Biomaterial Database

Autoimmune thrombocytopenia in primary antiphospholipid syndrome and systemic lupus erythematosus: the response to splenectomy. 1998

A J Hakim, and S J Machin, and D A Isenberg

Centre for Rheumatology, Department of Medicine, University College London, United Kingdom.

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

UI	MeSH Term	Description	Entries
D008180	Lupus Erythematosus, Systemic	A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.	Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D013156	Splenectomy	Surgical procedure involving either partial or entire removal of the spleen.	Splenectomies
D016208	Databases, Factual	Extensive collections, reputedly complete, of facts and data garnered from material of a specialized subject area and made available for analysis and application. The collection can be automated by various contemporary methods for retrieval. The concept should be differentiated from DATABASES, BIBLIOGRAPHIC which is restricted to collections of bibliographic references.	Databanks, Factual,Data Banks, Factual,Data Bases, Factual,Data Bank, Factual,Data Base, Factual,Databank, Factual,Database, Factual,Factual Data Bank,Factual Data Banks,Factual Data Base,Factual Data Bases,Factual Databank,Factual Databanks,Factual Database,Factual Databases
D016553	Purpura, Thrombocytopenic, Idiopathic	Thrombocytopenia occurring in the absence of toxic exposure or a disease associated with decreased platelets. It is mediated by immune mechanisms, in most cases IMMUNOGLOBULIN G autoantibodies which attach to platelets and subsequently undergo destruction by macrophages. The disease is seen in acute (affecting children) and chronic (adult) forms.	Autoimmune Thrombocytopenic Purpura,Idiopathic Thrombocytopenic Purpura,Purpura, Thrombocytopenic, Autoimmune,Werlhof's Disease,Autoimmune Thrombocytopenia,Immune Thrombocytopenia,Immune Thrombocytopenic Purpura,Thrombocytopenic Purpura, Autoimmune,Werlhof Disease,Autoimmune Thrombocytopenias,Autoimmune Thrombocytopenic Purpuras,Disease, Werlhof,Disease, Werlhof's,Idiopathic Thrombocytopenic Purpuras,Immune Thrombocytopenias,Immune Thrombocytopenic Purpuras,Purpura, Autoimmune Thrombocytopenic,Purpura, Idiopathic Thrombocytopenic,Purpura, Immune Thrombocytopenic,Purpuras, Autoimmune Thrombocytopenic,Purpuras, Idiopathic Thrombocytopenic,Purpuras, Immune Thrombocytopenic,Thrombocytopenia, Autoimmune,Thrombocytopenia, Immune,Thrombocytopenias, Autoimmune,Thrombocytopenias, Immune,Thrombocytopenic Purpura, Idiopathic,Thrombocytopenic Purpura, Immune,Thrombocytopenic Purpuras, Idiopathic,Thrombocytopenic Purpuras, Immune,Werlhofs Disease
D016736	Antiphospholipid Syndrome	The presence of antibodies directed against phospholipids (ANTIBODIES, ANTIPHOSPHOLIPID). The condition is associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses. In pregnancy it can cause abortion. Of the phospholipids, the cardiolipins show markedly elevated levels of anticardiolipin antibodies (ANTIBODIES, ANTICARDIOLIPIN). Present also are high levels of lupus anticoagulant (LUPUS COAGULATION INHIBITOR).	Anti-Phospholipid Antibody Syndrome,Anti-Phospholipid Syndrome,Antiphospholipid Antibody Syndrome,Hughes Syndrome,Anti Phospholipid Antibody Syndrome,Anti Phospholipid Syndrome,Antibody Syndrome, Anti-Phospholipid,Antibody Syndrome, Antiphospholipid,Antiphospholipid Antibody Syndromes,Syndrome, Anti-Phospholipid,Syndrome, Anti-Phospholipid Antibody,Syndrome, Antiphospholipid,Syndrome, Antiphospholipid Antibody,Syndrome, Hughes