Atraumatic Splenic Rupture: An Unusual Presentation of Hairy Cell Leukemia

Abstract

Spontaneous splenic rupture is an uncommon but life threatening acute emergency. Up to one half cases of Atraumatic splenic rupture (ASR) are preceded by splenomegaly. Hematological malignancies can present with splenomegaly and have clinical features of left upper quadrant (LUQ) pain and fullness. Abdominal pain is one of the commonest reasons for consulting Gastroenterology in the in-patient setting. We present a case of a patient who developed symptoms due to ASR which was not evident on initial imaging. While there are multiple causes of ASR, neoplastic etiologies account for about a third of these cases. Hairy Cell Leukemia (HCL) is a rare lymphoid neoplasm which often presents with nausea, vomiting, and LUQ pain. Splenomegaly is a common manifestation of this disease, although ASR occurs much less frequently. This report highlights a case of a 64-year-old woman with known comorbidities of hypertension and diabetes, who presented with persistent nausea, vomiting and epigastric pain, for one week. Prior to presentation, she had complained of intermittent abdominal pain for 16 months. A computerized tomography (CT) scan of abdomen done a year earlier had revealed a nonspecific wedgeshaped, low attenuating lesion in the spleen- suspicious for a splenic infarct. On examination, she was hemodynamically stable, with mild discomfort in the LUQ and palpable splenomegaly which was confirmed by imaging studies. The patient was thought to have gastroparesis secondary to uncontrolled diabetes mellitus, and was being treated conservatively. Patient was then noted to have a significant drop in Hematocrit without signs of active gastrointestinal bleeding. Her abdomen became distended with moderate LUQ tenderness. A repeat CT scan of abdomen showed evidence of intraperitoneal hemorrhage. The patient underwent emergent angiography and a bleeding splenic vessel was identified and embolized. She underwent an open splenectomy for ASR. A 6 cm laceration was found on the lower pole of the spleen. Pathology of the specimen unexpectedly returned positive for HCL. Patients with splenomegaly often present with symptoms of nausea, vomiting, and minimal LUQ tenderness. Careful clinical evaluation and urgent investigations should be undertaken in these patients as they may develop serious complications. ASR is an important and life threatening complication which may develop acutely or insidiously and should be monitored for in any patient with splenomegaly of unknown cause.Figure 1Figure 2