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E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 5  |  Page : 409
Thrombosis warning in children suffering from Henoch-Schonlein Purpura


1 Department of Dermatology and Venereology, First Affiliated Hospital, Dalian Medical University, Dalian, China
2 Department of Radiology, First Affiliated Hospital, Dalian Medical University, Dalian, China
3 Department of Dermatology and Venereology, Second Affiliated Hospital, Dalian Medical University, Dalian, China
4 Department of Pediatrics, First Affiliated Hospital, Dalian Medical University, China

Date of Web Publication30-Aug-2013

Correspondence Address:
Hong Ji
Department of Pediatrics, First Affiliated Hospital, Dalian Medical University, 222th Zhongshan Road, Dalian
China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.117349

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   Abstract 

Henoch-Schonlein Purpura (HSP) is one of the most common types of vasculitis in children. The characteristic clinical manifestations include non-thrombocytopenic purpura, arthritis or arthralgia, abdominal pain, gastrointestinal hemorrhage and renal abnormalities. Thrombosis has been reported as, a rare complication of HSP. We present the case of a 14-year-old boy who was diagnosed with HSP and suspected superior mesenteric vein thrombosis. We reviewed the relevant literature and found eight similar reported cases. HSP is associated with thrombosis and HSP itself and some risk factors may result in thrombosis. We suggest that physicians should monitor patients with HSP who are at a higher risk of developing thrombosis more closely.


Keywords: Henoch-Schonlein purpura, risk factors, thrombosis


How to cite this article:
Li L, Zhang J, Zhang Y, Ji H. Thrombosis warning in children suffering from Henoch-Schonlein Purpura. Indian J Dermatol 2013;58:409

How to cite this URL:
Li L, Zhang J, Zhang Y, Ji H. Thrombosis warning in children suffering from Henoch-Schonlein Purpura. Indian J Dermatol [serial online] 2013 [cited 2019 Feb 17];58:409. Available from: http://www.e-ijd.org/text.asp?2013/58/5/409/117349

What was known?
Henoch Schonlein Purpura is one of the most common types of vasculitis in children.



   Introduction Top


Henoch-Schonlein Purpura (HSP) is one of the most common types of vasculitis in children. It is a multisystem disorder that involves various joints and organs, and results from a leukocytoclastic vasculitis of small vessels. The characteristic clinical manifestations include non-thrombocytopenic purpura, arthritis or arthralgia, abdominal pain, gastrointestinal hemorrhage and renal abnormalities. Although patients with HSP are in a pro-thrombotic state, thrombosis is a rare complication, with only eight previously reported cases of HSP-associated thrombosis in the literature. However, thrombosis is a potentially life-threatening condition, and clinicians should have a high index of suspicion for high-risk patients. Here, we present a single case of HSP accompanied by a superior mesenteric vein (SMV) thrombosis.


   Case Report Top


A 14-year-old boy with no significant past medical or family history was admitted to our hospital complaining of abdominal pain for the previous 14 days. He had passed dark red and black stool for 7 days, and had an erythematous rash over the anterior aspect of the lower legs for 2 days with a day of bilateral ankle arthralgia. On general examination, his vital signs were stable and he had a palpable purpuric rash that predominantly affected the anterior aspect of his lower legs [Figure 1]; the bilateral ankles were swollen. Abdominal examination revealed generalized tenderness that was more obvious in the supraumbilical region. Per rectal examination revealed dark red stool. The fecal occult blood test was positive.
Figure 1: Palpable purpuric rash on the legs

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The laboratory results were as follows: hemoglobin level: 14.5 g/dL; leucocyte count: 25.27 × 10 9 /L with 81% polymorphonuclear leukocytes; lymphocyte count: 13%; platelet count: 492/mm 3 . Serum electrolytes, blood urea nitrogen and creatinine, liver function tests, urine analysis, erythrocyte sedimentation rate, antinuclear antibody and anticardiolipin antibody levels and the coagulation profile were all within their normal ranges. An abdominal computed tomography (CT) scan showed thickening of the proximal small intestinal wall with a peritoneal effusion. The mesenteric CT venography was reported as a suspected SMV thrombosis with edema of the proximal intestinal wall [Figure 2].
Figure 2: Mesenteric CT venography revealed heterogeneous contrast within the superior mesenteric vein with a suspected filling defect within the lumen (arrow). A thickened and blurred intestinal wall (arrowheads) as a result of edema was observed within the affected segments

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According to the clinical manifestations, laboratory results and radiological findings, the patient was diagnosed with HSP and suspected SMV thrombosis. He was treated with mezlocillin sodium, methylprednisolone and heparin. After 7 days, his abdominal pain resolved and a repeated abdominal CT scan revealed that the intestinal wall edema and peritoneal accumulation had improved. On the 10 th day of hospitalization, follow-up CT venography revealed homogeneous contrast throughout the SMV and no signs of venous thrombosis [Figure 3]. The pat ient had no abdominal pain, no new rash and no swollen joints. He was discharged from hospital on the 12 th day of admission. During the 2-month follow-up period, he had no new complaints.
Figure 3: Repeat CT showed that the intestinal wall edema and venous thrombosis had completely resolved. Homogenous contrast was observed within the superior mesenteric vein (arrow), and the intestinal wall was even and well - demarcated (arrowheads)

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   Discussion Top


Venous and arterial thromboses are rare but potentially life-threatening complications in patients with HSP. We conducted a review of the published literature and found eight other patients [1],[2],[3],[4],[5],[6],[7],[8] with HSP-associated thrombosis [Table 1]. Most patients were treated with anti-inflammatories and anticoagulants. The outcomes were good and most patients had no episode of relapse.
Table 1: Reported cases of HSP associated with thrombosis

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Hyperactivation of the coagulation system is a common phenomenon along with the impaired activation of the fibrinolytic system in HSP. [9] The two physiological systems could combine in leading to thrombosis. After prompt treatment with anti-inflammatories and anticoagulation, the symptoms and signs of HSP improved in our case. So the follow-up CT venography showed no signs of thrombosis in the SMV. The seventh reported case [7] showed that a high level of plasma factor VIII and homocysteine could be a risk factor for thrombosis. [10] Higher levels of homocysteine, which could be induced by endothelial injury associated with vasculitis, can cause hypercoagulability in patients with HSP. Furthermore, levels of von Willebrand factor (vWf) antigen, a marker of endothelial injury, are also elevated during the acute stage of HSP. Factor VIII, homocysteine and vWf antigen could be important risk markers that indicate patients at a higher risk of thrombosis.

Two other reported cases [2],[6] that showed HSP was associated with antiphospholipid syndrome (APA), which can present with thrombosis. APA has been reported as a cause of HSP. [6] Antiphospholipid antibodies can produce a procoagulant state by affecting the function of phospholipid binding proteins that are involved in the coagulation cascade. [11] Endothelial cells are also damaged in HSP. The combined effect of HSP and APA would cause a greater risk of arterial or venous thrombosis. In one further case [5] HSP-associated thrombosis occurred in the context of Behçet's disease, another form of vasculitis. It is likely that endothelial dysfunction combined with a hypercoagulable state could contribute to thrombosis.


   Conclusion Top


Thrombosis is comparatively rare in HSP patients, but physicians should pay close attention to patients at a higher risk of developing thrombosis as a consequence of its potentially life-threatening outcomes.

 
   References Top

1.Diana A, Gaze H, Laubscher B, De Meuron G, Tschantz P. A case of pediatric Henoch-Schonlein purpura and thrombosis of spermatic veins. J Pediatr Surg 2000;35:1843.  Back to cited text no. 1
[PUBMED]    
2.Monastiri K, Selmi H, Tabarki B, Yacoub M, Mahjoub T, Essoussi AS. Primary antiphospholipid syndrome presenting as complicated Henoch-Schönlein purpura. Arch Dis Child 2002;86:132-3.  Back to cited text no. 2
[PUBMED]    
3.Lind J, Mackay A, Withers SJ. Henoch-Schönlein purpura and priapism. J Paediatr Child Health 2002;38:526-7.  Back to cited text no. 3
[PUBMED]    
4.Sari I, Akar S, Secil M, Birlik M, Kefi A, Onen F, et al. Thrombosis and priapism in a patient with Henoch-Schonlein purpura. Rheumatol Int 2005;25:472-4.  Back to cited text no. 4
[PUBMED]    
5.Park YW, Park JJ, Lee JB, Lee SS. Development of Henoch-Schönlein purpura in a patient with Behçet's disease presenting with recurrent deep vein thrombosis. Clin Exp Rheumatol 2007;25:S96-8.  Back to cited text no. 5
[PUBMED]    
6.Abend NS, Licht DJ, Spencer CH. Lupus anticoagulant and thrombosis following Henoch-Schonlein purpura. Pediatr Neurol 2007;36:345-7.  Back to cited text no. 6
[PUBMED]    
7.Topaloglu R, Bayrakci US, Cil B, Orhon D, Bakkaloglu A. Henoch-Schonlein purpura with high factor VIII levels and deep venous thrombosis: An association or coincidenc? Rheumatol Int 2008;28:935-7.  Back to cited text no. 7
[PUBMED]    
8.Canpolat U, Yorgun H, Sahiner L, Kabakçi G. Myocardial infarction due to coronary thrombosis in a patient with Henoch-Schönlein Purpura. Herz 2012.  Back to cited text no. 8
    
9.Prandota J, Pankow-Prandota L, Kotecki L. Impaired activation of the fibrinolytic system in children with Henoch-Schönlein Purpura: Beneficial effect of hydrocortisone plus sigma-aminocaproic acidtherapy on disappearance rate of cutaneous vasculitis and fibrinolyis. Am J Ther 2001;8:11-9.  Back to cited text no. 9
[PUBMED]    
10.Kurekci AE, Gokce H, Akar N. Factor VIII levels in children with thrombosis. Pediatr Int 2003;45:159-62.  Back to cited text no. 10
[PUBMED]    
11.Ravelli A, Martini A. Antiphospholipid syndrome. Pediatr Clin North Am 2005;52:469-91.  Back to cited text no. 11
[PUBMED]    

What is new?
Thrombosis is a rare complication, with only eight previously reported cases of HSP.associated thrombosis.


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]

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