Priapism on Chronic Myeloid Leukemia with BCR-ABL1 Fusion gene Identified by Molecular Test: A Case Report
Yustisia Amalia, Paulus B. Notopuro*
Medicine, Airlangga University, Indonesia.
*Corresponding Author E-mail: paulus-b-n@fk.unair.ac.id
ABSTRACT:
The incidence of hematological malignancies has elevated in recent decades. Chronic Myeloid Leukemia (CML) is a hematopoietic stem cell malignant clonal disorder resulting in elevation of erythroid cells and platelets in peripheral blood and clear myeloid hyperplasia in the bone marrow. Priapism is one of a rare clinical manifestation and serious complication in Chronic Myeloid Leukemia (CML). It is cause due to hematological disorder is most likely due to venous obstruction as well as hyperviscosity due to an increased number of circulating leukocytes mature and immature forms. We report a 30-year-old male came to the emergency room, presented with priapism caused by Chronic Myeloid Leukemia (CML) with hyperleukocytosis. The rarity of this case reiterates the importance of thorough morphological, cytogenetic examination along with radiology in diagnosing, treatment and follow up of patients. Starting leukemia therapy to reduce the leukocyte count immediately, can solve the problem in hyperleukocytosis that caused priapism in Chronic Myeloid Leukemia (CML).
KEYWORDS: Priapism, Chronic Myeloid Leukemia, BCR-ABL, Cancer.
INTRODUCTION:
The incidence of hematological malignancies has elevated in recent decades1. Leukemia is the most common malignancy of children with a prevalence of 129 in one million1,2,3. In Indonesia, chronic myeloid leukemia (CML) was mostly found in productive age (36 years) while in Europe, many were elderly (64 years old)2,4. Chronic Myeloid Leukemia (CML) is a hematopoietic stem cell malignant clonal disorder resulting in elevation of erythroid cells and platelets in peripheral blood and clear myeloid hyperplasia in the bone marrow5,6,7. Chronic myeloid leukemia (CML) is also characterized by formation of Philadelphia chromosome8,9. Priapism is a rare presenting feature of chronic myeloid leukemia (CML). It accounts for the occurrence of priapism is 1-5% from 100.000 persons10,11. Priapism is an urological emergency, which must be treated early to prevent erectile dysfunction, and is defined as a persistent penile erection that continues hours (4 h) beyond, or is unrelated to sexual stimulation.
Priapism due to hematological disorder is most likely due to venous obstruction from microemboli/thrombi as well as hyperviscosity due to an increased number of circulating leukocytes mature and immature forms12,13,14. Here we present a rare case where priapism occurred in CML.
A 30 years old male presented with persistent painful erection of the penis that had lasted approximately 48 hours. His penis remained erect, painful, and swollen when he arrived at the emergency department. He denied recent intercourse, trauma, use of medications, and radiation therapy. He was alert and oriented. The vital signs revealed a body temperature of 36.5°C, pulse of 104 beats/minute, and blood pressure of 133/80 mmHg, and respiration of 20 beats/minute.
He was alert and oriented. The physical examination revealed that the spleen was palpable 4cm below the left costal margin. His conjunctiva was pale but no jaundice. On examination, the penis was erect, firm, and tender with superficial venous engorgement. Penile arterial blood gas revealed pO2 0 mmHg, pCO2 102 mmHg, and pH 6.8. Ultrasonography of penis, showed there is no flow from corpora cavernosa and suspected with low flow priapism (figure 1).
Figure 1. Penis ultrasonography with low flow priapism interpretation
Laboratory data showed hemoglobin 8.1g/dL, hematocrit 22.9%, white blood count 345810/mm3 , and platelets 500000/mm3. A peripheral blood smear demonstrated immature leukocytes in various stages of differentiation with 17% myeloblast, suspected as CML accelerated phase (figure 2) and also typical p210 BCR-ABL transcripts were detected confirming the diagnosis of CML.
Figure 2. Blood smear evaluation of CML
Treatment of the priapism was initially performed by cavernosa aspiration and epinephrine irrigation at emergency department under the impression of low flow-type priapism. The erection was relieved later by these procedures. For hyperleukocytosis, the patient was admitted to the hematology oncology ward. He was started on hydroxyurea therapy (50mg/kg/day) associated with intravenous fluid hydration. Recurrent priapism had not happened to him during his admission period. The patient was treated with imatinib mesylate for his CML and continued to report to us without any erectile dysfunction until the date of writing.
This type of research used quasi-experimental. The research held in June-July 2020 and the populations were all pregnant women at Cenrana and Tompobulu health center. The samples were 15 pregnant women at Cenrana health center as an experimental group and 15 pregnant women at Tompobulu health center as a control group. The tool used to measure the blood pressure is a Sphygmomanometers from GEA brand. The method of measurement is not hypertension if it is below 120/80mmHg, Pre-hypertension if 120/80mmHg -140/90mmHg, hypertension level 1 if more than 140/90mmHg-160/100mmHg, hypertension level 2 if more than 160/100mmHg. Urine protein was measured using the OneMed Gluco protein test. How to measure urine protein is each pregnant woman was asked 5cc of urine in a special reservoir, then let stand for 2 minutes until all the strips are immersed and read. If the strip is yellow it means negative urine protein and if it is green the urine protein content is positive. The intervention materials were crackers made from cassava, cassava leaves and pumpkin seeds which had their nutritional content analyzed as much as 25grams per day for 30 days. The crackers were delivered and given to the samples every evening by the enumerators to be consumed until they were finished at their respective homes. The statistical test used is the T test.
DISCUSSION:
Priapism as a result of hematologic malignancy is most likely caused by venous obstruction from microemboli/thrombi as well as hyperviscosity caused by the increased number of circulating leukocytes in mature and immature forms. Leukocytosis associated with leukemia causes stasis of venous blood and leads to “Low Flow” type of priapism also known as ischemic or anoxic priapism15. Leukemia accounts for approximately 1%–5% of priapism. Of this, Chronic Myeloid Leukemia (CML) accounts for 50% of the cases. However, priapism as a presenting feature of Chronic Myeloid Leukemia (CML) is rare and is noted in 1%–2% of Chronic Myeloid Leukemia (CML) patients15,16.
In our case, the patient was 30 years old with no history of Chronic Myeloid Leukemia (CML) came to the Emergency Room with priapism and leucocytosis. Based on the literature, patients in every age group are at risk of developing priapism. However, there are two peaks in the age distribution that tend to experience this condition. The peak in pediatric age is between 5 and 10 years, especially in patients with sickle cell disease. While the second peak age is at sexually active phase between 20 and 50 years17.
CML is a clonal bone marrow stem cell disorder in which proliferation of mature granulocytes (neutrophils, eosinophils, and basophils) and their precursors is the main finding. It is a type of myeloproliferative disease associated with a characteristic chromosomal translocation called the Philadelphia chromosome. Chronic Myeloid Leukemia (CML) diagnosis is established based on blood smear and positive BCR-ABL transcript. In Chronic Myeloid Leukemia (CML), the most common type of priapism is the ischemia one (low flow priapism). Patients usually complain of rigid erection, which may be accompanied by pain characterized by reduced to no cavernous blood flow at all18. In this case, the penile arterial blood gas showed characteristics of low flow priapism and no flow of cavernous blood flow in penile ultrasonography. The main mechanism of priapism in Chronic Myeloid Leukemia (CML) is the aggregation of leukemia cells in the corpora cavernosa and dorsal veins of the penis.
Priapism can cause irreversible cell injury and fibrosis if not treated within 24-48 hours. Aspiration of blood from corpora cavernosa could be the first line treatment with success rate is approximately 30%. At the same time, the underlying disease that cause priapism like Chronic Myeloid Leukemia (CML) should be treated well with cytoreductive therapy. In this patient, the first line treatment for priapism was successfully done and cytoreductive therapy with imatinib shows a good result. Imatinib mesylate is an antineoplastic agent which has its use in treating chronic myelogenous leukemia19. It also has been recognized that tyrosine kinase province of BCR-ABL protein is a possible healing target for the action of Chronic Myeloid Leukemia20.
In the other studied also claim that dasatinib could become first line drug in the pharmacotherapy of patients with chronic mylogenuous leukemia (CML). This is because the drug possesses tolerability and safety advantages over the other tyrosine kinase inhibitors21,22.
ACKNOWLEDGMENTS:
We would like to thank patient and family for their good participation and cooperation.
CONFLICT OF INTEREST:
None
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Received on 10.07.2022 Modified on 28.08.2022
Accepted on 04.10.2022 © RJPT All right reserved
Research J. Pharm. and Tech 2023; 16(5):2229-2232.
DOI: 10.52711/0974-360X.2023.00366