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 Table of Contents  
CASE REPORT
Year : 2017  |  Volume : 7  |  Issue : 1  |  Page : 27-29

Splenectomy for immune thrombocytopenia in systemic lupus erythromatosus in Nigeria


1 Department of Surgery, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria
2 Department of Haematology, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria
3 Department of Obstetrics and Gynaecology, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State, Nigeria

Date of Web Publication4-Apr-2018

Correspondence Address:
Dr. M M Dauda
Department of Surgery, Ahmadu Bello University and Ahmadu Bello University Teaching Hospital, Zaria, Kaduna State
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ais.ais_31_17

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  Abstract 


Systemic lupus erythromatosus (SLE), is an autoimmune disease characterized by autoreactive T cells and polyclonal activation of B cells. SLE is a rare disease in tropical Africa and its association with thrombocytopenia has not been reported in the English literature. This is a case of a 37-year-old female civil servant with SLE complicated by refractory thrombocytopenia and successfully managed with splenectomy. Splenectomy in the management of thrombocytopenia associated with SLE in Africans as in other regions of the world is indicated in cases refractory to other treatment options.

Keywords: Autoimmune disease Splenectomy, systemic lupus erythrocytosis, thrombocytopenia


How to cite this article:
Dauda M M, Abdulaziz H, Aminu S M, Mamman AI, Randawa A J. Splenectomy for immune thrombocytopenia in systemic lupus erythromatosus in Nigeria. Arch Int Surg 2017;7:27-9

How to cite this URL:
Dauda M M, Abdulaziz H, Aminu S M, Mamman AI, Randawa A J. Splenectomy for immune thrombocytopenia in systemic lupus erythromatosus in Nigeria. Arch Int Surg [serial online] 2017 [cited 2021 May 15];7:27-9. Available from: https://www.archintsurg.org/text.asp?2017/7/1/27/229179




  Introduction Top


Systemic lupus erythromatosus (SLE) is an autoimmune disease characterized by autoreactive T cells and polyclonal activation of B cells.[1] An estimated 25/10,000 black women aged 18–55 years are affected in the USA.[2] Thrombocytopenia occurs in 10–15% of the patients with SLE.[3] Therapeutic options for management of thrombocytopenia in SLE include corticosteroids, cytotoxic agents, immunoglobulins, and more recently monoclonal antibodies such as rituximab.[1]


  Case Report Top


S.R., a 37-year-old female civil servant noticed a macular, discoid lesion on the malar region of the face 13 years ago. These skin lesions gradually coalesce over the bridge of the nose. Initially these lesions were hyperpigmented but later became hypopigmented. The lesions were both painful and itchy. These symptoms become worse on exposure to sunlight and heat. These lesions gradually peeled off leaving a nonhemorrhagic raw pink area [Figure 1]a.
Figure 1: (a) Patient's face at presentation with multiple nonhemorrhagic macular lesions. (b) Patient's face 2 years post splenectomy

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Eight months after the commencement of these symptoms, she presented at the National Tuberculosis and Leprosy Training Center where she was diagnosed with SLE. She was placed on prednisolone 20 mg and chloroquine 300 mg daily. Over the following 3 months, there was no improvement, hence, she stopped the drugs. She then presented to our center where she was placed on prednisolone 20 mg daily and a topical steroid-based cream for twice daily application for 3 months. However, she defaulted from clinic visits.

Five years later she again presented with complaints of pain in the knee and ankle joints. She was managed with analgesics, and the dose of prednisolone was reduced to 10 mg daily. During the default, she attended a peripheral hospital where she was transfused with two units of blood for hemoptysis, menorrhagia, and polymenorrhea. Clinical examination showed moderate pallor and butterfly rash. There were no enlarged lymph nodes, no palpable organomegally, and cardiorespiratory systems were essentially normal. Laboratory investigations revealed a haematocrit of 0.22l/l, thrombocytopenia (platelet count 60 × 109/l), positive anti-nuclear antibodies (ANA), and lupus erythromatosus (LE) cells. However, she had normal direct antiglobulin test (DAT), indirect antiglobulin test (IAT), prothrombin time (PT), activated partial thromboplastin time (APTT), and bleeding time. Bone marrow aspirate showed increased megakaryocytopoiesis (functional left shift), and a bone marrow biopsy revealed predominant increase in the megakaryocytic series. HBsAg, HCV, and HIV serology were negative. A diagnosis of immune thrombocytopenia in SLE was made. She was transfused with two units of fresh blood and was placed on prednisolone and chloroquine. She was then referred under the care of a gynecologist.

Pelvic ultrasound scan showed normal pelvic organs and she was placed on lofeminal R; her menses became regular within 3 months. The lofeminal R was then stopped but menorrhagia recurred. She was then placed on Confidence pills R, but these were withdrawn due to severe headaches. She was replaced on lofeminal R but then had amenorrhea for 50 days. Thus, lofeminal R was withdrawn. Severe vaginal bleeding and headache ensued such that she had to be admitted. She was transfused 18 units of fresh whole blood over the next 30 days. At this time, a possible diagnosis of intracranial bleeding secondary to severe thrombocytopenia was made based on the findings of severe headache, photophobia, mild neck stiffness, retinal hemorrhage, petechial hemorrhage, and bleeding from the venepuncture site. The platelet count at this time was 13 × 109/l. Computed tomogram and lumbar puncture were not done due to the cost and severe thrombocytopenia, respectively. She was treated with intravenous immunoglobulin (IVIg), cyclosporine, methylprednisolone, danazol, antiD antibodies, and hematinics. She was discharged home after her symptoms abated.

Two weeks after the discharge, she developed severe bilateral lower limb pain and was referred to an orthopedic surgeon who placed her on clindamycin and physiotherapy. She was also given crutches to support her ambulation. Two months later, she complained of menorrhagia and dizziness. She was readmitted due to severe anemia, thrombocytopenia, and a clinical impression of osteomyelitis. Culture of the aspirate yielded Staphylococcus aureus. Other investigations revealed a hematocrit of 0.21l/l, platelet count of 25 × 109/l and WBC of 11.1 × 109/l. She continued to bleed per vaginum for 60 days during which she received 24 units of fresh whole blood. Her platelet count continued to decline to as low as 5 × 109/l. A decision to undertake splenectomy was entertained and the surgeons were invited.

Preoperatively, she had two units of fresh whole blood and one unit intraoperatively. The surgery was uneventful. Postoperatively, she had three units of fresh blood. She developed jaundice, with increased liver enzymes. She also bled from the edges of the surgical incision (primary hemorrhage) and developed urinary tract infection. She was managed appropriately and all postoperative problems resolved within 7 days. The response of the platelet count after surgery was dramatic and is depicted in [Table 1]. After achieving partial remission, her wound has sealed and she was discharged on oral penicillin and proguanil. She also received first dose of polyvalent pneumococcal vaccine at the community health immunization unit. She was followed-up irregularly for 4 years; she died from a trauma associated with a head injury.
Table 1: Pre and postoperative platelet counts

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  Discussion Top


The spleen has been implicated as the source of antiplatelet antibodies or the site for destruction of sensitized platelets.[4],[5] Although we considered platelet transfusion in the course of treating this patient, we did not have a functional apheresis machine at the time and the cumbersome nature of pooling multiple donors for a pint of platelets manually, made us drop the option of using platelet transfusion. However we were also aware that platelet transfusion should be avoided in autoimmune thrombocytopenia, heparin induced thrombocytopenia, thrombocytopenic purpura and in hemolytic uraemic syndrome.[6] At the time thrombomimetics like Romiplastin and eltrombopaq were still undergoing clinical trials.[6] The role of splenectomy in thrombocytopenia associated with SLE is still controversial; while some authors report excellent 80–100% partial or complete remission [7],[8],[9] without morbidity, some others have reported poor outcome.[10] The latter authors concluded that splenectomy does not cure thrombocytopenia in SLE patients. Hepburn and colleagues have reported that SLE can complicate auto-immune idiopathic thrombocytopenia.[11] We feel strongly that this is the situation in our patient, SLE, complicating idiopathic thrombocytopenia. SLE is a rare disease in tropical Africa and its association with thrombocytopenia has not been reported in the English literature. We successfully treated our patient with splenectomy in our centre. Splenectomy in the management of thrombocytopenia associated with SLE in Africans as in other regions of the world is indicated in cases refractory to other treatments. [Figure 1]b shows our patient 2 years post splenectomy.

We conclude that splenectomy can be safe in the management of patients with SLE complicated by refractory thrombocytopenia where nonsurgical approaches have failed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that name and initial will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Tanaka V, Tokunaga M. Rituximab reduce both quantity and quality of B cells in SLE. Rheumatology 2006;45:122-3.  Back to cited text no. 1
    
2.
Sabahi R, Anolik JH. B-cell–targeted therapy for Systemic lupus erythromatosus. Drugs 2006;66:1933-48.  Back to cited text no. 2
[PUBMED]    
3.
Chui A, Tam W, Cines D, Bussel JB. Immune thrombocytopenic purpura; pathophysiology in patients with persistent problems. Postgraduate Haematology. 5th ed. Blackwell: Oxford; 2005. pp 940-4.  Back to cited text no. 3
    
4.
You NY, Tefferi A, Nargomey DM. Outcome of splenectomy for thrombocytopenia associated with SLE. Ann Surg 2004;240:286-92.  Back to cited text no. 4
    
5.
Ruivia L, Gang L, Kai W, Yixin L, Qibing X, Yi L, et al. The role of Splenectomy in thrombocytopenia in SLE. Rheumatology 2011;31:9-15.  Back to cited text no. 5
    
6.
Hoffbrand AV, Paul AH. Bleeding disorders caused by vascular and platelet abnormalities. Hoffbrand's Essential Haematology. 7th ed. Chapter 25. John Wiley and Son's; 2016. pp 278-89.  Back to cited text no. 6
    
7.
Gruenberg JC, VanSlyks EJ, Abraham JP. Splenectomy in Systemic lupus erythromatosis. Am Surg 1986;52:366-70.  Back to cited text no. 7
    
8.
Campos LMA, Fiorot FJ, Silva CAA. Splenectomy for refractory thrombocytopenia in juvenile SLE. Paediatr Rheumatol 2008;6(Suppl. 1):248.  Back to cited text no. 8
    
9.
Stein RS, Flexner JM. Splenectomy for thrombocytopenia. South Med J 1984;77:1599-601.  Back to cited text no. 9
    
10.
Stephen H, John LM, Philip RG, Clement JM, Charles HM. Splenectomy does not cure the thrombocytopenia of SLE. Ann Intern Med 1985;102:325-8.  Back to cited text no. 10
    
11.
Hepburn MJ, English JC, Keeling JH. Auto-immune idiopathic thrombocytopenia with subsequent occurrence of SLE. Curtis 1997;60:185-7.  Back to cited text no. 11
    


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