Case Presentation: A 45-year-old female with a history of a remote history of left tibia chondrosarcoma requiring surgical resection presented to our hospital with two weeks of persistent fevers after returning from Jamaica. She reported three months of drenching night sweats, 10-pound weight loss over two months, and generalized myalgias. Upon admission, imaging revealed diffuse neck, axilla, chest, retroperitoneal, and mesenteric lymphadenopathy and multiple hypoattenuating splenic masses, concerning for a hematologic malignancy or infectious process. On hospital day three, while awaiting Interventional Radiology (IR) lymph node biopsy, she acutely developed severe left upper quadrant (LUQ) abdominal pain radiating to her left shoulder while utilizing incentive spirometry (IS). Urgent CT scan of the abdomen and pelvis was done showing a Grade 5 splenic injury with active extravasation into the peritoneum with moderate LUQ hemoperitoneum. General surgery was immediately consulted and initially recommended consulting IR for intervention given she was hemodynamically stable. However, she quickly started to develop hemorrhagic shock requiring massive transfusion protocol, transfer to the surgical ICU, and underwent emergent exploratory laparotomy and splenectomy for spontaneous splenic rupture. Ultimately, pathology of the spleen revealed EBV-positive classic Hodgkin Lymphoma (HL). She transferred to the Bone Marrow Transplant team and received her first cycle of Doxorubicin, Vinblastine, and Dacarbazine on hospital day 10 and discharged on hospital day 11.
Discussion: This case highlights the rare but serious occurrence of spontaneous splenic rupture in a patient with undiagnosed HL, warranting immediate recognition and urgent intervention. Abdominal pain is a frequently reported symptom in patients with splenic rupture. In approximately 20% of cases, a sharp radiating pain to the left shoulder, also known as Kehr’s sign, is seen. This is caused by irritation of the diaphragm from blood or other irritants in the peritoneal cavity. In larger splenic injuries, hypovolemic shock can be a common presenting symptom. Ultrasound can be used in an emergent setting for the rapid identification of hemoperitoneum, but CT scan with IV contrast is the standard for diagnosis. Causes of atraumatic splenic rupture include infectious, inflammatory, neoplastic, drug or treatment related, pregnancy related, and idiopathic. Non-Hodgkin lymphomas such as Diffuse Large B-Cell Lymphoma and Mantle Cell Lymphoma, have been associated with spontaneous splenic rupture. Although rare, there have been reports of spontaneous splenic rupture in patients with HL. The pathophysiology of atraumatic splenic rupture in the setting of hematologic malignancy involves substantial splenic infiltration of malignant cells which can lead to splenomegaly and increased splenic fragility. There is no current evidence that suggests utilizing IS can lead to splenic rupture and was likely coincidental timing in our case and ultimately due to her underlying HL. Splenic rupture is a true surgical emergency that can result in the rapid onset of hemodynamically instability and death. In select, stable patients, nonoperative management or splenic artery embolization can be successful, less invasive approaches. However, emergent splenectomy is still the cornerstone of treatment for splenic rupture.
Conclusions: Spontaneous splenic rupture requires a high clinical index of suspicion with prompt recognition and treatment.