Myomatous erythrocytosis syndrome associated with an 8-kg uterine leiomyoma: a case report of diagnostic evaluation and perioperative management.
In patients with uterine leiomyoma and unexplained erythrocytosis, consider myomatous erythrocytosis syndrome and the potential benefits of surgical intervention.
Where it sits
this study against the rest of the leuprorelin corpusSummary and findings
This case report describes a 55-year-old woman with marked erythrocytosis associated with a giant uterine leiomyoma, which persisted despite long-term leuprorelin treatment. After ten therapeutic phlebotomies and a total abdominal hysterectomy, hemoglobin levels decreased from 20.1 g/dL to 11.4 g/dL at one-month follow-up. The tumor was histologically confirmed as benign.
Abstract
Myomatous erythrocytosis syndrome (MES) is a rare condition in which erythrocytosis associated with uterine leiomyoma resolves after tumor removal. A 55-year-old woman with a giant uterine mass that had continued to enlarge despite long-term leuprorelin treatment was referred for marked erythrocytosis. Treatment-related amenorrhea made the timing of natural menopause uncertain. Magnetic resonance imaging showed a heterogeneous uterine mass exceeding 30 cm. Hemoglobin was 20.1 g/dL and serum erythropoietin (EPO) was 45.4 mIU/mL. BCR-ABL1 testing was negative, and no JAK2 V617F, JAK2 exon 12, CALR, or MPL mutation was detected, supporting secondary rather than clonal erythrocytosis. Ten weekly therapeutic phlebotomies reduced hemoglobin to 15.8 g/dL. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed after multidisciplinary perioperative planning. Prominent omental collateral vessels and markedly dilated ovarian veins increased surgical complexity. Because the giant tumor restricted posterior pelvic access, hysterectomy was completed using a retrograde approach. The 8060-g specimen was histologically confirmed as a benign uterine leiomyoma, and superficial cystic structures were identified as D2-40-positive dilated lymphatic channels. No thrombotic event occurred. At one-month follow-up, hemoglobin and serum EPO had decreased to 11.4 g/dL and 16.0 mIU/mL, respectively, without recurrent erythrocytosis. Although tumor EPO expression was not evaluated, postoperative normalization of serum EPO and resolution of erythrocytosis supported the clinical diagnosis of MES. MES should be considered in patients with uterine leiomyoma and unexplained erythrocytosis; hematologic evaluation, preoperative phlebotomy, and multidisciplinary surgical planning may enable safe treatment.
Background
Myomatous erythrocytosis syndrome (MES) is characterized by erythrocytosis linked to uterine leiomyoma, which typically resolves post-tumor removal. Prior knowledge indicates that treatment options and outcomes for MES are limited, necessitating further exploration of management strategies. This case report highlights the diagnostic evaluation and perioperative management of a patient with a significant uterine mass and associated erythrocytosis.
Methods
This case report describes a 55-year-old woman with a giant uterine leiomyoma exceeding 30 cm, who underwent ten therapeutic phlebotomies and a total abdominal hysterectomy with bilateral salpingo-oophorectomy. The primary outcome measures included changes in hemoglobin and serum erythropoietin levels. The surgical approach was adapted due to the size of the tumor and associated complications.
Results
The primary endpoint showed that hemoglobin decreased from 20.1 g/dL to 15.8 g/dL after ten phlebotomies. At one-month follow-up, hemoglobin further decreased to 11.4 g/dL, and serum EPO decreased from 45.4 mIU/mL to 16.0 mIU/mL. No thrombotic events occurred during the perioperative period.
Interpretation
The findings support the notion that surgical intervention can lead to normalization of hemoglobin and serum EPO levels in patients with MES. However, the clinical significance of the changes in hemoglobin and EPO levels should be interpreted cautiously, considering the absence of long-term follow-up data and the single case nature of the report. The lack of evaluation of tumor EPO expression also limits the conclusions that can be drawn regarding the pathophysiology of MES.
Key findings
- Hemoglobin was 20.1 g/dL and serum erythropoietin (EPO) was 45.4 mIU/mL before treatment.
- Ten weekly therapeutic phlebotomies reduced hemoglobin to 15.8 g/dL.
- At one-month follow-up, hemoglobin decreased to 11.4 g/dL and serum EPO to 16.0 mIU/mL.
Limitations
- Single case report limits generalizability.
- No long-term follow-up data reported.
- Absence of tumor EPO expression evaluation.