{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T20:31:38Z","timestamp":1783542698022,"version":"3.55.0"},"reference-count":135,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2023,7,19]],"date-time":"2023-07-19T00:00:00Z","timestamp":1689724800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Deutsche Forschungsgemeinschaft","award":["SU122\/3-1"],"award-info":[{"award-number":["SU122\/3-1"]}]},{"name":"Deutsche Forschungsgemeinschaft","award":["2008\/833"],"award-info":[{"award-number":["2008\/833"]}]},{"name":"Sonnenstrahl e.V.","award":["SU122\/3-1"],"award-info":[{"award-number":["SU122\/3-1"]}]},{"name":"Sonnenstrahl e.V.","award":["2008\/833"],"award-info":[{"award-number":["2008\/833"]}]},{"name":"Schornsteinfeger gegen Krebs","award":["SU122\/3-1"],"award-info":[{"award-number":["SU122\/3-1"]}]},{"name":"Schornsteinfeger gegen Krebs","award":["2008\/833"],"award-info":[{"award-number":["2008\/833"]}]},{"name":"Novartis Pharma","award":["SU122\/3-1"],"award-info":[{"award-number":["SU122\/3-1"]}]},{"name":"Novartis Pharma","award":["2008\/833"],"award-info":[{"award-number":["2008\/833"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Pediatric chronic myeloid leukemia (CML) is a very rare malignancy (age-related incidence 0.1\/100,000) typically presenting with leucocyte counts &gt;100,000\/\u00b5L. However, clinical signs of leukostasis are observed at diagnosis in only approximately 10% of all cases and among these, priapism is infrequent. Here, we analyze data from pediatric CML registries on the occurrence of priapism heralding diagnosis of CML in 16\/491 (3.2%) boys (median age 13.5 years, range 4\u201318) with pediatric CML. In the cohort investigated, duration of priapism resulting in a diagnosis of CML was not reported in 5 patients, and in the remaining 11 patients, occurred as stuttering priapism over 3 months (n = 1), over 6 weeks (n = 1), over 1\u20132 weeks (n = 2), over several days (n = 2), or 24 h (n = 1), while the remaining 4 boys reported continuous erection lasting over 11\u201312 h. All patients exhibited splenomegaly and massive leukocytosis (median WBC 470,000\/\u00b5L, range 236,700\u2013899,000). Interventions to treat priapism were unknown in 5 patients, and in the remaining cohort, comprised intravenous fluids \u00b1 heparin (n = 2), penile puncture (n = 5) \u00b1 injection of sympathomimetics (n = 4) \u00b1 intracavernous shunt operation (n = 1) paralleled by leukocyte-reductive measures. Management without penile puncture by leukapheresis or exchange transfusion was performed in 3 boys. In total, 7 out 15 (47%) long-term survivors (median age 20 years, range 19\u201325) responded to a questionnaire. All had maintained full erectile function; however, 5\/7 had presented with stuttering priapism while in the remaining 2 patients priapism had lasted &lt;12 h until intervention. At its extreme, low-flow priapism lasting for longer than 24 h may result in partial or total impotence by erectile dysfunction. This physical disability can exert a large psychological impact on patients\u2019 lives. In a narrative review fashion, we analyzed the literature on priapism in boys with CML which is by categorization stuttering or persisting as mostly painful, ischemic (low-flow) priapism. Details on the pathophysiology are discussed on the background of the different blood rheology of hyperleukocytosis in acute and chronic leukemias. In addition to the data collected, instructive case vignettes demonstrate the diagnostic and treatment approaches and the outcome of boys presenting with priapism. An algorithm for management of priapism in a stepwise fashion is presented. All approaches must be performed in parallel with cytoreductive treatment of leukostasis in CML which comprises leukapheresis and exchange transfusions \u00b1 cytotoxic chemotherapy.<\/jats:p>","DOI":"10.3390\/jcm12144776","type":"journal-article","created":{"date-parts":[[2023,7,19]],"date-time":"2023-07-19T21:21:46Z","timestamp":1689801706000},"page":"4776","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Priapism at Diagnosis of Pediatric Chronic Myeloid Leukemia: Data Derived from a Large Cohort of Children and Teenagers and a Narrative Review on Priapism Management"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9522-8838","authenticated-orcid":false,"given":"Meinolf","family":"Suttorp","sequence":"first","affiliation":[{"name":"Pediatric Hematology and Oncology, Medical Faculty, TU Dresden, 01307 Dresden, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie","family":"Sembill","sequence":"additional","affiliation":[{"name":"Pediatric Oncology and Hematology, Department of Pediatrics and Adolescent Medicine, University Hospital Erlangen, 91054 Erlangen, Germany"},{"name":"Comprehensive Cancer Center Erlangen-EMN (CCC ER-EMN), 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1174-5799","authenticated-orcid":false,"given":"Krzysztof","family":"Kalwak","sequence":"additional","affiliation":[{"name":"Supraregional Center of Pediatric Oncology \u201cCape of Hope\u201d, Wroclaw Medical University, 50-556 Wroclaw, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4523-1676","authenticated-orcid":false,"given":"Markus","family":"Metzler","sequence":"additional","affiliation":[{"name":"Pediatric Oncology and Hematology, Department of Pediatrics and Adolescent Medicine, University Hospital Erlangen, 91054 Erlangen, Germany"},{"name":"Comprehensive Cancer Center Erlangen-EMN (CCC ER-EMN), 91054 Erlangen, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frederic","family":"Millot","sequence":"additional","affiliation":[{"name":"Inserm CIC 1402, University Hospital of Poitiers, 86000 Poitiers, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2023,7,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1002\/ajh.25011","article-title":"Chronic myeloid leukemia: 2018 update on diagnosis, therapy and monitoring","volume":"93","author":"Jabbour","year":"2018","journal-title":"Am. 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