Autoimmune thrombocytopenia in primary antiphospholipid syndrome and systemic lupus erythematosus: the response to splenectomy

Abstract

Objective: To study the outcome of splenectomy in the management of thrombocytopenia.

Methods: Cases of systemic lupus erythematosus (SLE) or primary antiphospholipid syndrome (PAPS) complicated by severe thrombocytopenia were identified from a database of patients attending outpatients over the period 1978 to 1996. Clinical presentation, laboratory investigations, and response to medical treatment and/or splenectomy were documented.

Results: A total of 17 patients had severe thrombocytopenia; splenectomy was performed on 13: 9 with SLE, 3 with PAPS, and 1 with lupus-like disease (LLD). After splenectomy, six of nine patients with SLE and all three patients with PAPS gained complete remission of thrombocytopenia. There were no complications from splenectomy. The remaining four patients, three with SLE and one with PAPS, remained in a stable partial or full remission with oral medication and did not require splenectomy.

Conclusion: This study re-emphasizes the place for splenectomy in SLE patients and supports its role in the management of thrombocytopenia in PAPS.