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Table of Contents
LETTER TO EDITOR
Year : 2018  |  Volume : 34  |  Issue : 2  |  Page : 263-264

Anesthetic management of laparoscopic splenectomy in a case of Evans Syndrome with systemic lupus erythematosis


1 Department of Anesthesiology and Intensive care, Nizam's Institute of Medical Sciences, Panjagutta, Hyderabad, Telangana, India
2 Department of Surgical gastroenterology, Nizam's Institute of Medical Sciences, Panjagutta, Hyderabad, Telangana, India

Date of Web Publication16-Jul-2018

Correspondence Address:
Nirmala Jonnavithula
Departments of Anesthesiology and Intensive care, Nizam's Institute of Medical Sciences, Panjagutta, Hyderabad - 500 082, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9185.173374

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How to cite this article:
Jonnavithula N, Pasupuleti SL, Thumma V, Ramachandran G. Anesthetic management of laparoscopic splenectomy in a case of Evans Syndrome with systemic lupus erythematosis. J Anaesthesiol Clin Pharmacol 2018;34:263-4

How to cite this URL:
Jonnavithula N, Pasupuleti SL, Thumma V, Ramachandran G. Anesthetic management of laparoscopic splenectomy in a case of Evans Syndrome with systemic lupus erythematosis. J Anaesthesiol Clin Pharmacol [serial online] 2018 [cited 2019 Nov 22];34:263-4. Available from: http://www.joacp.org/text.asp?2018/34/2/263/173374



Madam,

Evans Syndrome is a rare autoimmune disorder and by definition is the combination of both concurrent and sequential development of idiopathic thrombocytopenic purpura (ITP) and Coombs positive autoimmune hemolytic anemia (AIHA) in the absence of underlying disorder.[1] Although the true incidence is not known, it is estimated to effect 3.7-5% of patients with ITP or AIAH at the onset.[2] The dysregulation of the immune system produces several autoantibodies against red blood cells and platelets leading to early destruction by complement and reticuloendothelial systems.[3] The spleen has been proposed as either as a site of destruction or as a source of autoantibodies production.[4] The presence of associated systemic lupus erythematosis (SLE), warrants a more aggressive line of management than either condition presenting alone with poorer prognosis. We present case of Evans syndrome with ongoing bleeding diathesis, anticipated anesthetic risks and challenges scheduled for elective laparoscopic splenectomy in the presence of depleted platelet count.

A 26-year-old female patient presented with bleeding gums, menorrhagia and petechiae for the last 1-month, and 3 years back she had similar complaints along with jaundice. She was diagnosed to have “steroid-resistant Evans syndrome” associated with SLE. She had remission with two doses of rituximab. The present medication consisted of oral prednisolone 30 mg, hydroxychloroquine 200 mg, injection insulin and calcium supplements. General examination revealed petechiae over trunk and limbs. Her investigations are shown in [Table 1]. Electrocardiogram, two-dimensional echocardiogram and chest X-ray was normal. Ultrasonography of the abdomen revealed mild splenomegaly and distended gallbladder. She has a short neck with Mallampati Grade II. Vital parameters were normal except mild tachycardia. She was vaccinated to for pneumococcal, meningococcal and haemophilus infections. Premedication consisted of oral pantoprazole; alprazolam and rest of the medications were continued except insulin.
Table 1: Laboratory investigations

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On the day of surgery baseline, blood glucose was 65 mg/dL and corrected to 156 mg/dL with 25% dextrose. A large bore 16G intravenous (IV) cannula was secured; 1 unit single donor platelets (SDP) and hydrocortisone 25 mg IV was given prior to the surgery. Anesthesia was induced, and oral endotracheal tube was placed with minimal manipulation. Crepe bandages were applied to the lower limbs. Intraoperative analgesia was with IV fentanyl 80 μg bolus followed by 20 μg slow IV as required. Another unit of SDP and 3 units of platelet rich plasma and 2 units of packed red cells were given to counter the blood loss of approximately 800 mL. The procedure lasted for 5 ½ h with stable hemodynamics, and the urine output was 400 mL. Postoperative course was uneventful; IV hydrocortisone 25 mg was repeated every 6th hourly for 24 h. Platelet count was 70,000 mm 3, and there was no bleeding. She was discharged home on 10th postoperative day. Follow-up after 1-month was unremarkable.

Anesthetic management includes a thorough preoperative assessment, perioperative continuation of steroid/immunosuppression and an additional dose of steroid for possible suppression of hypothalamo-pituitary adrenal axis and evaluation of systemic organ involvement. Airway assessment may reveal unanticipated difficult airway, subglottic/laryngeal edema. Avoid trauma and bleeding during airway management. Strict asepsis must be maintained as these patients are at intrinsic susceptibility and also immunosuppressants.[5] A careful preanesthetic evaluation, a definitive anesthetic strategy with special considerations to risk of airway bleed, thrombocytopenia, systemic organ involvement and perioperative steroid replacement is mandatory.



 
  References Top

1.
Evans RS, Takahashi K, Duane RT, Payne R, Liu C. Primary thrombocytopenic purpura and acquired hemolytic anemia; evidence for a common etiology. AMA Arch Intern Med 1951;87:48-65.  Back to cited text no. 1
    
2.
Norton A, Roberts I. Management of Evans syndrome. Br J Haematol 2006;132:125-37.  Back to cited text no. 2
    
3.
Wang W, Herrod H, Pui CH, Presbury G, Wilimas J. Immunoregulatory abnormalities in Evans syndrome. Am J Hematol 1983;15:381-90.  Back to cited text no. 3
    
4.
Savasan S, Warrier I, Ravindranath Y. The spectrum of Evans' syndrome. Arch Dis Child 1997;77:245-8.  Back to cited text no. 4
    
5.
Kang I, Park SH. Infectious complications in SLE after immunosuppressive therapies. Curr Opin Rheumatol 2003;15:528-34.  Back to cited text no. 5
    



 
 
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