Life-threatening anemia with severe and acute bleeding due to probable uterine adenomyosis: a case report from Syria

Introduction

Adenomyosis is a common, benign, estrogen-dependent gynecological disease, usually seen in reproductive-age women (1,2). It is described by endometrial glands and stroma pathologically demonstrated within the myometrium, surrounded by smooth muscle hyperplasia (1).

Although adenomyosis is considered a disease of multiparous women over 40-year-old, it is increasingly identified in young nulliparous women, affecting their fertility potential. True prevalence of adenomyosis is unknown, as histopathologic examination after hysterectomy is the gold standard for diagnosis. Similarly, prevalence at imaging is also unclear, since there are many controversies in terms of imaging diagnostic criteria (1). But generally, prevalence ranges from 5% to 70% (3).

Symptoms of adenomyosis and their severity are heterogeneous among women, but classically menorrhagia, dysmenorrhea, chronic pelvic pain, and infertility are the most common (1,4). It is classified in the PALM-COEIN FIGO classification causes of abnormal uterine bleeding (AUB) (Polyp, Adenomyosis, Leiomyoma, Malignancy and hyperplasia, Coagulopathy, Ovulatory dysfunction, Endometrial, Iatrogenic, and Not yet classified-International Federation of Gynecology and Obstetrics) (3). However, one-third of patients are asymptomatic (1). Moreover, this disease often coexists with other gynecological disorders like leiomyoma and endometriosis (5).

Diagnosis of adenomyosis have been more possible and accurate recently, due to the development of non-invasive diagnostic tools [magnetic resonance imaging (MRI) and Doppler transvaginal ultrasound (TVUS)]. (6). As for now, TVUS is considered first step in approaching and diagnosing adenomyosis especially in the hands of experts (7), 3D TVUS showed best sensitivity when adjusting the junctional zone and examine the infiltration and distortion it (8). Many tries were done to have specific criteria to diagnose adenomyosis by ultrasound (US) as the morphological uterus sonographic assessment (MUSA) 2015, 2018 and 2021 criteria (7). However, there is still a lack of consensus on imaging diagnostic criteria (6).

We present the first case in Syria, showing adenomyosis as the probable main cause of severe and acute life-threatening anemia. Although literature has reported six cases of severe anemia with hemoglobin (Hb) levels >2 g/dL, only one of them has demonstrated adenomyosis as a coexisting disease with multiple uterine leiomyoma. Nevertheless, none of them has presented with acute hemorrhage (9-12). We present this article in accordance with the CARE reporting checklist (available at https://amj.amegroups.com/article/view/10.21037/amj-24-3/rc).

Case presentation

A 39-year-old multigravida woman, G₄P₄A₀L₄, with no relevant medical history, was referred from another hospital to our emergency department of our university hospital (Hospital of Obstetrics and Gynecology, Faculty of Medicine, Damascus University) in 2023, for acute and severe bleeding and Hb level 2.7 g/dL. Patient was shocked, not oriented, and patient’s history taken from her family revealed that for the past 5 days she has been experiencing irregular mild vaginal painless bleeding, which has increased in intensity and severity on the day of presentation. Two units of red blood cells were transfused while referring to our hospital. Except that, patient’s menstrual cycles used to be regular. She had never used any form of oral contraceptive pills, and had no relevant previous medical, obstetrical, surgical or familial history.

On admission, the patient is unconscious, pallid, and asthenic, with blood pressure (BP) of 80/60 mmHg, heart rate (HR) of 110 bpm, and weak pulse. There were no specific signs when examining the abdomen. Gynecological examination did not reveal anything but bleeding. Blood testing revealed Hb level of 6.5 g/dL, hematocrit (Hct) 17.8%, white blood cell count (WBC) 8,100/µL, platelet count 108,000/µL, createnine 1, serum glutamate pyruvate transaminase (SGPT) 51 U/L, serum glutamic-oxaloacetic transaminase (SGOT) 12 U/L, prothrombin time (PT) 79%, and international normalized ratio (INR) 1.16. Transvaginal ultrasonography showed normal uterus in shape, echogenicity, and size (6 cm × 6 cm), and normal endometrial thickness and adnexa (Figure 1).

Figure 1 Transvaginal ultrasonography showing normal uterine shape and echogenicity, measuring 6 cm × 6 cm without any signs of adenomyosis.

Patient was immediately transported to the intensive care unit (ICU). Despite continuous transfusing blood products and after completing of another 2 units of red blood cells, vaginal bleeding did not stop nor decreased, and patient’s status kept on deteriorating. A decision to take the patient to operation room and perform a hysterectomy was made. Pfannenstiel incision was made, and total hysterectomy was performed (Figure 2). Later, another 4 units of red blood cells and 8 units of plasma were transfused in the ICU where the patient stayed for the 24 hours postoperatively. Post-operative period was uneventful and the patient was discharged home on the fourth post-operative day with Hb level of 11.4 g/dL. Histopathological findings confirmed the diagnosis of adenomyosis (multiple focal clusters of endometrial glands) and complex glandular hyperplasia in the endometrium.

Figure 2 Gross inspection of total abdominal hysterectomy reveals normal uterine shape with diffuse thickened myometrium.

All procedures performed 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 case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

Adenomyosis is a chronic disease which usually presents with menorrhagia, dysmenorrhea, chronic pelvic pain, and infertility. However, it rarely presents in patients with acute bleeding. We present the first case in Syria, showing adenomyosis as the probable main cause of severe and acute life-threatening anemia (Hb level 2.7 g/dL).

Although it is the first case reported in Syria, and the life-saving management to our patient was done before knowing exactly the diagnosis, this case rises the concern regarding dealing with emergency patients. The fact that the US done before surgery with the hands of an expert did not show signs of adenomyosis says a lot about the importance of developing a specific criterion to diagnose adenomyosis and the importance of strict guideline regarding dealing with emergency patients. In our case, hysterectomy was done to save the life of the patient—which worked properly—however, was there any other option or way of management? It is maybe due to the late appearance of patient at our hospital that made us have no time to think or try other choices.

It had a lot of discussion in our hospital. The pathology confirmed the presence of adenomyosis as the major pathology. In excised specimen, but it was a debate between pathologist regarding endometrial hyperplasia, we thought it was the best to document everything. Why did we expect that the acute bleeding and low Hb was due to adenomyosis not other pathology, first, endometrial hyperplasia does not make Hb level reaches these low numbers, and even if it did, transfusion blood products would make the patient stable as the uterine contract, the continuously deteriorating Hb levels despite transfusion and monitoring in the ICU made us think that there was a pathology interfering with normal defense mechanism as uterine muscle contraction and it is in our case the adenomyosis.

To date, only six cases of severe anemia with Hb levels >2 g/dL have been reported (9-12). Only one of them has demonstrated adenomyosis as a coexisting disease with multiple uterine leiomyoma (9). Other three cases were diagnosed as fibroid in different sites of the uterus (9,11), and the diagnosis in the last two cases was unknown (10,12). Nevertheless, none of them has presented with acute hemorrhage. The patient’s ages ranged from 21 to 49 years, which corresponds to our patient’s age (39 years old), and Hb levels ranged from 1.1 to 1.9 g/dL. Herein, in the present case, although Hb level is a little above these mentioned cases, but this is the first case that identifies adenomyosis as the main cause of life-threatening anemia.

Conclusions

Although adenomyosis is a common, chronic gynecological disease in reproductive-age women, which usually coexists with other gynecological disorders, it is uncommon to present with acute and life-threatening bleeding and anemia.

Acknowledgments

We really want to thank and appreciate the hard work and crucial help of Basel Ahmad M.D.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://amj.amegroups.com/article/view/10.21037/amj-24-3/rc

Peer Review File: Available at https://amj.amegroups.com/article/view/10.21037/amj-24-3/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://amj.amegroups.com/article/view/10.21037/amj-24-3/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) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for 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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