Priapism as a Result of Chronic Myeloid Leukemia: Case Report, Pathology, and Review of the Literature

Abstract

Introduction Priapism is rare-presenting feature in male patients with chronic myeloid leukemia (CML). Several hypotheses for pathogenesis have been described. Management has been controversial; some authors described resolution following priapism-specific interventions, and others recommended addition of CML-specific therapy or even CML-specific therapy alone. Aim In this report, we describe presentation and management of a man with refractory priapism that was the first presenting manifestation of CML. We also report, for the first time, the pathology sections of the sinusoidal tissue in such cases. Literature is reviewed for similar cases and their outcome. Methods A 21-year-old male patient presented with painful priapism that started 6 days earlier and failed aspiration–irrigation. CBC revealed marked leucocytosis. Oncology care diagnosed CML, and treatment with Imatinib was commenced with prior semen cryopreservation. Following remission, a penile prosthesis was implanted, assisted by optical corporotomy. Sinusoidal tissue biopsy was stained by hematoxylin/eosin (H&E) and CD34. Main Outcome Measures Pathology sections of cavernous tissue following CML-induced priapism. Results The penile implant survived without complications. H&E examination of the sinusoidal tissue biopsy revealed leukemic infiltration associated with vascular endothelial damage. CD34 staining showed the mixed picture of leukemic infiltrates, intact vascular endothelium with lumena showing leukemic cells, alternating with destroyed vessels, and no vascular lumena and ruminants of endothelial cells. Conclusion Priapism can be the first manifestation of previously undetected CML. The pathological picture of sinusoidal tissue in such cases is presented. In the case at hand, a complete blood picture was helpful in early diagnosis of CML and early initiation of targeted chemotherapy along with the corporal irrigation/aspiration or shunt surgery. It is therefore recommended to have a CBC examined at presentation of any case of ischemic priapism of unknown etiology, early initiation of CML therapy along with aspiration/irrigation, preferably cryopreserving a semen sample before CML therapy.

Priapism as the First Sign of Chronic Myeloid Leukemia

“…Различают два вида приапизма -ишемический (веноокклюзионный, или низкопоточный) и неишемический (артериальный, или высококопоточный) [10]. Дифференциальный диагноз между ишемическим и неишемическим приапизмом можно провести на основании исследования газового состава крови, полученной при пункции кавернозных тел [11].…”

“…Неишемический приапизм обычно возникает вследствие травмы. Причинами ишемического приапизма могут быть интракорпоральные инъекции вазоактивных субстанций, таких как папаверин, простагландин Е1, фентоламин, а также гипервискозный синдром при таких гематологических заболеваниях, как истинная полицитемия, ХМЛ, множественная миелома [10]. Онкогематологические заболевания чаще всего становится причиной развития веноокклюзивной (ишемической, низкопоточной) формы приапизма.…”

“…У больных ХМЛ приапизм возникает вследствие гиперлейкоцитоза, сопровождающегося лейкостазами и гипервискозностью, что обычно наблюдается при увеличении количества лейкоцитов крови более 100×10 9 /л, что, в свою очередь, приводит к обструкции вен тромбами и микротромбами [10]. Другой патогенетический фактор, приводящий к развитию приапизма при ХМЛ, -это избыточная продукция цитокинов и молекул адгезии лейкемическими клетками, что приводит к повышенной секвестрации клеток в микрососудах [10] Первое описание приапизма у больного ХМЛ было сделано в 1974 г. S.M. Schreibman и соавт.…”

Malign Priapism Secondary to Renal Cell Carcinoma Provoked Paraneoplastic Leukemoid Reaction: Report of The First Case

“…The most common cause of low flow priapism is idiopathic, while the cause of high flow priapism is mostly perineal or penile trauma. Other causes of ischemic priapism include hematological diseases such as sickle cell disease, hyperviscosity syndromes such as chronic myeloid leukemia, polycythemia vera, multiple myeloma and hypercoagulable state (7).…”

“…The mechanism of priapism in cases with leukemia is hypothesized to be due to hyperviscosity caused by hyperleukocytosis and aggregation of leukocytes inside the sinusoids of the corpora cavernosa resulting in sinusoidal engorgement and erection of the penis. Blockage of emissary veins and dorsal veins results in stoppage of the venous return (7).…”