Spontaneous splenic rupture as a severe complication in a patient with chronic myeloid leukemia: a case report

¹Department of Clinical Hematology and Bone Marrow Transplantation, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakesh, Morocco

^&Corresponding author
Mohamed Zioui, Department of Clinical Hematology and Bone Marrow Transplantation, Mohammed VI University Hospital, Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakesh, Morocco

Introduction    

Spleen rupture, a life-threatening situation, is often due to abdominal or thoracic trauma [1]. Spontaneous or atraumatic splenic rupture is a very rare complication [2]. Spontaneous splenic rupture (SSR) was first described in the 19^th century [3]. In the majority of cases, SSR is caused by hematological malignancies (30.3%), infectious diseases (27.3%), and neoplastic diseases (20.0%). Other causes are drug-induced (9.2%) or mechanical disorders (6.8%) [4]. However, the association of SSR with chronic myeloid leukemia (CML) is very rare (0.72%) [5]. Splenectomy is the radical treatment for spontaneous splenic rupture [6]. We report a rare case of SSR as an acute complication of CML in a young man, requiring immediate diagnostic and therapeutic management to avoid hypovolemic shock.

Patient and observation     

Patient information: a 35-year-old patient, a former chronic smoker and previously treated for pulmonary tuberculosis. Referred to us for management of hyperleukocytosis found during investigation of persistent dyspnea.

Clinical findings: he was admitted conscious, apyretic, respiratory, and hemodynamically unstable, with oxygen saturation in arterial blood by pulse oximetry (SaO₂) of 92% in room air. Clinical examination found a distended abdomen with splenomegaly and signs of right heart failure: jugular vein turgor, painful hepatomegaly, and leg oedema.

Diagnostic assessment: the blood count showed hyperleukocytosis (leukocytes: 508 000/mm³, neutrophils: 259 080/mm³, lymphocytes: 1 016/mm³ with the presence of a myeloma: metamyelocytes: 111 760/mm³, myelocytes: 76 200/mm³ and promyelocytes: 50 800/mm³), thrombocytosis: 882 000/mm³ and hemoglobin level: 11.7g/dl. Bone marrow aspiration showed an appearance in favor of CML with an estimated 3% marrow blast count. Karyotype confirmed the presence of the translocation t(9;22) (Figure 1). A chest X-ray revealed cardiomegaly with bilateral interstitial syndrome. Abdominal CT scan showed homogeneous hepatosplenomegaly with dilatation of the suprahepatic veins and inferior vena cava.

Timeline of the current episode: he then presented with acute, severe abdominal pain with a CT-scan appearance consistent with SSR over heterogeneous splenomegaly with foci of infarction and extensive hemoperitoneum (Figure 2).

Therapeutic interventions: the patient was operated on for open splenectomy due to worsening hemodynamic status. Pathological examination of the splenectomy specimen revealed large necrotic foci with some atypical cells. Immunohistochemistry showed subacute inflammatory and necrotic changes with no underlying tumor proliferation.

Follow-up and outcome of interventions: the post-operative course was straightforward, the abdominal pain subsided, and the patient was put on an antiplatelet therapy based on low molecular weight heparin. The diagnosis of CML was retained, and the patient was put on Imatinib 400 mg/day with favorable follow-up and good clinical and biological outcome leading to hematological remission.

Patient perspective: during treatment, the patient was satisfied with the level of care provided to him.

Informed consent: the consent was obtained from the patient so that we could use the information for this case report.

Discussion     

Spontaneous splenic rupture or SSR in hematological malignancies is rare. Hodgkin's lymphoma and CML are the most frequently implicated [7]. Spontaneous splenic rupture can reach a mortality rate of up to 21% in the case of neoplastic diseases [8]. The physiophatology of SSR in hematological disorders is still unclear. In CML, SSR can be caused by infiltration of splenic parenchyma by tumor cells, leading to excessive spleen overload beyond the spleen's capacity to compensate and expand [9]. In addition, concomitant coagulation abnormalities may be responsible for subscapular hemorrhage and splenic infarction [5]. In our patient, leukemic infiltration and infarction are likely to be at the origin of the splenic rupture. However, our patient's hemostatic test was normal.

Diagnosis of SSR is very delicate. Patients present with acute abdominal pain, the most frequent symptom (95%) in patients with hematological malignancies, typically radiating to the left shoulder, combined with fever, nausea or emesis [4,5]. Abdominal ultrasound and CT scans, with a sensitivity and specificity of at least 95% in detecting splenic lesions, contribute to the diagnosis of splenic rupture and help rule out other causes of acute abdomen [5]. It often shows splenomegaly with splenic lacerations and intraperitoneal or subcapsular bleeding, as in our case [10]. Spontaneous splenic rupture (SSR) can be treated either surgically (splenectomy) or conservatively (clinical monitoring) [1]. Splenic artery embolization can be a safe and rapid procedure for patients for whom conservative management is planned [5]. However, the idea of performing a total splenectomy, the treatment option chosen in our case, may be justified due to tumor infiltration of the splenic parenchyma which may have already affected splenic function, resulting in functional hyposplenism [9].

Conclusion     

This case report illustrates a rare and critical entity. Every physician should be alert to the possibility of atraumatic splenic rupture in the presence of acute abdominal pain, particularly in patients with haematological malignancies. Accurate clinical diagnosis, abdominal CT scan and prompt therapeutic intervention can improve the prognosis of patients already compromised by neoplasia.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

Patient management: Mohamed Zioui, Fatima Zahra Lahlimi and Illias Tazi. Manuscript drafting: Mohamed Zioui and Fatima Zahra Lahlimi. Manuscript revision: Illias Tazi. All the authors have read and approved the final version of the manuscript.

Acknowledgments     

We thank Dr Abakarim Ouadii for his contribution to this work and all the clinical staff who participated in treating the patient.

Figures     

Figure 1: karyotype with t(9,22)

Figure 2: abdominal CT scan of splenic rupture

References