Giant Uterine Fibroid Complicated by Abnormal Erythrocytosis in a 33-Year-Old Woman: A Case Report

Abstract

Patient: Female, 33-year-old

Final Diagnosis: Myomatous erythrocytosis syndrome

Symptoms: Progressive abdominal distension • plethoric facies • reddish-purple nail beds

Clinical Procedure: —

Specialty: Obstetrics and Gynecology

Objective: Rare disease

Background

Uterine fibroids are common benign gynecologic tumors characterized by abnormal uterine bleeding, menorrhagia, and anemia. Myomatous erythrocytosis syndrome (MES), a rare secondary erythrocytosis associated with fibroids, presumably results from ectopic erythropoietin production by the fibroids, which activates the Janus kinase 2 (JAK-2) pathway and increases red blood cell counts. MES clinically presents with abdominal distension, skin discoloration, and menstrual irregularities. Management is complex due to substantial risks of intraoperative bleeding and thromboembolism. This report describes a 33-year-old woman with MES. After myomectomy, her erythrocytosis normalized, but she developed postoperative bleeding.

Case Report

A 33-year-old woman presented with a 2-year history of progressive abdominal distension and an abdominal mass comparable in size to a 20-week gravid uterus, plethoric facies, and reddish-purple nail beds. Her hemoglobin level was 19.7 g/dL; hematocrit was 60.4%. Ultrasound and computed tomography confirmed a 25×20×12 cm fibromatous uterus. To reduce thromboembolic risk, she received low-molecular-weight heparin and intravenous hydration preoperatively. Abdominal myomectomy was performed, during which substantially increased uterine and fibroid vascularity was observed. However, the patient experienced severe postoperative hemorrhage (1800 mL/24 h), warranting a second operation for hemostasis. She recovered well and was discharged on postoperative day 14; her hemoglobin level normalized. Pathologic examination confirmed uterine leiomyoma, supporting the diagnosis of MES.

Conclusions

MES is a rare condition characterized by large uterine fibroids and erythrocytosis; postoperative bleeding and thrombosis are common complications. This case highlights the importance of accurate diagnosis and effective management. Erythropoietin-related therapeutic targets for MES are needed.

Introduction

Uterine fibroids, the most common benign tumors of the female reproductive system, frequently cause chronic menorrhagia in approximately 30% of symptomatic patients, leading to iron-deficiency anemia in up to 50% of these individuals; paradoxically, they can also induce erythrocytosis [1–3]. This rare phenomenon, termed myomatous erythrocytosis syndrome (MES), is characterized by increased erythrocyte mass. Surgical resection of fibroids consistently normalizes hematologic parameters, fulfilling diagnostic criteria for paraneoplastic erythrocytosis [4–6]. Since its first description in 1953, fewer than 60 cases of MES have been reported worldwide [6,7]. The recognized mechanism involves erythropoietin (EPO) secretion by uterine fibroids, which drives red blood cell overproduction. MES treatment is challenging because large fibroids and severe erythrocytosis increase thrombotic and postoperative bleeding risks [8]. This case report describes a patient with MES who developed postoperative bleeding after abdominal myomectomy, warranting a second operation for hemostasis. This case highlights the complexity of diagnosing and treating MES; it suggests that future research should focus on optimizing perioperative management strategies and exploring EPO-related therapeutic targets.

Case Report

A 33-year-old woman presented with a 2-year history of progressive abdominal distension. On physical examination, she exhibited plethoric facies, reddish-purple nail beds, and an enlarged abdominal mass comparable in size to a 20-week gravid uterus. She denied cardiopulmonary symptoms, tobacco use, and residence at high altitude. Ultrasound and computed tomography revealed a large, round mass occupying the abdominal and pelvic cavities, consistent with a large uterine fibroid. Abundant and tortuous vascularity was noted within the mass (Figure 1).

Figure 1.

Computed tomography and ultrasound scans show a pelvic and abdominal mass measuring 24.74×12.83 cm, originating from the uterus, with indistinct boundaries between the mass and uterine tissue. The cervix is also visible (A, B). Ultrasound imaging demonstrates abundant vascular flow signals on the surface of the mass (C).

Admission laboratory testing demonstrated severe erythrocytosis and an abnormal coagulation profile. The patient’s hemoglobin level was 19.7 g/dL, and her hematocrit was 60.4%. Coagulation studies showed a prolonged activated partial thromboplastin time (aPTT) of 50.7 s (reference 28–44 s) and a low fibrinogen level of 2.01 g/L (reference 2–4 g/L). The prothrombin time was 15.8 s (reference 11–16 s), with an international normalized ratio of 1.26 (reference 0.81–1.36) and decreased prothrombin activity of 69% (reference 80–120%). Testing of the serum EPO level or Janus kinase 2 (JAK-2) mutations was not conducted. Additionally, attempted venesection at another institution was unsuccessful due to clotting in the median cubital vein.

After multidisciplinary team consultation, the decision was made to proceed with an abdominal myomectomy. Because of the patient’s erythrocytosis and history of oral contraceptive use, preoperative management included intravenous hydration for hemodilution and prophylactic enoxaparin (4000 IU once daily), which was initiated 3 days before surgery and discontinued 24 h preoperatively. Intraoperatively, a large mass originating from the uterine fundus with a smooth surface was identified, measuring approximately 25×20×12 cm (Figure 2). The upper portion of the mass was partially covered by the greater omentum, and the anterior uterine wall demonstrated abundant vasculature (Figure 3). The mass was excised, with approximately 100 mL of intraoperative blood loss.

Figure 2.

A large mass originating from the uterine fundus, measuring approximately 25 ×20×12 cm.

Figure 3.

The anterior uterine wall showed abundant blood vessels (arrow).

Two hours postoperatively, pelvic drainage reached 500 mL, with dark red, blood-tinged fluid. In response, 20 IU of oxytocin was administered intravenously, 0.2 mg of ergometrine maleate was administered intramuscularly to enhance uterine contraction, and 1 g of tranexamic acid was administered intravenously for hemostasis. Concurrent laboratory investigations revealed the following coagulation parameters: prothrombin time 18.1 s, aPTT 39.3 s, international normalized ratio 1.52, fibrin degradation products 61.27 μg/mL (reference 0–5 μg/mL), D-dimer 12 530 ng/mL (reference 0–500 ng/mL), and hemoglobin 14.1 g/dL. Given these abnormalities, 200 mL of fresh frozen plasma were transfused to address the coagulopathy. However, within 24 h postoperatively, pelvic drainage totaled 1800 mL of predominantly bright red blood, indicating substantial bleeding. Although coagulation parameters showed no major change, the hemoglobin level declined to 9.2 g/dL, prompting a second emergency operation. During this procedure, abundant vascularity on the uterine surface was observed, with active bleeding at suture sites. The sutures were reinforced, and a B-Lynch suture technique was utilized to achieve hemostasis. Oxytocin was also administered intramyometrially, and the patient’s vital signs were closely monitored.

Postoperative pathologic examination confirmed a uterine leiomyoma without degeneration. On postoperative day 3, low-molecular-weight heparin was administered to prevent thrombosis. The hemoglobin level subsequently increased to 11.3 g/dL and remained stable over the following months (Figure 4). The patient recovered well and was discharged on postoperative day 14.

Figure 4.

Perioperative hematologic trajectory. Left longitudinal axis indicates hemoglobin (g/dL), whereas right axis indicates hematocrit (HCT,%). The horizontal axis represents days since admission. A rapid decline in hemoglobin and HCT occurred on the first postoperative day due to postoperative bleeding. At the 6-month follow-up, both parameters had normalized and remained within the normal range, supporting the diagnosis of myomatous erythrocytosis syndrome.

Discussion

This case report describes a postoperative bleeding complication in a patient with MES after treatment. It demonstrates recognition of the classic presentation of this condition, outlines the clinical diagnostic process, and discusses effective management.

The leading hypothesis suggests that MES results from ectopic EPO secretion by fibroids, which binds to the erythropoietin receptor (EPOR) and activates the JAK-2 signaling pathway, promoting proliferation and differentiation of erythroid progenitor cells [8,9]. Pollio et al found that both EPO and EPOR are strongly expressed in fibroids; EPOR is particularly prominent in vascular endothelial cells [10]. This interaction stimulates cellular proliferation and angiogenesis, supporting tumor growth [11]. Furthermore, 95% of analyzed fibroids have exhibited detectable EPO mRNA and protein expression, indicating a dose-dependent growth regulation mechanism [12]. This mechanism also explains the association of MES with large fibroids and abundant vasculature – local hypoxia from expanding fibroids further stimulates EPO secretion, exacerbating erythrocytosis.

Mui et al reviewed 57 MES cases and reported a mean presentation age of 48.7 years. Common signs and symptoms included abdominal and pelvic distension or masses, skin discoloration, and menstrual irregularities [8]. It is critical to distinguish polycythemia vera, a myeloproliferative neoplasm characterized by JAK-2 mutations and normal or low EPO levels, from MES, which presents with elevated serum EPO levels in the absence of JAK-2 mutations [13]. In the present case, relevant diagnostic tests were not performed due to insufficient awareness of MES, and the diagnosis was made retrospectively based on available findings.

Large uterine fibroids and abundant vasculature in MES pose substantial intraoperative and postoperative bleeding risks. Additionally, associated erythrocytosis greatly increases the risk of arterial and venous thromboembolism [14,15]. Hysterectomy and myomectomy remain the primary therapeutic options for MES [8]. This report of a 33-year-old woman who experienced severe postoperative hemorrhage (1800 mL) after myomectomy illustrates a rare but serious complication.

Analysis of serial coagulation profiles clarifies this paradox. The preoperative findings – considerable prolongation of aPTT, hypofibrinogenemia, and decreased prothrombin activity – indicated chronic, localized consumptive coagulopathy. Although prophylactic enoxaparin may have contributed, the low fibrinogen level is a definitive marker of systemic clotting factor consumption, implicating the massive, hypervascular fibroid as the primary source. We speculate that the tumor served as a focus for continuous, low-grade intravascular coagulation within its extensive vascular network. The surgical procedure then acted as a potent trigger, overwhelming compensatory mechanisms and precipitating a fulminant systemic consumptive process. This decompensation was reflected by the catastrophic postoperative elevation of D-dimer and fibrin degradation products, which indicated massive fibrin formation and degradation, ultimately resulting in critical hemostatic failure unresponsive to fresh frozen plasma transfusion.

Our preoperative evaluation was inadequate. Therefore, we retrospectively developed a recommended diagnostic and treatment algorithm (Figure 5). Preoperatively, the following measures may be used to reduce hematocrit levels below 0.60 prior to surgery: weekly venesection (1–2 sessions per week), saline infusion for blood dilution, prophylactic administration of low-molecular-weight heparin and acetylsalicylic acid (75–81 mg/day), and physical therapy such as pneumatic compression stockings [8,16,17]. To mitigate intraoperative and postoperative bleeding risks, it is advisable to establish central venous access during surgery for rapid fluid resuscitation; administer gonadotropin-releasing hormone analogs to reduce fibroid volume [17]; and use oxytocin to promote uterine vasoconstriction. When dissecting larger fibroids, particular attention should be directed to their abundant and aberrantly proliferative vasculature, with careful dissection and identification of anatomical structures. Antonio Macciò and colleagues successfully treated a patient with a 5400-g uterine fibroid, erythrocytosis, and obesity using laparoscopic hysterectomy; laparoscopy is indicated for patients with multiple thrombotic risks [18]. Among patients who are ineligible for surgery or prefer nonsurgical options, uterine artery embolization is a safe and effective alternative. Uterine artery embolization substantially reduces postoperative complications and maintains hematocrit levels in the normal range, without requiring substantial reduction in fibroid size [19,20].

Figure 5.

Diagnostic and treatment flowchart for myomatous erythrocytosis syndrome. GnRH, gonadotropin-releasing hormone.

Conclusions

This case report describes a rare instance in which a young patient with MES underwent myomectomy and experienced severe postoperative bleeding. It underscores the importance of accurate diagnosis and effective management of MES. Patients presenting with large uterine fibroids and erythrocytosis should be carefully evaluated for this condition. Perioperative management must consider the increased risks of postoperative bleeding and thrombosis. Future therapeutic strategies for MES should also consider exploration of EPO-related targets.