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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 7  |  Issue : 4  |  Page : 143-147

Splenic peliosis and rupture − A surgical emergency: Case report and review of the available literature


Department of Surgery, Aga Khan University & Hospital, Karachi, Pakistan

Date of Web Publication18-Jan-2017

Correspondence Address:
Dr. Muhammad R Khan
Associate Professor of Surgery, Department of Surgery, Aga Khan University & Hospital, Stadium Road, Karachi
Pakistan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1658-5127.198508

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  Abstract 

Peliosis is an extremely rare condition of unknown etiology, and its pathogenesis is characterized by multiple, cyst-like, blood-filled cavities predominantly involving a mononuclear phagocytic system. Isolated splenic peliosis is extremely uncommon, but may present as a potentially life-threatening situation. An awareness of its clinical condition and prompt management may improve the clinical outcomes. The role of prophylactic splenectomy is not clear in this condition.

Keywords: Peliosis, rupture, spleen


How to cite this article:
Begum S, Khan MR. Splenic peliosis and rupture − A surgical emergency: Case report and review of the available literature. J Appl Hematol 2016;7:143-7

How to cite this URL:
Begum S, Khan MR. Splenic peliosis and rupture − A surgical emergency: Case report and review of the available literature. J Appl Hematol [serial online] 2016 [cited 2020 May 29];7:143-7. Available from: http://www.jahjournal.org/text.asp?2016/7/4/143/198508


  Introduction Top


Peliosis is an extremely rare condition of unknown etiology, and its pathogenesis is characterized by multiple, cyst-like, blood-filled cavities predominantly involving a mononuclear phagocytic system (liver, spleen, bone marrow, and lymph nodes).[1] The liver is the most commonly involved organ; isolated splenic peliosis is extremely uncommon. Splenic peliosis has been found to be associated with chronic debilitating conditions, for example, malignancy, infections, and ingestion of certain medications, such as anabolic steroids.[2] Patients with splenic peliosis are usually asymptomatic, but may present with symptoms if the organ ruptures spontaneously leading to hemoperitoneum. We reviewed the available literature on this subject and reported another case of a 34-year-old woman who presented to our emergency room with severe abdominal pain and hemoperitoneum secondary to spontaneously ruptured spleen. She underwent an emergency laparotomy and splenectomy. Later, histopathological studies revealed her condition to be splenic peliosis.

Case Presentation

A 34-year-old, previously healthy woman, presented to the emergency department of our hospital with 12 h history of severe left upper quadrant abdominal pain. The pain was continuous and radiated to the back. There was no history of abdominal trauma or any other gastrointestinal symptoms. On initial assessment, she was hemodynamically stable but having pallor facies. Her abdomen was protuberant with mild generalized tenderness more marked in the left upper quadrant. Baseline investigations showed hemoglobin of 9.8 g/dL with hematocrit of 29.6%. Her platelets, coagulation profile, serum amylase, and lipase were within normal limits. Initial ultrasound of abdomen showed a 3 cm × 3 cm heterogeneous, predominantly echogenic, lesion in the spleen, which was suspected as hemangioma. There was some free fluid in the abdomen. Erect chest X-rays showed no evidence of pneumoperitoneum. Subsequently, a computerized tomography (CT) scan of the abdomen was done, which revealed a grade III laceration in the spleen along with moderate hemoperitoneum predominantly in the perisplenic location. A diagnosis of grade III splenic laceration was made, and the initial plan was conservative management with serial clinical assessment. During the next 12 h, she developed progressive tachycardia and a drop in hemoglobin to 7.9 g/dL. In view of her deteriorating clinical status, she was planned for an urgent exploratory laparotomy. Per-operatively, there was approximately 500 cc of frank blood in the peritoneal cavity. The spleen ruptured with a big clot around it; therefore, splenectomy was performed. She remained well after surgery and was discharged home on day 5 after surgery.

The spleen was sent for histopathological evaluation. On gross inspection, the size of the specimen was 10 cm × 6 cm × 3 cm, and weight was around 110 g. On microscopic examination, splenic parenchyma exhibited markedly dilated spaces filled with hemorrhage and focally fibrin; these findings were consistent with splenic peliosis [Figure 1] and [Figure 2].
Figure 1: Splenic parenchyma exhibiting markedly dilated spaces filled with hemorrhage and focal fibrin

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Figure 2: High-power view of splenic sinuses exhibiting splenic peliosis

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  Discussion Top


The term peliosis was first used by Wagner in 1861 to describe the gross appearance of lesions found on the cut surface of a liver affected by the condition.[3] It is mostly seen in organs belonging to the mononuclear phagocytic system including the liver (most common), the spleen, the bone marrow, and the lymph nodes; however, it can also appear in the lungs, the kidneys, and the parathyroid glands.[1] Isolated splenic peliosis was not reported till 1978,[4] as its pathological occurrence was considered only in association with the more common peliosis hepatis.[5]

The exact cause of peliosis is not clear; however, many etiological agents have been postulated as the cause. Drugs and toxins including chronic alcoholism, corticosteroids, oral contraceptives, tamoxifen, azathioprine, and androgens have been linked with the occurrence of peliosis.[6] Patients with AIDS may develop peliosis in association with bacillary angiomatosis and parenchymal bacillary peliosis. This is due to secondary infection with Bartonella henselae and a similar organism, Rochalimaea henselae.[7] Other infective agents such as hepatitis B and C,[8] Staphylococcus aureus, and tuberculosis are also thought to be associated with peliosis.[2],[6] The development of peliosis is associated with both monoclonal gammopathies, for example, multiple myeloma and Waldenstrom’s macroglobulinemia, and malignancies such as Hodgkin’s disease, hepatoma, and seminoma.[2] Additionally, the condition has also been described in patients with diabetes mellitus, end-stage renal disease, and renal transplantation.[9]

Peliosis is a clinically silent condition until it ruptures, after which it manifests as an acute abdomen and life-threatening hemorrhage as in the case of our patient. The clinical significance of this entity is as follows: (a) propensity to rupture spontaneously or with trivial trauma; (b) identification of underlying cause; and (c) resolution, although rare, after discontinuation of culprit medication/condition. Diagnosis of peliosis is usually established by a histopathologic examination of the resected organ or at autopsy. For splenic peliosis, the parafollicular areas were the most common sites of the lesion histologically, and this feature seemed to be important for the histologic differentiation of peliosis from simple dilatation of splenic sinuses resulting from passive congestion.[10]

Ultrasound features of peliosis of the spleen are nonspecific; however, on CT scans, it manifests as multiple small, well-defined hypoattenuating, cyst-like lesions that demonstrate significant enhancement with loss of definition of the lobules and septa and do not exert mass effect on the splenic vessels coursing through them.[11],[12] The nodules may show fluid–fluid levels, which are thought to reflect a hematocrit effect that shows enhancement in their dependent portions.[10] Calcification or extracapsular extension is not seen. However, if lesions rupture, subcapsular hematoma, laceration, and intraperitoneal hemorrhage may be evident on CT as in the case of our patient. This is of importance in recognizing the risk of spontaneous splenic rupture and in avoiding interventional procedures, such as liver biopsy or percutaneous transhepatic cholangiography, which may precipitate iatrogenic life-threatening organ rupture.

Because the condition remains asymptomatic, diagnosis of peliosis is usually difficult. In asymptomatic patients, it is identified only on autopsy or as an incidental finding on imaging done for other purposes. Review of literature revealed only 20 cases of spontaneous rupture of the spleen till date, and we report the twenty-first case. Patients came to medical attention only when the diagnosis was complicated by rupture, and in all patients, presentation was either by abdominal pain or circulatory shock, as shown in [Table 1]. Patients may not remember about the trivial trauma causing rupture of the spleen and might present to the emergency room with abdominal pain such as our patient. Because of low index of suspicion for the diagnosis, majority of the patients underwent contrast-enhanced CT before surgical intervention.
Table 1: Summary of previously published studies (1980–2016)

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Once diagnosis of peliosis is established, one must try to look for the underlying cause, which could be infections, drug-induced, immunodeficiency, hematological disorder, or any malignancy. Review of literature showed that 7 out of 21 reported patients were associated with either a hematological disorder or malignancy,[8] with one patient on long-term erythropoietin and renal replacement therapy[18] and another one on danazol for idiopathic thrombocytopenic purpura (ITP).[15] Once diagnosed with rupture, surgery is the only life-saving measure. The outcome depends on the clinical condition of the patient at presentation and the underlying cause. Except two, all of the patients in the reported cases were alive after splenectomy. The causes of mortality in these two patients were disseminated intravascular coagulation and adult respiratory distress syndrome in a patient after a viperine bite, and he died before any surgical intervention;[1] and multiorgan failure in an elderly patient with ITP who was on danazol therapy.[15]

Splenic rupture requires urgent surgical intervention; however, there are no guidelines describing surgical management in incidental peliosis of the spleen. In high-risk patients such as those on anabolic steroids, malignancies, and hematological disorders, screening ultrasound examination at 6–12-month interval might help establish an earlier diagnosis of peliosis in organs such as the liver and the spleen. Clinicians should have high index of suspicion in patients with preexisting risk factors and advise patients receiving anabolic steroids, growth factors, and erythropoietin to avoid high-impact physical activity and contact sports. The diagnosis of splenic rupture should be considered in patients who develop abdominal pain in this context. Early recognition and withdrawal of the offending agent may reverse the complication. The role of prophylactic splenectomy in patients with preexisting peliosis is not established; however, in patients with high risk for rupture, laparoscopic splenectomy could be offered to avoid future risk of life-threatening hemorrhage.


  Conclusions Top


Splenic rupture with associated peliosis is an extremely rare condition, which can lead to life-threatening hemorrhage. High index of suspicion is required for timely diagnosis and management. Review of available literature suggests that avoidance of high-impact physical activity, withdrawal of offending agent, and laparoscopic splenectomy may help avoid the mortality associated with this condition.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.[28]

 
  References Top

1.
Lal A, Singhal M, Sharma N, Bhalla A, Khandelwal N. Isolated splenic peliosis with spontaneous rupture after a viperine bite. Am J Emerg Med 2014;32:194.e3-4.  Back to cited text no. 1
    
2.
Celebrezze JP Jr, Cottrell DJ, Williams GB. Spontaneous splenic rupture due to isolated splenic peliosis. South Med J 1998;91:763-4.  Back to cited text no. 2
    
3.
Wagner E. Ein fall von Blutcysten in der Leber. Arc Heilkunde 1861;2:369-70.  Back to cited text no. 3
    
4.
Joseph F, Younis N, Haydon G, Adams DH, Wynne S, Gillet MB et al. Peliosis of the spleen with massive recurrent haemorrhagic ascites, despite splenectomy, and associated with elevated levels of vascular endothelial growth factor. Eur J Gastroenterol Hepatol 2004;16:1401-6.  Back to cited text no. 4
    
5.
Lacson A, Berman LD, Neiman RS. Peliosis of the spleen. Am J Clin Pathol 1979;71:586-90.  Back to cited text no. 5
    
6.
Gábor S, Back F, Csiffáry D. Peliosis lienis: Uncommon cause of rupture of the spleen. Pathol Res Pract 1992;188:380-2.  Back to cited text no. 6
    
7.
Slater LN, Welch DF, Min KW. Rochalimaea henselae causes bacillary angiomatosis and peliosis hepatis. Arch Intern Med 1992;152:602-6.  Back to cited text no. 7
    
8.
Giri N, Pitel PA, Green D, Alter BP. Splenic peliosis and rupture in patients with dyskeratosis congenita on androgens and granulocyte colony-stimulating factor. Br J Hematol 2007;138:815-7.  Back to cited text no. 8
    
9.
Fidelman N, LaBerge JM, Kerlan RK Jr. SCVIR 2002 film panel case 4: Massive intraperitoneal hemorrhage caused by peliosis hepatis. J Vasc Interv Radiol 2002;13:542-5.  Back to cited text no. 9
    
10.
Tada T, Wakabayashi T, Kishimoto H. Peliosis of the spleen. Am J Clin Pathol 1983;79:708-13.  Back to cited text no. 10
    
11.
Lashbrook DJ, James RW, Phillips AJ, Holbrook AG, Agombar AC. Splenic peliosis with spontaneous splenic rupture: Report of two cases. BMC Surg 2006;6:9.  Back to cited text no. 11
    
12.
Shimono T, Yamaoka T, Nishimura K, Naya M, Hojo M, Yamamoto E et al. Peliosis of the spleen: Splenic rupture with intraperitoneal hemorrhage. Abdom Imaging 1998;23:201-2.  Back to cited text no. 12
    
13.
Diebold J, Audouin J. Peliosis of the spleen. Report of a case associated with chronic myelomonocytic leukemia, presenting with spontaneous splenic rupture. Am J Surg Pathol 1983;7:197-204.  Back to cited text no. 13
    
14.
Etzion Y, Benharroch D, Saidel M, Riesenberg K, Gilad J, Schlaeffer F. Atraumatic rupture of the spleen associated with hemophagocytic syndrome and isolated splenic peliosis. Case report. APMIS 2005;113:555-7.  Back to cited text no. 14
    
15.
Javier Peñalver F, Somolinos N, Villanueva C, Sánchez J, Monteagudo D, Gallego R. Splenic peliosis with spontaneous splenic rupture in a patient with immune thrombocytopenia treated with danazol. Haematologica 1998;83:666-7.  Back to cited text no. 15
    
16.
Choudhuri S, Cavet J. Spontaneous splenic rupture due to isolated splenic peliosis in a case of multiple myeloma. Br J Hematol 2009;144:146.  Back to cited text no. 16
    
17.
Lo CM. A case of splenic peliosis associated with pathologic rupture. Hong Kong J Emerg Med 2007;14:174-8.  Back to cited text no. 17
    
18.
Hakoda S, Shinya H, Kiuchi S. Spontaneous splenic rupture caused by splenic peliosis of a hemodialysis patient with chronic renal failure receiving erythropoietin. Am J Emerg Med 2008;26:109.e1–2.  Back to cited text no. 18
    
19.
Adachi K, Ui M, Nojima H, Takada Y, Enatsu K. Isolated splenic peliosis presenting with giant splenomegaly and severe coagulopathy. Am J Surg 2011;202:e17-9.  Back to cited text no. 19
    
20.
Qureshi S, Choong AM, Tadrous PJ, Bhutiani RP. ‘Not just another appendicitis!’ − A case report of acute abdominal pain caused by splenic rupture secondary to isolated splenic peliosis. Ann R Coll Surg Engl 2009;91:W1-4.  Back to cited text no. 20
    
21.
Tsokos M, Püschel K. Isolated peliosis of the spleen: Report of 2 autopsy cases. Am J Forensic Med Pathol 2004;25:251-4.  Back to cited text no. 21
    
22.
Supe A, Desai C, Rao PP, Madiwale C, Joshi A. Isolated massive splenic peliosis. Indian J Gastroenterol 2000;19:87-8.  Back to cited text no. 22
[PUBMED]    
23.
Ortega Deballon P, Ferńandez Lobato R, Ortega Muñoz P, Artuñedo Pé P, Ferńandez Luengas D, Moreno Azcoita M. Splenic peliosis: A cause of spontaneous splenic rupture. Surgery 1999;126:585-6.  Back to cited text no. 23
    
24.
Kohr RM, Haendiges M, Taube RR. Peliosis of the spleen: A rare cause of spontaneous splenic rupture with surgical implications. Am Surg 1993;59:197-9.  Back to cited text no. 24
    
25.
Hirose H, Ohishi A, Nakamura H, Sugiura H, Umezawa A, Hosada Y. Fatal splenic rupture in anabolic steroid-induced peliosis in a patient with myelodysplastic syndrome. Br J Haematol 1991;78:128-9.  Back to cited text no. 25
    
26.
Gábor Z, Back F, Csiffáry D. Two-stage splenic rupture leading to the diagnosis of hepatic peliosis. Orv Hetil 1990;131:1371-3.  Back to cited text no. 26
    
27.
Garcia RL, Khan MK, Berlin RB. Peliosis of the spleen with rupture. Hum Pathol 1982;13:177-9.  Back to cited text no. 27
    
28.
Parsons MA, Slater D, Platts M, Fox M. Splenic peliosis associated with rupture in a renal transplant patient. Postgrad Med J 1980;56:796-7.  Back to cited text no. 28
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]


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