it is necessary to put more effort into this issue in Turkey.

Published
2018
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10. Experience with Pediatric Chronic Immune Thrombocytopenia over 30 Years in the Era before Eltrombopag

Author

Begum S. Koc, Gul Nihal Ozdemir, Javid Alakbarli, Hilmi Apak, and Tiraje Celkan

Subjects
children, thrombocytopenia, chronic ITP, splenectomy, Pediatrics, RJ1-570
Abstract

Background: There is limited information on the natural course of chronic ITP in children. We aimed to evaluate the clinical and demographic characteristics of children with chronic ITP in the era before the availability of eltrombopag. Methods: A total of 86 children with chronic ITP between 1978–2014 were included. Demographic findings, laboratory results, clinical signs, bleeding scores, response time and time of complete remission were recorded. Results: The male/female ratio was 1.09, and median follow-up time was 3 years (range: 1.5–17 years). The median age at diagnosis of chronic ITP was 7 years (range: 2–17), and the median initial platelet count was 10 × 109/L (range: 1–66 × 109/L). Petechiae/ecchymoses were the most common clinical sign (86%) and followed by mucosal bleeding (39.5%). Severe bleeding was seen in 5% of the patients. None of them had intracranial hemorrhage. Twenty patients underwent splenectomy, and the rate of complete remission was 70%. Spontaneous complete remission was seen in 29% of the patients, and the median time to spontaneous complete remission was 3 years. Conclusions: Our study showed that almost one-third of patients with chronic ITP experienced spontaneous complete remission in an average of 3 years, and splenectomy provided satisfactory results in severe cases. This study demonstrates the natural history of chronic ITP in childhood before the era of eltrombopag.

Published
2024
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11. PB2401: DETERMINING MOLECULAR BACKGROUND OF A GROUP OF CHILDHOOD LEUKEMIA/LYMPHOMA PATIENTS WITH POSITIVE FAMILY HISTORY FROM TÜRKIYE

Author

Gizem Önder, Ufuk Amanvermez, Cengiz Canpolat, Fatih Erbey, Davut Albayrak, Elif Ince, Gul Nihal Ozdemir, Yunus Murat Akcabelen, Nese Yarali, Namik Yasar Ozbek, Ikbal Okbozkaya, Turan Bayhan, Dilek Kacar, Omer Dogru, Koray Yalcin, Nihat Bugra Agaoglu, Ozkan Ozdemir, Ugur Ozbek, and Ozden Hatirnaz Ng

Subjects
Diseases of the blood and blood-forming organs, RC633-647.5
Published
2023
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12. Bleeding risk of surgery and its prevention in patients with inherited platelet disorders

Author

Sara Orsini, Patrizia Noris, Loredana Bury, Paula G. Heller, Cristina Santoro, Rezan A. Kadir, Nora C. Butta, Emanuela Falcinelli, Ana Rosa Cid, Fabrizio Fabris, Marc Fouassier, Koji Miyazaki, Maria Luisa Lozano, Pamela Zúñiga, Claire Flaujac, Gian Marco Podda, Nuria Bermejo, Remi Favier, Yvonne Henskens, Emmanuel De Maistre, Erica De Candia, Andrew D. Mumford, Gul Nihal Ozdemir, Ibrahim Eker, Paquita Nurden, Sophie Bayart, Michele P. Lambert, James Bussel, Barbara Zieger, Alberto Tosetto, Federica Melazzini, Ana C. Glembotsky, Alessandro Pecci, Marco Cattaneo, Nicole Schlegel, and Paolo Gresele

Subjects
Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Excessive bleeding at surgery is a feared complication in patients with inherited platelet disorders. However, very few studies have evaluated the frequency of surgical bleeding in these hemorrhagic disorders. We performed a worldwide, multicentric, retrospective study to assess the bleeding complications of surgery, the preventive and therapeutic approaches adopted, and their efficacy in patients with inherited platelet disorders: the Surgery in Platelet disorders And Therapeutic Approach (SPATA) study. We rated the outcome of 829 surgical procedures carried out in 423 patients with well-defined forms of inherited platelet disorders: 238 inherited platelet function disorders and 185 inherited platelet number disorders. Frequency of surgical bleeding was high in patients with inherited platelet disorders (19.7%), with a significantly higher bleeding incidence in inherited platelet function disorders (24.8%) than in inherited platelet number disorders (13.4%). The frequency of bleeding varied according to the type of inherited platelet disorder, with biallelic Bernard Soulier syndrome having the highest occurrence (44.4%). Frequency of bleeding was predicted by a pre-operative World Health Organization bleeding score of 2 or higher. Some types of surgery were associated with a higher bleeding incidence, like cardiovascular and urological surgery. The use of pre-operative pro-hemostatic treatments was associated with a lower bleeding frequency in patients with inherited platelet function disorders but not in inherited platelet number disorders. Desmopressin, alone or with antifibrinolytic agents, was the preventive treatment associated with the lowest bleedings. Platelet transfusions were used more frequently in patients at higher bleeding risk. Surgical bleeding risk in inherited platelet disorders is substantial, especially in inherited platelet function disorders, and bleeding history, type of disorder, type of surgery and female sex are associated with higher bleeding frequency. Prophylactic pre-operative pro-hemostatic treatments appear to be required and are associated with a lower bleeding incidence.

Published
2017
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