Transcatheter arterial embolization for retroperitoneal bleeding in patients with lumbar artery injury and essential thrombocythemia: a case description

Introduction

Essential thrombocythemia (ET) is a clonal hematopoietic stem cell disease. The condition is typified by the presence of persistent nonreactive thrombocytosis and is frequently accompanied by elevated white blood cell (WBC) levels and lymphoproliferative and myeloproliferative disorders. The mortality rate of ET is believed to be associated with vascular risk factors, including thrombosis and bleeding (1). Due to the distinct nature of this condition, patients with ET are at an elevated risk of bleeding and coagulation. Nonetheless, the diagnosis and treatment regimens for thrombotic risk are fairly comprehensive. However, few studies exist regarding the incidence of bleeding in patients with ET, and diagnosis and treatment have not been standardized. In general, retroperitoneal hemorrhage resulting from lumbar artery injury is most commonly encountered in the context of interventional puncture procedures, anticoagulation therapy, or malignancy (2). This clotting disorder leads to bleeding and can also develop into rare but life-threatening retroperitoneal hematoma. This paper describes a case of closed traumatic lumbar artery hemorrhage in a patient with ET and provides an analysis with reference to the related literature. It is hoped that this report can contribute to the standardization of interventional embolization among patients with lumbar artery hemorrhage.

Case presentation

An 86-year-old male was admitted for injuries sustained in a fall the previous day, followed by transient syncope (1–2 min) and subsequent diminished consciousness, with full spontaneous recovery after approximately 2 hours. He had been diagnosed with ET for 2 years, and bone marrow puncture aspiration indicated the following: (I) features consistent with a myeloproliferative neoplasm (MPN); (II) a positive JAK2 V617F mutation with an allele burden of 41%; (III) a von Willebrand factor antigen (vWF:Ag) level of 60% (reference range, 50–160%). The patient initially received subcutaneous interferon alpha-2b (IFN-α2b) at a dose of 300 μg once daily, which was subsequently transitioned to a combination regimen of hydroxyurea (15 mg/kg/day) and aspirin (100 mg once daily). The patient had a medical history of suboptimal medication adherence. The day before admission, the patient fell out of bed and had low back pain. At first, it was not taken seriously. After the fall, he experienced syncope lasting 1–2 min, with spontaneous recovery occurring 2 hours post-event. He regained consciousness but remained confused and disoriented. He was admitted to the emergency department with recurrent symptoms. The admission vital signs were as follows: blood pressure, 90/45 mmHg; heart rate, 65 beats/min; and body temperature, 36.8 ℃. The right upper abdomen was slightly swollen, the right kidney area was slightly swollen, and the patient’s avoidance to pressure via touch was obvious. The laboratory test findings were as follows: WBC, 37.96×10⁹/L [normal value (4.0–10.0)×10⁹/L], neutrophil percentage, 90.2%; hemoglobin level, 140 g/L (normal value 100–150 g/L); platelet count, 728×10⁹/L [normal value (100–300)×10⁹/L]; red blood cell count, 4.02×10¹²/L [normal value (4.0–5.5)×10¹²/L]; C-reactive protein level, 3.1 mg/L (normal value 0–10 mg/L); and D-dimer level, 0.32 µg/mL (normal value 0–0.55 µg/mL). Abdominal computed tomography (CT) (Figure 1) indicated right psoas major and iliopsoas swelling, along with retroperitoneal bleeding. After intensive rehydration, blood pressure, hemostatic therapy (1 g of tranexamic acid administered intravenously), and other treatment, there was no significant increase in blood pressure. The hemoglobin count was 104 g/L, the hematocrit level was 29.30%, the platelets count was 362×10⁹/L, and the blood pressure was 85/56 mmHg (sustained by vasoactive drugs). Due to the patient’s hemodynamic instability, emergency direct digital subtraction angiography (DSA) examination—and not contrast-enhanced CT—was performed. During the operation, it was clearly evident that there had been multiple contrast agent extravasation in the fourth lumbar artery (Figure 2). We successfully super-selected the fourth lumbar artery on the right side, and gelatin sponge particles (560–710 µm; Alicon Pharmaceutical, Hangzhou, China) were selected based on the vessel diameter. Contrast injection showed reflux, and two microcoils with a diameter of 2 mm and a length of 2 cm were sent through the microcatheter, followed by four microcoils with a diameter of 3 mm and a length of 3 cm (Retracta, Cook Medical, Bloomington, IN, USA). A 3-mm Scepter C balloon catheter (Terumo Medical Corporation, Aliso Viejo, CA, USA) was used to temporarily occlude the lumbar artery, reducing blood flow to facilitate the effective release of coils while avoiding ectopic embolization. The distal segment of the lumbar artery disappeared, and the contrast agent refluxed into the aorta (Figure 3). After the operation, the patient was transferred to the intensive care unit where he received a transfusion of two units of packed red blood cells. The review at 3 hours indicated the following: red blood cell count, 4.58×10¹²/L; hemoglobin level, 124.0 g/L; and hematocrit level, 34.2%. Abdominal CT on the next day showed no significant increase in retroperitoneal hematoma, and confirmed stable position of the L4 arterial embolization coils (Figure 4). The patient’s postoperative circulation was stable, and there was no further bleeding. All procedures in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Figure 1 Retroperitoneal hemorrhage (blue arrow) with swelling of the right iliopsoas muscle (red arrow).

Figure 2 (Top to bottom) Right L1, L2, L3, and L4 lumbar arteries (blue arrow). Superselected microcatheter in the right L4 lumbar artery with active contrast extravasation (red arrows) at the bleeding site.

Figure 3 The distal disappearance of the lumbar artery and contrast reflux into the aorta (blue arrow).

Figure 4 Retroperitoneal hemorrhage (blue arrow) and the implanted metal embolization coil (red arrow).

Discussion

Over the past decade, there has been a growing awareness of the importance of addressing bleeding caused by coagulation dysfunction in patients with ET. However, due to functional defects such as platelet adhesion and aggregation, platelets cannot accumulate normally after local injury, which inevitably increases the risk of bleeding (3). Furthermore, the European Leukemia Network (ELN) identifies the age of patients with ET over 60 years old as an independent risk factor for bleeding (4). In addition to the increased risk of bleeding caused by age itself, it is crucial to consider other factors related to older adult patients, specifically their poor medical compliance. Moreover, one study found that patients with ET and the JAK2 V617F mutation had a higher WBC (also known as a polycythemia vera-like phenotype) (5). In another study, there was a U-shaped relationship between WBC and the risk of bleeding in the essential thrombocythemia (ET) cohort. A too-high or too-low WBC will increase the risk of bleeding (6). It is clear that neutrophils play a role in the destruction of the extracellular matrix caused by the release of metalloproteinases and elastase. However, this does not fully explain how leukocytes cause bleeding. Extreme thrombocytosis (platelet count >1,000×10⁹/L) and leukocytosis (leukocyte count ≥11×10⁹/L) have been identified as significant risk factors for bleeding (7). In this case, the patient’s platelet count did not increase substantially, yet a bleeding event still occurred. This is consistent with the findings of Tefferi et al. (8), who reported a case of a 61-year-old patient who experienced mucocutaneous hemorrhage despite a platelet count of 488×10⁹/L. In the present case, while the role of platelet count cannot be completely ruled out, it is clear that the high shear stress caused by increased blood viscosity plays a major pathogenic role. Common causes of retroperitoneal bleeding are listed in Table 1.

Anatomically, the lumbar artery arises directly from the abdominal aorta. Consequently, injury to this vessel results in significant hemorrhage at a rapid pace due to the high-pressure arterial flow. It is crucial to diagnose and treat acute lumbar artery bleeding promptly, as conservative treatment is largely ineffective, and a delay in appropriate treatment can be life-threatening. In hemodynamically unstable patients refractory to volume resuscitation, early utilization of DSA as a first-line diagnostic and therapeutic modality has been shown to significantly improve survival rates (9) while minimizing delays associated with non-essential imaging. In cases with stable hemodynamics, multi-detector CT is still recommended, especially for defining the scope of hematoma, identifying multifocal bleeding sources such as the Adamkiewicz artery, and avoiding spinal cord ischemia during embolization of the lumbar artery. Lumbar artery anatomical variation requires detailed angiography (10). Open surgery often leads to catastrophic bleeding due to the large and abundant collateral supply in the retroperitoneal space. Lumbar artery interventional embolization is the preferred approach for preventing the bleeding caused by surgical decompression. Meanwhile, arterial embolization is the optimal choice for patients with coagulation dysfunction and unpredictable hemodynamic instability of large soft-tissue hematoma. Angiography is the ideal solution for identifying a clear bleeding point in the early stages, determining the size of the bleed, and promptly stopping it. We successfully treated the patient by embolizing the bleeding artery with a suitably sized microballoon during the operation. This procedure achieved hemostasis and slowed down the blood flow while also occluding the collateral vessels. A coil was then used to reinforce the embolization, preventing rebleeding and achieving a better hemostatic effect. We began by embolizing and stopping the bleeding with particles of a smaller size. Given that the patient’s condition had stabilized and the bleeding artery was expanding, there was a risk of rebleeding. The embolization coil was used during the operation for embolization and reinforcement, ensuring a more effective treatment. This embolization coil (Retracta, Cook Medical) relies on cilia to promote blood coagulation to achieve the desired embolization effect. A single-center study of 36 patients treated with transcatheter arterial embolization (TAE) reported that 28 patients (77.8%) achieved clinical success, confirming that TAE is a safe and effective treatment (11). However, further prospective studies are required to assess the safety and clinical outcomes of individuals with specific coagulation abnormalities. Recent studies have indicated that coronavirus disease 2019 (COVID-19) is a novel risk factor for spontaneous retroperitoneal hemorrhage. The high fibrinolytic state, coagulation factor consumption, and cytokine storm in patients with severe COVID-19 may aggravate the bleeding tendency, even in patients treated with anticoagulant therapy. Lalatović et al. (12) reported a successful case of embolization for fatal bleeding in patients with COVID-19, further confirming the value of TAE in this population.

Conclusions

ET management’s primary objective remains the prevention of fatal vascular complications. In addition to the currently discussed risk of thrombosis, there is a clear need for corresponding targeted guidelines for high-risk bleeding risks such as advanced age (over 60 years old), extreme thrombocytosis (platelet count over 1,000×10⁹/L), leukocytosis (WBC count of 11×10⁹/L), and drug induction (anticoagulant and antiplatelet drugs) during clinical treatment. These patients are at risk of developing hemorrhagic diseases such as retroperitoneal hemorrhage, and arterial embolization should be performed as an emergency intervention, as it is a safe, effective, and less invasive treatment option with excellent results.

Acknowledgments

None.

Footnote

Funding: None.

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-24-2435/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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