Arthur Kavanaugh, MD

Disclosures

May 25, 2001

Question

Please discuss the treatment options for thrombocytopenia associated with systemic lupus erythematosus (SLE), and the indications for splenectomy in these cases.

Ahmed El-Rafie, MBBCh

Response from Arthur Kavanaugh, MD

Thrombocytopenia associated with SLE can be a difficult clinical problem. I will assume that we are discussing the idiopathic thrombocytopenic purpura (ITP)-like thrombocytopenia related to SLE. However, other potential causes of thrombocytopenia seen in SLE patients include adverse effects of medications, hypersplenism (for reasons other than SLE), thrombotic thrombocytopenic purpura (TTP) related to SLE, pseudothrombocytopenia, and myelophthisic cancers.

The treatment of ITP-like thrombocytopenia in SLE is similar to that for the idiopathic condition itself. Most patients (certainly those with potentially life-threatening or severe thrombocytopenia) receive corticosteroids, often initially in high doses (eg, 1 mg/kg prednisone equivalent, in split doses). Patients with less extreme depression of platelet counts may receive a less aggressive intervention. Acutely, immunoglobulin G (IgG) in large intravenous (IV) doses (eg, 2 g/kg) has proved efficacious. Both corticosteroids and IV IgG may be undesirable as long-term treatments because of toxicity and cost concerns.

A number of other agents have been assessed for use in ITP and SLE thrombocytopenia. The androgenic hormones such as danazol have been used with some success, as has the sulfa drug dapsone. For patients who are Rhesus positive (Rh+), treatment with RhoGAM may offer an alternative to IV IgG.

Additional immunomodulatory agents have been used, including cyclophosphamide and azathioprine. Often, patients treated with such agents have active, severe SLE involvement in other organ systems, providing additional need for such aggressive therapy. But these drugs have also been used when thrombocytopenia is the only or predominant manifestation. Cyclosporine presents another alternative therapy that has been assessed more in ITP than SLE. Vinca alkaloids, although they have been used for ITP, are associated with frequent adverse effects and should therefore be reserved for patients failing other treatments.

The question of splenectomy is always difficult in SLE. There is some older literature suggesting that patients with thrombocytopenia in SLE respond less well to this procedure than patients with ITP itself. However, there are reports of individual patients with SLE who did indeed have good sustained responses. Therefore, it should be considered as a possible intervention in refractory patients who have failed other treatments.

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