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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 14  |  Issue : 2  |  Page : 172-174

Splenic peliosis and spontaneous splenic rupture: A rare complication following liver transplantation


Department of Hepatology, Anaesthesiology and Liver Transplantation, Apollo Hospitals, Navi Mumbai, Maharashtra, India

Date of Submission17-Dec-2019
Date of Acceptance17-May-2020
Date of Web Publication06-Jul-2020

Correspondence Address:
Dr. Amey Dilip Sonavane
C-1804; Azziano, Rustomjee Urbania, Majiwada, Thane West - 400 601, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijot.ijot_71_19

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  Abstract 


Peliosis is characterized by multiple blood-filled cyst-like cavities within the parenchyma of solid organs. It is a rare condition and mostly affects the liver. Splenic peliosis is even rarer, especially following solid organ transplantation with barely few cases reported in the literature. We describe a rare case of splenic peliosis complicated by spontaneous splenic rupture following deceased donor liver transplantation. Timely diagnosis and urgent re-exploration followed by splenectomy salvaged the patient.

Keywords: Liver transplantation, splenic peliosis, splenic rupture


How to cite this article:
Sonavane AD, Shah K, Raut V, Thorat A, Raj C A, Ambekar A, Parmar T, Sen S, Sawant A, Chaksota H, Vasanth S, Nagral A, Mirza D. Splenic peliosis and spontaneous splenic rupture: A rare complication following liver transplantation. Indian J Transplant 2020;14:172-4

How to cite this URL:
Sonavane AD, Shah K, Raut V, Thorat A, Raj C A, Ambekar A, Parmar T, Sen S, Sawant A, Chaksota H, Vasanth S, Nagral A, Mirza D. Splenic peliosis and spontaneous splenic rupture: A rare complication following liver transplantation. Indian J Transplant [serial online] 2020 [cited 2020 Aug 5];14:172-4. Available from: http://www.ijtonline.in/text.asp?2020/14/2/172/289053




  Introduction Top


Spontaneous splenic rupture is rare and occurs in a diseased spleen. Peliosis is a pathological condition predominantly affecting the liver and less commonly the spleen. It is characterized by multiple blood filled cavities within the parenchyma of solid organs. Peliosis has been associated with many conditions, including hematologic malignancies, chronic alcoholism, oral contraceptive use, acquired immuno deficiency syndrome and rarely post-transplant immunosuppressed state. An extremely infrequent sequelae of splenic peliosis is spontaneous splenic rupture. We describe a rare case splenic peliosis complicated by spontaneous splenic rupture after living donor liver transplantation.


  Case Report Top


A 43-year-old male underwent deceased donor liver transplantation in 2018 for cryptogenic decompensated chronic liver disease. The surgery was uneventful. Initial immunosuppression consisted of intravenous hydrocortisone and oral tacrolimus. Tacrolimus dose was gradually increased (to maintain a trough level between 7 and 10), and mycophenolate mofetil was added on postoperative day 4. On postoperative day 5, he had high drain output and prerenal acute kidney injury which was managed with reduction of tacrolimus dosage, intravenous albumin, and crystalloids. On postoperative day 13, he had sudden onset shortness of breath. He also complained of acute abdominal distension and pain in the left hypochondrium exacerbated by movement. On physical examination, he was apyrexial, exhibited tachycardia (115 beats/min) and hypotension (blood pressure 96/60 mmHg). Cardiorespiratory examination was unremarkable. Abdominal examination revealed tenderness in the left hypochondrium without features of peritonism.

He was shifted to the emergency care unit and volume resuscitated. An urgent triphasic computed tomography (CT) of the abdomen showed a large hematoma in the perisplenic region arising from the upper pole of the spleen with active extravasation of the intravascular contrast from the upper pole of the spleen into the perisplenic hematoma. The spleen showed few lacerations extending through the splenic parenchyma and few areas of focal hypoattenuation [Figure 1] and [Figure 2]. The hematoma extended along the left paracolic gutter into the pelvis with high-density ascites. The splenic and hepatic artery as well as the portal and splenic veins were normal in caliber without evidence of any aneurysm. He was taken to the operation theater for re-exploration immediately. He underwent splenectomy with evacuation of the intra-abdominal clot. The splenic hematoma weighed 1.28 kg. From within the peritoneal cavity, 2000 ml of free blood was evacuated.
Figure 1: Computed tomography scan of the abdomen reveals a large hematoma in the perisplenic region arising from the upper pole of the spleen with active extravasation of the contrast (green arrows). The spleen shows few lacerations extending through the splenic parenchyma.

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Figure 2: Computed tomography scan (coronal) of the abdomen showing few areas of hypoattenuation (red arrows) in the splenic parenchyma.

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Macroscopically, the spleen was ruptured at its upper pole with active bleeding [Figure 3]. The grafted liver appeared normal. Histopathological examination revealed areas of blood-filled lakes in the parafollicular region of the splenic red pulp. These blood-filled vascular spaces did not have any endothelial or epithelial lining. These findings were consistent with peliosis of the spleen [Figure 4]. The ruptured area of the spleen showed neutrophilic infiltration and congestion of the red pulp. The patient had spontaneous recovery post splenectomy. He was shifted back to the ward within 48 h and was discharged 5 days later. He was started on aspirin a week later in view of thrombocytosis. At 6-month follow-up, he is asymptomatic.
Figure 3: Gross appearance of the spleen. Splenic rupture seen at the upper pole.

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Figure 4: Histological examination of the spleen (original magnification, 4; H and E stain) shows a blood-filled lake (arrow) within the splenic parenchyma.

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  Discussion Top


The word “pelios” has its origin in Greek literature and implies to a purple or dusky macroscopic appearance of the organ. Microscopically, it is characterized by the presence of multiple blood-filled cyst-like lesions within solid organs. Isolated splenic peliosis (peliosis lienis) after solid organ transplantation is rare with few cases reported worldwide. Peliosis can affect any organ of the mononuclear phagocytic system (liver, spleen, lymph nodes, and bone marrow) as well as lungs, parathyroid glands, kidneys, stomach, and small intestine.[1]

In a systematic review of 613 cases of spontaneous splenic rupture, the most common etiologies associated with splenic rupture were infectious diseases (n = 143), colonoscopy procedure (n = 87), hematologic conditions (n = 84) and nonhematologic neoplasms (n = 48), amyloidosis (n = 24), internal trauma such as cough or vomiting (n = 17), and rheumatologic diseases (n = 10).[2] Infections such as bacillary angiomatosis (especially in immunodeficient states), Bartonella henselae, Rochalimaea henselae, Staphylococcus aureus, tuberculosis, and hepatitis B and C viruses are associated with splenic peliosis. Toxins such as ethanol and thiotrust and drugs such as oral contraceptives, azathioprine, tamoxifen, corticosteroids, and androgens have been associated. There is also a link between malignancies such as Hodgkin's disease, seminomas, and monoclonal gammopathies with peliosis. There appears to be a relation between dysregulation of the immune system and this condition. Peliosis of the spleen is diagnosed during radiological investigations, surgery, or during autopsy (in asymptomatic individuals). Macroscopically, the spleen may appear nodular with the cut surface demonstrating numerous cyst-like blood-filled cavities. On histopathology, the lesions are observed in the parafollicular area of the red pulp and appear like round-to-oval-shaped dilated sinuses filled with blood. Sometimes, the cysts are breached by arteries that project into the lumen, possibly explaining the sudden and disastrous bleeding.[3]

Spontaneous rupture or rupture after trivial trauma of the affected organ is the most feared complication. Antiplatelet agents and anticoagulants can precipitate such an event. The patient presents like an acute abdomen with life-threatening hemorrhage. The diagnosis can be established by imaging studies. An ultrasound examination reveals an echogenic mass with numerous poorly defined foci of varying hypoechogenecity. Hypodense cyst-like lesions in the spleen without mass effect are visualized on noncontrast CT images. On contrast-enhanced CT images, the lesions demonstrate substantial enhancement, and the splenic lobules and septae show an obscured appearance. Fluid–fluid levels are occasionally demonstrated. Once the lesions rupture, a capsular breach, subcapsular hematoma with associated intraperitoneal hemorrhage is visualized.[4]

Few case reports of splenic peliosis associated with splenic rupture post solid organ transplant have been reported in literature. The first case was reported in 1980, where a renal transplant recipient developed symptoms of splenic rupture. He had received testosterone injections for anemia along with immunosuppressive medications, and both these medications were thought to be the causative factor.[5] One patient developed spontaneous splenic rupture 4 months post liver transplantation, when he fell over ice in winter and had syncopal episodes thereafter. He complained of abdominal pain and became diaphoretic and dyspneic. A CT scan diagnosed the condition, and he underwent a life-saving emergent laparotomy.[6] Another case was reported in a recipient of simultaneous liver and renal transplant. He presented with hypovolemia on the 8th postoperative day, in a manner similar to our case. Diagnostic laparoscopy revealed splenic rupture following which he underwent an emergency splenectomy. Aggressive immunosuppression was thought to be the causative factor.[7]

Splenic rupture is a medical emergency and requires urgent surgical intervention. The diagnosis should be considered in certain high-risk patients presenting with acute abdominal pain. It may be worthwhile to perform screening ultrasound examinations at 6–12-month intervals in this high-risk patient group (those on anabolic steroids or patients with hematological and nonhematological malignancies). Prophylactic laparoscopic splenectomy may have an evolving role in the future when the condition is further characterized and specific risk factors delineated.

The exact cause of splenic peliosis and spontaneous rupture remains unknown in our case. A retrospective review of pretransplant CT scan of the abdomen did not reveal any obvious splenic pathology other than mild splenomegaly secondary to portal hypertension. It may be worthwhile to consider that the immunosuppressant medications used in the posttransplant period may have a role in the pathogenesis. However, the immunosuppressive therapy consisted of 100 mg hydrocortisone, followed by a direct taper to 20 mg of prednisolone from postoperative day 2 (as per institutional protocol). Furthermore, tacrolimus dose was slightly lowered as the patient had developed renal dysfunction just before the episode of spontaneous splenic rupture. A posttraumatic splenic hematoma secondary to surgical handling was also considered, however the macroscopic and histopathological picture was not consistent with a posttraumatic hematoma.

In conclusion, splenic peliosis and spontaneous splenic rupture should be considered as a differential diagnosis in post solid organ transplant patients presenting with acute abdominal pain and hypovolemia. Prompt diagnosis and emergency splenectomy can salvage the patient from this life-threatening complication. In addition, emergency care as well as attending physicians and surgeons should be aware about the diagnosis and management of peliosis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Tsokos M, Erbersdobler A. Pathology of peliosis. Forensic Sci Int 2005;149:25-33.  Back to cited text no. 1
    
2.
Aubrey-Bassler FK, Sowers N. 613 cases of splenic rupture without risk factors or previously diagnosed disease: A systematic review. BMC Emerg Med 2012;12:11.  Back to cited text no. 2
    
3.
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. 3
    
4.
Abbott RM, Levy AD, Aguilera NS, Gorospe L, Thompson WM. From the archives of the AFIP: Primary vascular neoplasms of the spleen: Radiologic-pathologic correlation. Radiographics 2004;24:1137-63.  Back to cited text no. 4
    
5.
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. 5
    
6.
Raghavan R, Alley S, Tawfik O, Webb P, Forster J, Uhl M. Splenic peliosis- A rare complication following liver transplantation. Digestive Dis Sci 1999;44:1128-31.  Back to cited text no. 6
    
7.
Börcek P, Özdemir BH, Yılmaz Akçay E, Haberal M. Splenic peliosis resulting in spontaneous splenic rupture in a concomitant hepatic and renal allograft recipient. Exp Clin Transplant 2016;14:114-5.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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