Volume 6 Supplement 1

15thPaediatric Rheumatology European Society (PreS) Congress

Open Access

Splenectomy for refractory thrombocytopenia in juvenile systemic lupus erythematosus

  • LMA Campos1,
  • FJ Fiorot1 and
  • CAA Silva1
Pediatric Rheumatology20086(Suppl 1):P248

DOI: 10.1186/1546-0096-6-S1-P248

Published: 15 September 2008

Background

The prevalence of autoimmune thrombocytopenia in juvenile systemic lupus erythematosus (JSLE) ranges from 7–52% (mean 14,5%) but profound rates (<50,000/mL) are uncommon. Although the exact mechanism is unknown, the spleen has been implicated either as the source of antiplatelet antibodies or the site of sensitized platelets destruction. Splenectomy is rarely indicated due to the increased risk of severe infections and controversial effectiveness.

Materials and methods

From 1983 to 2007, 5079 patients were followed at the Pediatric Rheumatology Unit and JSLE occurred in 228 (4.5%). We report three female JSLE patients with refractory thrombocytopenia to whom splenectomy was indicated.

Results

Data are described in table 1. Thrombocytopenia preceded JSLE diagnosis in a mean of 30 months. All patients had initial normal bone marrow and positive antiphospholipid antibodies.
Table 1

Clinical and laboratorial manifestations and treatment of refractory thrombocytopenia

Patient

SSR

ACS

MJS

Age (years)

16

12

12

Months between JSLE/splenectomy

1

38

10

Previous treatment

Pd, MP, CYC

Pd, MP, HCQ, IVG, AZA, C, RTX

Pd, MP, HCQ, IVG, AZA, C, MMF, RTX

Platelet count

3,000/mL

7,000/mL

10,000/mL

Splenectomy

Held

Held

Indicated (cancelled due to bone marrow hypoplasia-toxicity?)

Platelet count (time after surgery)

107,000/mL (2 w)

469,000/mL (2 m)

140,000/mL (1 d)

396,000/mL (3 d)

-

Recurrences

none

1 month after

-

Pd = prednisone; MP = Methylprednisolone pulse; CYC = Cyclophosphamide; HCQ = Hydroxychloroquine; IVG = Gammaglobulin; AZA = Azathioprine; C = Cyclosporine; MMF = mycophenolate mofetil; RTX = Rituximab

Conclusion

Splenectomy should be considered for the treatment of thrombocytopenia refractory cases. Results are variable. Bone marrow hypoplasia should be excluded.

Authors’ Affiliations

(1)
Instituto da Criança – Clinical Hospital, University of São Paulo

Copyright

© Campos et al; licensee BioMed Central Ltd. 2008

This article is published under license to BioMed Central Ltd.

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