Article, Hematology

Spontaneous rupture of the spleen: a rare but important differential of an acute abdomen

Case Report

Spontaneous rupture of the spleen: a rare but important differential of an acute abdomen

Abstract

Spontaneous rupture of the spleen, although previously documented, is a rare phenomenon. It commonly occurs in a pathologic spleen, usually owing to hematological manifesta- tion. We describe a rare incident of Spontaneous splenic rupture presenting to an emergency department as a first manifestation. The purpose of this case report is to highlight the importance of considering spontaneous rupture of the spleen as a rare but important differential of an acute abdomen.

Spontaneous “pathologic” rupture of the spleen is a rare complication of hematological malignancies. It is associated with high mortality and, therefore, requires early diagnosis and treatment to avert a fatal outcome. We describe a case of nontraumatic splenic rupture presenting as the first manifesta- tion of an underlying infiltrating acute lymphoblastic leukemia. A 23-year-old previously healthy man was brought in by ambulance to the emergency department with a 5-hour history of severe abdominal pain and a background of

24 hours preceding back pain. On admission, he was clinically shocked with tachypnea, tachycardia, an unrecord- able blood pressure, and a Glasgow Coma Scale score of 13. Cardiorespiratory examination was unremarkable, but his abdomen was grossly distended with tenderness in the left upper quadrant and absent bowel sounds.

Immediate fluid resuscitation was instigated, and basic laboratory investigations were requested. A portable ultra- sound scan demonstrated free fluid in the abdomen, and laboratory findings included pH 6.7; hemoglobin level, 5.2; white blood cell count, 142.9; and platelets, 34. The patient was subsequently intubated and aggressively resuscitated, including 6 U of universal Rhesus-negative, “O”-negative blood transfusion; inotropic support; and sodium bicarbonate infusion. Despite such measures, the patient became increasingly unstable.

The patient had an emergency laparotomy within 1 hour of presentation, which revealed 3 L of free blood in the peritoneal cavity and a massively enlarged soft spleen, ruptured along its convex posterior surface. The splenic artery and vein were immediately clamped, and the spleen was removed. Sixteen units of blood had been transfused

perioperatively with 4 U of fresh frozen plasma and platelets. Despite this, the patient had an abnormal bleeding time and eventually developed disseminated intravascular coagulo- pathy postoperatively. The patient continued to bleed on intensive care unit from his abdominal drain, wound sites, and nose. A total of 26 U of Packed red blood cells, 20 U of fresh frozen plasma, 20 U of cryoprecipitate, and 6 pools of platelets was transfused. He received vitamin K and Novoseven (Novo Nordisk Ltd, West Sussex, UK) (recom- binant coagulation factor VIIa) cover for a relaparotomy. This revealed no obvious bleeding site but continued generalized ooze from raw sites, and therefore, the abdominal cavity was packed. Further laparotomy to remove the abdominal packs was performed 48 hours later. The patient sadly developed 5 organ system failures despite continued ITU management and finally succumbed to progressive disseminated intravas- cular coagulopathy and sepsis.

Pathology confirmed an enlarged spleen measuring 210 x 140 x 80 mm (weighing 1080 g) with a disrupted capsule. The splenic parenchyma was nodular, soft, and friable with 1 hilar lymph node (10 mm in its maximum dimension). Micro- scopically, the spleen and hilar lymph nodes showed a diffuse infiltrate of monomorphic lymphoid cells, and a histologic diagnosis of precursor T-lymphoblastic leukemia was made. A true “spontaneous” Nontraumatic rupture of a nondiseased spleen, as defined by Orloff and Peskin [1], is extremely rare. The spleen is often involved in hematological malignancies, and although rare, the nontraumatic pathologic splenic rupture is now well chronicled in the literature [2,3]. The commonest cause is hematological malignancies, but others include infection (Epstein-Barr virus, malaria, HIV), pancreatitis, and other rarer congenital and miscellaneous causes [4-7]. Within the Bauer et al [2] series of 53 splenic rupture cases with hematological malignancies, the acute leukemias were com- monest (40%), followed by Hodgkin’s and non-Hodgkin’s lymphoma (27%) and chronic lymphocytic (CLL) leukemia and chronic myeloid leukemia (CML) (together, 18%). A larger series of Giagounidis et al [3] of 136 pathologic splenic rupture cases with hematological malignancies found 34% had acute leukemia; 34%, non-Hodgkin’s lymphoma; and

18%, CML.

Analysis of such case series revealed that most were in adults, and other risk factors for rupture were male sex and severe splenomegaly, although the latter did not seem to correlate with outcome [2,3]. Other factors apparently

0735-6757/$ – see front matter (C) 2008

733.e6 Case Report

unrelated to the prognosis included the disease type, duration, and past therapy [2,3]. Interestingly, the actual mechanism of splenic rupture is still unclear, but it is thought to be related to parenchymal congestion by the malignant cells [8]. Although current evidence is weak, other suggested factors are splenic infarcts and coagulopathies [2]. The only practical and most important determinant of survival in these patients was whether they received prompt surgical manage- ment or not: untreated, there was nearly a 100% mortality compared to two thirds surviving the early postoperative period [2,3].

Few cases of this catastrophic event have been reported as the first presentation of an underlying malignancy, and these were perhaps related to a poorer outcome [9-12]. Like previous reports of nontraumatic pathological splenic rupture, our case presented suddenly and with little warning. The combination of severe upper abdominal pain, distension, and hemodynamic shock should alert the doctor to this catastrophic differential of the acute abdomen. It can be confused with a perforated viscus, pancreatitis, an aortic aneurism, or an acute coronary syndrome, particularly in the elderly and in the presence of Kehr’s sign (diaphragmatic irritation resulting in referred pain to the left shoulder). There is little evidence on the optimal initial imaging, but prompt portable ultrasound or computed tomographic scanning if the patient is stable helps identify free fluid within the abdomen and may reveal splenomegaly or evidence of splenic pathology and rupture. However, in such scenarios, surgeons should be involved at an earlier stage with a view for urgent laparotomy when clinical suspicion is high.

Shin-Jae Rhee BSc, MBBS Yezen Sheena MBBS Charles Imber MB, BChir Department of Surgery

University College London Hospital NHS Trust

NW3 2YT London, UK E-mail address: [email protected]

doi:10.1016/j.ajem.2007.11.003

References

  1. Orloff M, Peskin GW. Spontaneous rupture of the normal spleen, a surgical enigma. Surg Gynecol Obstet 1958;106:1-11.
  2. Bauer TW, Haskins GE, Armitage JO. Splenic rupture in patients with hematologic malignancies. Cancer 1981;48:2729-33.
  3. Giagounidis AAN, Burk M, Meckenstock G, Koch AJ, Schneider W. Pathologic rupture of the spleen in hematologic malignancies: two additional cases. Ann Hematol 1996;73:297-302.
  4. Papesch M, Watkins R. Epstein-Barr virus Infectious mononucleosis. Clin Otolaryngol 2001;26:3-8.
  5. Toussi HR, Cross KS, Sheehan SJ, Bouchier-Hayes D, Leahy AL. Spontaneous splenic rupture: a rare complication of acute pancreatitis. Br J Surg 1996;83:632.
  6. Yoshizawa J, Mizuno R, Yoshida T, Kanai M, Kurobe M, Yamazaki Y. Spontaneous rupture of splenic hamartoma: a case report. J Pediatr Surg 1999;34:498-9.
  7. Karassa FB, Isenberg DA. Spontaneous rupture of the spleen: an unusual complication of systemic lupus erythematosus. Lupus 2001;10:876-8.
  8. Hynes HE, Silberstein MN, Fawcett KJ. Spontaneous rupture of the spleen in acute leukemia. Report of 2 cases. Cancer 1964;17:1356-60.
  9. Saba HI, Garcia W, Hartmann RC. Spontaneous rupture of the spleen: an unusual presenting feature in Hodgkin’s lymphoma. South Med J 1983;76(2):247-9.
  10. Haj M, Zaina A, Wiess M, et al. Pathologic-spontaneous-rupture of the spleen as a presenting sign of splenic T-cell lymphoma–case report with review. Hepatogastroenterology 1999;46(25):193-5.
  11. Zieren J, Paul M, Scharfenberg M, Muller JM. The spontaneous splenic rupture as first manifestation of mantle cell lymphoma, a dangerous rarity. Am J Emerg Med 2004;22(7):629-31.
  12. Mason KD, Juneja SK. Spontaneous rupture of the spleen as the presenting feature of the blastoid variant of mantle cell lymphoma. Clin Lab Haematol 2003;25(4):263-5.