Myomatous erythrocytosis syndrome

Myomatous erythrocytosis syndrome is a rare medical condition characterized by the presence of uterine fibroids (myomas) and an associated increase in red blood cell mass, leading to erythrocytosis. This syndrome is notable for its unique combination of gynecological and hematological abnormalities.

Pathophysiology

The exact mechanism underlying myomatous erythrocytosis syndrome is not fully understood. However, it is believed that the uterine fibroids may produce erythropoietin, a hormone that stimulates the production of red blood cells in the bone marrow. This overproduction of erythropoietin leads to erythrocytosis, which is characterized by an elevated hematocrit and hemoglobin levels.

Clinical Presentation

Patients with myomatous erythrocytosis syndrome typically present with symptoms related to both the uterine fibroids and the increased red blood cell mass. Common symptoms include:

• Menorrhagia (heavy menstrual bleeding)
• Pelvic pain
• Abdominal distension
• Fatigue
• Headaches
• Dizziness

Diagnosis

The diagnosis of myomatous erythrocytosis syndrome involves a combination of clinical evaluation, laboratory tests, and imaging studies. Key diagnostic steps include:

• Complete blood count (CBC) to assess hemoglobin and hematocrit levels
• Measurement of serum erythropoietin levels
• Pelvic ultrasound or MRI to identify and evaluate uterine fibroids
• Exclusion of other causes of erythrocytosis, such as polycythemia vera and chronic hypoxia

Treatment

The treatment of myomatous erythrocytosis syndrome focuses on managing both the uterine fibroids and the erythrocytosis. Treatment options may include:

• Myomectomy or hysterectomy to remove the uterine fibroids
• Medications to reduce erythropoietin production or red blood cell mass
• Phlebotomy to decrease hematocrit levels

Prognosis

The prognosis for patients with myomatous erythrocytosis syndrome varies depending on the severity of the condition and the effectiveness of the treatment. Surgical removal of the uterine fibroids often leads to a resolution of the erythrocytosis and improvement in symptoms.

Related Pages

• Uterine fibroids
• Erythrocytosis
• Erythropoietin
• Myomectomy
• Hysterectomy
• Polycythemia vera