Unusual Presentation of Priapism Associated with Acute and Chronic Myeloid Leukemia in Two Patients: Emergency Management

Gaye, O; Thiam, Ngor Mack; Cassell, Ayun; Gueye, Serigne Mourtalla; Sow, Yaya; Fall, B.; Ndoye, Alain Khassim

doi:10.1155/2020/4982432

Cited by 8 publications

(15 citation statements)

References 19 publications

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“…Several cases presenting a sudden painful erection due to ischemic priapism, as in our case, have been reported 4,9,10 . Besides, subacute clinical presentations have also been described 6,7,9 .…”

Section: Discussionsupporting

confidence: 61%

Priapism as the first manifestation in chronic myeloid leukemia: A case report and focused review of literature

Melo

Orozco

Castellanos

et al. 2021

Clinical Case Reports

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Chronic myeloid leukemia (CML) is a clonal hematopoietic stem cell disorder characterized by a reciprocal translocation between the long arms of chromosomes 9 (ch 9) and 22 (ch 22). This results in the juxtaposition of the BCR gene from ch9 on ch22, encoding the BCR-ABL1 gene, which is transcribed into mRNA and translated into the BCR-ABL1 protein. The BCR-ABL1 gene is always present in CML cases and provides a unique biomarker for diagnosis and monitoring response to the treatment. Furthermore, it acts as a susceptible drug target. 1 The incidence of CML is consistent worldwide, at 1%-1.5 per 100,000 people. Despite the median age of onset of CML is 40-60 years, it may occur in children and the elderly population. It has a slight male predominance and has three stages: chronic phase (CP), accelerated phase (AP), and blast crisis (BC). 2 CML patients typically present CP with leukocytosis, splenomegaly, fatigue, night sweats, malaise, weight loss, left upper quadrant pain, discomfort, and satiety. Other symptoms include lymphadenopathy, hepatomegaly, skin infiltration, bone pain, thrombosis and/or bleeding, retinal hemorrhages, extramedullary mass, and

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Section: Discussionsupporting

confidence: 61%

Priapism as the first manifestation in chronic myeloid leukemia: A case report and focused review of literature

Melo

Orozco

Castellanos

et al. 2021

Clinical Case Reports

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“…Some patients who are at high risk for priapism include users of intracorporal injection therapy for erectile dysfunction, coagulation disorders such as sickle cell disease and CML. 2,4 In CML, hyperleukocytosis is thought to be the main cause of priapism. The main mechanism is the aggregation of leukemia cells in the corpora cavernosa and dorsal veins of the penis.…”

Section: Discussionmentioning

confidence: 99%

“…1 The data needed in the management of patients with this case are erection duration, pain scale, trauma, complete blood count, peripheral blood smear, penile blood gas analysis, bone marrow and polymerase chain reaction for BCR-ABL1 if necessary. 1,2,4 In CML, the most common type of priapism is the ischemia one (veno-occlusive). Patients usually complain of rigid erection, which may be accompanied by pain characterized by reduced to no cavernous blood flow at all.…”

Section: Discussionmentioning

confidence: 99%

“…9 In the case of CML in general, The IRIS study describes the effectiveness of imatinib therapy for complete hematological response (CHR), major cytogenetic response (McyR) and complete cytogenetic response (CcyR). 4 Leukapheresis can cause a rapid decrease in intravascular leukemia cells and improve tissue perfusion as well as complaints related to leukostasis (generally show pulmonary and central nervous system manifestations). One leukapheresis procedure can reduce the leukocyte count by 30-60%.…”

Section: Discussionmentioning

confidence: 99%

“…One of the causes of high-flow abnormalities is penile injury, while low-flow priapism is commonly caused by blood disorders such as sickle cell anemia and chronic myeloid leukemia (CML). [2][3][4] Hematological abnormalities account for 20% of the incidence of priapism while leukemia cases account for 1-5% of priapism in men. The theory related to the occurrence of priapism is the dysregulation of nitric oxide (NO) in penile vascularization.…”

Section: Introductionmentioning

confidence: 99%

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Case Report: Priapism as the clinical presentation of chronic myeloid leukemia in accordance with reports created during last twenty years: a case report and literature review

et al. 2021

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Priapism in chronic myeloid leukemia (CML) appears to be an infrequent manifestation as well as a crucial emergency. Here, we report an 18-year-old male presenting with a persistent erection of penis for 20 days. We evaluate and compare the reported cases during the past 20 years discussing the management of CML patients experiencing priapism. Cytoreductive therapy followed by leukapheresis, the administration of tyrosine kinase inhibitor, and intra-cavernosal blood aspiration may resolve the symptoms of priapism. Early intervention for cytoreduction and aspiration are the pivotal keys to successfully impeding the complications.

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The value of leukapheresis for treatment of priapism as presenting feature of chronic myeloid leukemia—Case report and review of literature

Velde¹,

Tiellemans

Lil

et al. 2022

eJHaem

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Priapism is a rare presenting feature of chronic myeloid leukemia (CML) in male patients. Treatment aims to relieve symptoms and to prevent erectile dysfunction. Several treatment modalities exist, however no standard treatment is recommended. We evaluated literature concerning different treatment approaches and evaluate the value of leukapheresis in treatment of priapism. The literature search resulted in 57 included articles, consisting of 53 studied patients. Patients had a mean age of 25.3 years, average time from onset to presentation at the hospital was 2 days, and mean white blood cell (WBC) count was 344 × 109/L. Most patients (67.9%) were treated with a combined approach (different modalities were radiological, urological, and oncological treatment). Twelve patients, with a mean WBC count of 365 × 109/L, received leukapheresis. Only two of them reported erectile dysfunction after treatment. Priapism is an urological emergency requiring urgent multidisciplinary treatment. We highlight the importance of local urological therapy combined with systemic therapy for CML. Therapeutic leukapheresis should be applied when available and with no other contraindications.

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Unusual Presentation of Priapism Associated with Acute and Chronic Myeloid Leukemia in Two Patients: Emergency Management

Cited by 8 publications

References 19 publications

Priapism as the first manifestation in chronic myeloid leukemia: A case report and focused review of literature

Priapism as the first manifestation in chronic myeloid leukemia: A case report and focused review of literature

Case Report: Priapism as the clinical presentation of chronic myeloid leukemia in accordance with reports created during last twenty years: a case report and literature review

The value of leukapheresis for treatment of priapism as presenting feature of chronic myeloid leukemia—Case report and review of literature

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