Splenic artery embolization for the treatment of spontaneous splenic rupture in hemophilia A: a case report

Introduction

Hemophilia is an X-linked recessive bleeding disorder caused by genetic defects in specific proteins involved in blood coagulation. This condition typically manifests as a disease in males, while females are often carriers with a 50% chance of passing the mutant allele to their offspring (1). Hemophilia is classified into two main types: hemophilia A and hemophilia B. The former results from a deficiency of coagulation factor VIII (FVIII), whereas the latter is due to a deficiency of coagulation factor IX (FIX) (1). The classification of hemophilia is based on the activity levels of FVIII or FIX: levels below 1% are considered severe, levels between 1% and 5% are classified as moderate, and levels greater than 5% but less than 40% are deemed mild (2).

The primary clinical characteristic of hemophilia is prolonged spontaneous and/or traumatic bleeding, which most commonly occurs within the musculoskeletal system. This bleeding primarily manifests as intra-articular hemorrhages in large synovial joints, such as the ankle, knee, and elbow, and often extends to the shoulder, wrist, and hip joints. Hemophilic hemorrhages are also prevalent in muscle and mucosal soft tissues, while they are less common in other soft tissues, the brain, and visceral organs (1). In patients with severe hemophilia, however, bleeding can also occur in visceral organs, in addition to the more common joint and muscle bleeding. However, spontaneous splenic rupture in adults with hemophilia A has been reported infrequently. Furthermore, there is limited literature on the treatment of this patient population using SAE. Given the spleen’s crucial role in immune function, it is preferable to pursue spleen-preserving therapies whenever possible, a perspective that is now widely endorsed.

Coagulation factor replacement therapy is the first-line treatment for hemophilia; however, it is often insufficient to control active bleeding. In recent years, the development of interventional radiology has enabled transcatheter arterial embolization (TAE) to quickly identify and accurately embolize bleeding vessels. TAE was first utilized for hemostasis in cases of gastrointestinal hemorrhage in 1972 and has since become an important treatment for traumatic hemorrhage, gastrointestinal hemorrhage, tumor-related bleeding, postoperative hemorrhage, and other types of bleeding caused by various factors (3,4). Although rebleeding or reoperation may occur following TAE hemostasis, this technique can rapidly halt bleeding and reduce the risk of massive hemorrhage (5). Building on the success of TAE, the development of splenic artery embolization (SAE) has further enhanced the precision and effectiveness of transcatheter embolization techniques, particularly in complex bleeding scenarios. SAE has been reported and validated for clinical safety and efficacy over the past 20 years, demonstrating its advantages in controlling bleeding (6-8). In the following sections, we will present a case of SAE treatment in a patient with hemophilia A who experienced a spontaneous splenic rupture. We present this case in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-2025-141/rc).

Case presentation

A 20-year-old male patient with hemophilia A presented with intermittent epigastric distension and discomfort with a 3-day history. Physical examination revealed the following vital signs: temperature of 38.8 ℃, heart rate (HR) of 140 beats per minute, respiratory rate (RR) of 22 breaths per minute, and blood pressure (BP) of 107/77 mmHg. Abdominal examination indicated a soft abdomen with mild tenderness in the epigastric region. Hemoglobin levels were monitored upon admission, decreasing from 116 to 83 g/L. Coagulation tests revealed the following results: prothrombin time (PT) of 14.2 seconds, PT activity of 67%, activated partial thromboplastin time (APTT) of 63.6 seconds, and fibrinogen (FIB) level of 6.44 g/L. Coagulation factor activity for FVIII was measured at 5%, classifying the condition as moderate based on FVIII activity. An enhanced computed tomography (CT) scan of the abdomen conducted at our hospital suggested a mass-like area of increased density in the splenogastric hiatus, possibly indicating a simple hematoma or a splenic cyst within the spleen, along with potential hemorrhage and pelvic bleeding (Figure 1A-1C). All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s). This study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Figure 1 Imaging of a patient with spontaneous splenic rupture. (A-C) Ruptured spleen with hematoma formation (red arrows). (D) Angiography demonstrated a collateral vessel distal to the inferior pole of the splenic artery with evidence of contrast extravasation (red arrow). (E,F) Embolization with gelatin sponge particles resulted in successful occlusion of the target vessel.

After a thorough review of the medical history, which revealed no recent trauma or minor injuries, the likelihood of spontaneous splenic rupture (a grade 4 splenic laceration) (9) was considered high, due to the high risk of hemorrhagic complications associated with surgery, the decision was made to proceed with interventional radiology after administration of coagulation factors, the patient was administered FVIII supplementation, cryoprecipitate transfusion, and fresh frozen plasma. Interventional radiologists successfully performed a super-selective SAE (Figure 1D-1F). Following these interventions, the patient’s FVIII level increased to 31%, and hemoglobin stabilized at 100 g/L. After achieving stabilization and improvement, the patient was transferred to the hemophilia center for further consultation. After the patient was discharged, we conducted a telephone follow-up. The family reported that the patient has since been receiving regular treatment at the designated hemophilia center and has had no further bleeding episodes.

Discussion

Coagulation factor replacement therapy is effective in the vast majority of cases and is considered the treatment of choice in individuals with hemophilia who experience bleeding (10-12). However, coagulation factor replacement therapy may be less effective in some cases, particularly in patients with unexplained bleeding or the presence of inhibitors (13,14). In such cases, TAE may serve as an alternative treatment strategy.

Sharma et al. reported two patients with traumatic splenic rupture due to hemophilia, both of whom experienced transient hemodynamic instability. One patient was a 39-year-old male with hemophilia A who underwent successful SAE, while the other was a 13-year-old male with hemophilia B whose condition improved with conservative management (15). Terry and Boswell presented a case of a 13-year-old boy who exhibited a grade 4 splenic laceration, acute blood loss anemia, and hypotension, which was managed nonoperatively (16). A study by Xia et al. indicated that SAE for traumatic splenic rupture could significantly shorten operative time, reduce the incidence of postoperative complications and intraoperative hemorrhage, and improve postoperative immune function (17). Consequently, there are no known reports of spontaneous splenic rupture in hemodynamically stable hemophilia A patients treated with SAE. SAE may be considered for patients with contraindications to surgery; however, further studies are necessary to confirm its efficacy and role (14). The hemophiliac in this study had a moderate deficiency of FVIII. Although he was hemodynamically stable, the risk of hemorrhage from surgery was extremely high; however, after a multidisciplinary team (MDT) consultation, he was successfully treated with SAE.

In a study conducted by Kim et al., angiography was performed on five hemophiliac patients who were experiencing difficult-to-control bleeding after treatment with coagulation factor concentrate replacement or bypass medications (14). Four patients—two with spontaneous bleeds, one with retroperitoneal hemorrhage, and one with post-pneumonia hemoptysis—underwent TAE and were discharged in improved condition. The results of a retrospective study by Spiliopoulos et al. indicated that TAE is safe and effective in selected cases, extending the therapeutic window for patients. Among the 17 patients in the study, two with hemophilia experienced iatrogenic bleeding: one with a right renal artery branch hemorrhage following a renal biopsy and the other with a medically induced pseudoaneurysm of the right deep femoral artery. Both patients achieved successful hemostasis through TAE without hemorrhage recurrence (18). These studies confirm that TAE is a safe, feasible, and effective intervention for hemophiliac patients experiencing bleeding.

Conclusions

This study demonstrates the effectiveness of SAE in controlling bleeding through a case of spontaneous splenic rupture in a patient with hemophilia A. Following SAE, the patient’s bleeding was successfully managed, saving his life and avoiding the risks associated with delayed surgery. This case underscores the importance of SAE as a spleen-preserving treatment option for patients with hemophilia A. Since the spleen plays a vital role in immune function, preserving it should be prioritized whenever possible. However, this case also highlights the need for careful evaluation of the patient’s overall condition, including coagulation factor levels and hemodynamic stability, when considering SAE in hemophilia patients. Additional case studies are necessary to confirm the effectiveness of SAE in managing spontaneous splenic rupture among hemophilia patients.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-2025-141/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-2025-141/prf

Funding: This study was supported by Cuiying Scientific and Technological Innovation Program of The Second Hospital & Clinical Medical School, Lanzhou University (No. CY2024-MS-A07) and Municipal Special Healthcare Program of Lanzhou Science and Technology Bureau.

Conflicts of Interest: Both authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-2025-141/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 performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s). This study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from patient for the publication of this case report 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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