SRU TOSHIBA RESIDENT TEACHING CASE
Atraumatic Splenic Rupture Maaz Sohail Maqbool, MB, BCh, BAO, Ruth Govier Brush, MD, and Mindy Northrup, RDMS, RVT CLINICAL HISTORY A 56-year-old white male patient with chronic myelomonocytic leukemia presented with acute severe left upper quadrant pain, anemia, thrombocytopenia, and increasing leukocytosis. A bone marrow biopsy demonstrated transformation to acute myelomonocytic leukemia, and induction chemotherapy was initiated. The initial computed tomography (CT) findings are discussed subsequently. Two days after the initiation of induction chemotherapy, the patient developed worsening of the left upper quadrant pain. The patient denied any history of trauma. Laboratory examination revealed increasing anemia, and an abdominal ultrasound was performed followed by emergent CT (Figs. 1Y3). The findings confirmed the diagnosis of atraumatic splenic rupture (ASR).
DISCUSSION Atraumatic splenic rupture, also known as pathologic or spontaneous splenic rupture, is a rare life-threatening condition first described in 2 patients with leukemia by Rokitansky in 1861.1 As evidenced by its name, ASR excludes splenic rupture secondary to blunt trauma or iatrogenic causes (eg, surgical, interventional, and invasive medical procedures). Its incidence is unknown; however, a recent systematic literature review of 632 publications reporting 845 cases dating from 1980 to 2008 documented an average of 30 newly described cases per year,2 with a 2:1 male predominance and a mean age of 45 years. In 93% of the cases, there was an underlying pathologic condition, which were grouped into 6 categories in order of most to least frequent occurrence as follows: neoplastic (including malignant and nonmalignant hematologic conditions), infectious (predominantly mononucleosis), inflammatory (eg, pancreatitis, vasculitis), drug/treatment related, mechanical (eg, peripartum, thrombotic), and idiopathic. The pathophysiology of ASR is uncertain, and 3 underlying mechanisms have been hypothesized3 as follows: mechanical distention secondary to diffuse parenchymal infiltration (usually associated with leukemia/lymphoma), splenic infarct leading to capsular hemorrhage and rupture, and underlying coagulopathy. There have been case reports of ASR in patients with acute myeloid leukemia after administration of chemotherapeutic agents such as granulocyte colony-stimulating factor/granulocyte macrophage colonystimulating factor and idarubicin, as well as after peripheral blood stem cell transplantation. Imatinib has also been associated with splenic rupture in patients with chronic myelogenous leukemia with myelofibrosis.4,5 It has been hypothesized that granulocyte colonystimulating factor triggers extramedullary hematopoiesis and blood cell sequestration, leading to splenic congestion and rupture. Symptoms and signs of ASR include abdominal pain in all cases, left upper quadrant tenderness and rigidity, left shoulder pain (Kehr sign), nausea, vomiting, dizziness, hypotension, and syncope.6 Laboratory evaluation usually reveals anemia and marked leukocytosis in cases involving underlying hematologic and infectious conditions. Imaging evaluation with ultrasound has been shown to have 91% to 100% specificity for the diagnosis.7 A sonographic grading system of splenic injury has been developed that divides Received for publication May 8, 2014; accepted May 12, 2014. Department of Radiology, University of Missouri Healthcare, Columbia, MO. The authors declare no conflict of interest. Reprints: Maaz Sohail Maqbool, MB, BCh, BAO, Department of Radiology, University of Missouri Healthcare, One Hospital Dr, DC069.10, Columbia, MO 65212 (e-mail: [email protected]). Copyright * 2014 by Lippincott Williams & Wilkins
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Ultrasound Quarterly
& Volume 30, Number 3, September 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Ultrasound Quarterly
& Volume 30, Number 3, September 2014
Atraumatic Splenic Rupture
FIGURE 1. A and B, Axial and coronal contrast-enhanced CT images at presentation reveal homogeneous splenomegaly with perisplenic fluid.
FIGURE 2. A, Transabdominal gray scale ultrasound reveals large layering hematoma of varying echogenicity causing mass effect upon the spleen (asterisk). B, No evidence of mass or of active bleeding on color imaging.
FIGURE 3. AYC, Axial, coronal, and sagittal contrast-enhanced CT images demonstrate a large evolving subcapsular splenic hematoma and new ascites throughout the abdomen. * 2014 Lippincott Williams & Wilkins
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cases into low-grade or high-grade depending upon whether the splenic capsule is intact or ruptured.8 Computed tomography is utilized to determine the extent of intraperitoneal fluid and to assess for potential extrasplenic etiology or complications. The overall mortality rate of ASR is 12.2%, and the underlying neoplastic and infectious disorders are associated with a higher odds ratio of mortality.2 Other risk factors include male sex, splenomegaly, and age older than 40 years. The mainstay of treatment is emergent splenectomy and transfusion of blood products because conservative or nonoperative interventional management has lower success rates and increased complications eventually requiring splenectomy. Complications include splenic infarction, which has been found in up to 50% of patients with leukemia and in 20% of patients with lymphoma.8 Patients require vaccinations against encapsulated organisms to avoid postsplenectomy infections. Because of the rarity of ASR, a high index of clinical suspicion must be maintained in symptomatic patients with a relevant past medical history. Clinical assessment and laboratory evaluation should be accompanied by emergent ultrasound evaluation due to its ready availability and high sensitivity for detecting splenic rupture and intra-abdominal free fluid. If rupture is confirmed, emergent splenectomy and replacement of
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blood products is required, with patients subsequently requiring immunization to prevent postoperative infection. REFERENCES 1. Rokitansky KF. Two recent cases of spontaneous rupture of the spleen. Wochenblatt der Zeitschrift der k k Gesellschaft der Aerzte in Wien. 1861;17:42Y44. 2. Renzulli P, Hostettler A, Schoepfer AM, et al. Systematic review of atraumatic splenic rupture. Br J Surg. 2009;96(10):1114Y1121. 3. Kasper C, Jones L, Fujita Y, et al. Splenic rupture in a patient with acute myeloid leukemia undergoing peripheral blood stem cell transplantation. Ann Hematol. 1999;78:91Y92. 4. Veerappan R, Morrison M, Williams S, et al. Splenic rupture in a patient with plasma cell myeloma following G-CSF/GM-CSF administration for stem cell transplantation and review of the literature. Bone Marrow Transplant. 2007;40:361Y364. 5. Ganeshan DM, et al. Complications of oncologic therapy in the abdomen and pelvis: a review. Abdom Imaging. 2013;38:1Y21. 6. Tan A, Ziari M, Salman H, et al. Spontaneous rupture of the spleen in the presentation of acute myeloid leukemia. J Clin Oncol. 2007;25(34):5519Y5520. 7. Keller HW, Isenberg J, Takai S. Diagnose und Therapie der Milzruptur. Chirurg. 1995;66:1092Y1096. 8. Gorg C, Colle J, Gorg K, et al. Spontaneous rupture of the spleen: ultrasound patterns, diagnosis and follow-up. Br J Radiol. 2003;76:704Y711.
* 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Atraumatic Splenic Rupture Maaz Sohail Maqbool, MB, BCh, BAO, Ruth Govier Brush, MD, and Mindy Northrup, RDMS, RVT CLINICAL HISTORY A 56-year-old white male patient with chronic myelomonocytic leukemia presented with acute severe left upper quadrant pain, anemia, thrombocytopenia, and increasing leukocytosis. A bone marrow biopsy demonstrated transformation to acute myelomonocytic leukemia, and induction chemotherapy was initiated. The initial computed tomography (CT) findings are discussed subsequently. Two days after the initiation of induction chemotherapy, the patient developed worsening of the left upper quadrant pain. The patient denied any history of trauma. Laboratory examination revealed increasing anemia, and an abdominal ultrasound was performed followed by emergent CT (Figs. 1Y3). The findings confirmed the diagnosis of atraumatic splenic rupture (ASR).
DISCUSSION Atraumatic splenic rupture, also known as pathologic or spontaneous splenic rupture, is a rare life-threatening condition first described in 2 patients with leukemia by Rokitansky in 1861.1 As evidenced by its name, ASR excludes splenic rupture secondary to blunt trauma or iatrogenic causes (eg, surgical, interventional, and invasive medical procedures). Its incidence is unknown; however, a recent systematic literature review of 632 publications reporting 845 cases dating from 1980 to 2008 documented an average of 30 newly described cases per year,2 with a 2:1 male predominance and a mean age of 45 years. In 93% of the cases, there was an underlying pathologic condition, which were grouped into 6 categories in order of most to least frequent occurrence as follows: neoplastic (including malignant and nonmalignant hematologic conditions), infectious (predominantly mononucleosis), inflammatory (eg, pancreatitis, vasculitis), drug/treatment related, mechanical (eg, peripartum, thrombotic), and idiopathic. The pathophysiology of ASR is uncertain, and 3 underlying mechanisms have been hypothesized3 as follows: mechanical distention secondary to diffuse parenchymal infiltration (usually associated with leukemia/lymphoma), splenic infarct leading to capsular hemorrhage and rupture, and underlying coagulopathy. There have been case reports of ASR in patients with acute myeloid leukemia after administration of chemotherapeutic agents such as granulocyte colony-stimulating factor/granulocyte macrophage colonystimulating factor and idarubicin, as well as after peripheral blood stem cell transplantation. Imatinib has also been associated with splenic rupture in patients with chronic myelogenous leukemia with myelofibrosis.4,5 It has been hypothesized that granulocyte colonystimulating factor triggers extramedullary hematopoiesis and blood cell sequestration, leading to splenic congestion and rupture. Symptoms and signs of ASR include abdominal pain in all cases, left upper quadrant tenderness and rigidity, left shoulder pain (Kehr sign), nausea, vomiting, dizziness, hypotension, and syncope.6 Laboratory evaluation usually reveals anemia and marked leukocytosis in cases involving underlying hematologic and infectious conditions. Imaging evaluation with ultrasound has been shown to have 91% to 100% specificity for the diagnosis.7 A sonographic grading system of splenic injury has been developed that divides Received for publication May 8, 2014; accepted May 12, 2014. Department of Radiology, University of Missouri Healthcare, Columbia, MO. The authors declare no conflict of interest. Reprints: Maaz Sohail Maqbool, MB, BCh, BAO, Department of Radiology, University of Missouri Healthcare, One Hospital Dr, DC069.10, Columbia, MO 65212 (e-mail: [email protected]). Copyright * 2014 by Lippincott Williams & Wilkins
208
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Ultrasound Quarterly
& Volume 30, Number 3, September 2014
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Ultrasound Quarterly
& Volume 30, Number 3, September 2014
Atraumatic Splenic Rupture
FIGURE 1. A and B, Axial and coronal contrast-enhanced CT images at presentation reveal homogeneous splenomegaly with perisplenic fluid.
FIGURE 2. A, Transabdominal gray scale ultrasound reveals large layering hematoma of varying echogenicity causing mass effect upon the spleen (asterisk). B, No evidence of mass or of active bleeding on color imaging.
FIGURE 3. AYC, Axial, coronal, and sagittal contrast-enhanced CT images demonstrate a large evolving subcapsular splenic hematoma and new ascites throughout the abdomen. * 2014 Lippincott Williams & Wilkins
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Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
209
Ultrasound Quarterly
Maqbool et al
cases into low-grade or high-grade depending upon whether the splenic capsule is intact or ruptured.8 Computed tomography is utilized to determine the extent of intraperitoneal fluid and to assess for potential extrasplenic etiology or complications. The overall mortality rate of ASR is 12.2%, and the underlying neoplastic and infectious disorders are associated with a higher odds ratio of mortality.2 Other risk factors include male sex, splenomegaly, and age older than 40 years. The mainstay of treatment is emergent splenectomy and transfusion of blood products because conservative or nonoperative interventional management has lower success rates and increased complications eventually requiring splenectomy. Complications include splenic infarction, which has been found in up to 50% of patients with leukemia and in 20% of patients with lymphoma.8 Patients require vaccinations against encapsulated organisms to avoid postsplenectomy infections. Because of the rarity of ASR, a high index of clinical suspicion must be maintained in symptomatic patients with a relevant past medical history. Clinical assessment and laboratory evaluation should be accompanied by emergent ultrasound evaluation due to its ready availability and high sensitivity for detecting splenic rupture and intra-abdominal free fluid. If rupture is confirmed, emergent splenectomy and replacement of
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& Volume 30, Number 3, September 2014
blood products is required, with patients subsequently requiring immunization to prevent postoperative infection. REFERENCES 1. Rokitansky KF. Two recent cases of spontaneous rupture of the spleen. Wochenblatt der Zeitschrift der k k Gesellschaft der Aerzte in Wien. 1861;17:42Y44. 2. Renzulli P, Hostettler A, Schoepfer AM, et al. Systematic review of atraumatic splenic rupture. Br J Surg. 2009;96(10):1114Y1121. 3. Kasper C, Jones L, Fujita Y, et al. Splenic rupture in a patient with acute myeloid leukemia undergoing peripheral blood stem cell transplantation. Ann Hematol. 1999;78:91Y92. 4. Veerappan R, Morrison M, Williams S, et al. Splenic rupture in a patient with plasma cell myeloma following G-CSF/GM-CSF administration for stem cell transplantation and review of the literature. Bone Marrow Transplant. 2007;40:361Y364. 5. Ganeshan DM, et al. Complications of oncologic therapy in the abdomen and pelvis: a review. Abdom Imaging. 2013;38:1Y21. 6. Tan A, Ziari M, Salman H, et al. Spontaneous rupture of the spleen in the presentation of acute myeloid leukemia. J Clin Oncol. 2007;25(34):5519Y5520. 7. Keller HW, Isenberg J, Takai S. Diagnose und Therapie der Milzruptur. Chirurg. 1995;66:1092Y1096. 8. Gorg C, Colle J, Gorg K, et al. Spontaneous rupture of the spleen: ultrasound patterns, diagnosis and follow-up. Br J Radiol. 2003;76:704Y711.
* 2014 Lippincott Williams & Wilkins
Copyright © 2014 Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
