Thrombotic Thrombocytopenic Purpura as an Initial Presentation of Primary Sjo¨gren’s Syndrome

Abstract

A healthy woman presented with ecchymoses due to thrombocytopenia, with numerous bone marrow megakaryocytes, microangiopathic haemolytic anaemia, disorientation, irritability, and normal renal function. Thrombotic thrombocytopenic purpura (TTP) was diagnosed and treated successfully by plasma exchange therapy, both on presentation and during a further three relapses. The TTP was considered idiopathic until, 4 months later, definite primary Sjogren's syndrome (1 degree SS) was diagnosed following the appearance of sicca symptoms. Only four similar cases have been cited in the literature. TTP should be added to the varied haematological manifestations that may occur in patients with 1 degree SS. The possible presentation of 1 degree SS not with classic sicca symptoms but rather with haematological abnormalities, including TTP, should be recognised.

A Fulminant Case of Renal Vein Thrombosis in a Patient with Autoimmune Disorder and Membranous Nephropathy

“…To the best of our knowledge, the present case is the first report in the English language literature in which renal necrosis developed due to rapid onset of RVT in a patient with an autoimmune disorder and membranous nephropathy, which finally required nephrectomy. (7). In the present case there were no aggressive findings suggestive of TMA-like fragmented red blood cells,…”

Thrombotic thrombocytopenic purpura complicating Sj�gren?s syndrome with crescentic glomerulonephritis and membranous nephritis

“…A review of the literature revealed six other such cases (Table 1). 8,[25][26][27] Three (patients 1, 5, 7 in Table 1) of the total seven cases of TTP complicating SS (including ours) had cellular infiltration or immunological findings in the glomeruli. All three patients had a rapidly fatal course despite plasma exchange.…”

Acquired Thrombotic Thrombocytopenic Purpura in a Patient with Pernicious Anemia

“…Multiple case reports have described the development of TTP in the background of SLE [9–11, 20, 21]. Similarly, there are cases of cooccurrence of Sjögren's syndrome and TTP [12, 22–24]. One particular study investigated the prevalence of concurrent autoimmune disorders in 76 patients with TTP [13].…”