Thrombotic thrombocytopenic purpura in a previously splenectomized patient

Late Appearance of Thrombotic Thrombocytopenic Purpura after Autoimmune Hemolytic Anemia and in the Course of Chronic Autoimmune Thrombocytopenic Purpura: Two Case Reports

“…The fact that TTP developed in patient 2 in the ab sence of the spleen may be of relevance concerning the debated role of the spleen in the pathogenesis of TTP [16,21,23,24], This case seems to exemplify the previously reported view that the spleen does not play a crucial role in the pathogenesis of TTP [10,16,23].…”

“…A variety of immunological disorders in cluding systemic lupus erythematosus [2,3], rheuma toid arthritis [4,5], polymyositis [6], Sjogren's syn drome [7] and Graves' disease [8] have been found to be associated with TTP. However, the association of TTP with autoimmune hematological conditions has rarely been reported (9)(10)(11). We describe here this un usual occurrence in 2 patients.…”

“…However, there are only a few re ports of patients with autoimmune hematologic disor ders who subsequently developed TTP. Only one in stance of AITP was reported preceding TTP by some months [10], and it should be stressed that the patient had also hypothyroidism. In another instance immune-mediated pancytopenia and TTP developed concurrently [11], One sister of a patient with TTP had AITP and another sister had systemic lupus erythem atosus [14], Whether the TTP syndrome in those pa tients, as in the patients described here, was immunemediated or occurred independently is a matter of speculation; what is interesting is the fact that the two relatively unusual syndromes AIHA and TTP and AITP and TTP occurred in the same person.…”

Warm-Antibody Autoimmune Hemolytic Anemia Developing after Thrombotic Thrombocytopenic Purpura

“…There are very few cases described in the literature with TTP associated with autoimmune hematological conditions, most of them involving ITP [14, 15, 16, 17]. To our knowledge, there have been only two cases reporting the association between TTP and AIHA.…”

Renal Involvement in Patients with Thrombotic Thrombocytopenic Purpura

“…In their review of 271 cases ofTTP in the literature, involvement of the kidney manifested by significant proteinuria, hema turia, pyuria, and casts, or by azotemia, was demonstrable in 88% of the reported cases in which these data were recorded. Despite these observations, several articles that appeared in the literature thereafter did not consider kid ney involvement an important feature of the disease, although the article by Amorosi and Ultmatin [24] was quoted in their references, and despite the fact that the cases reported had abnormal urinalysis, azotemia, and morphologic evidence of renal involvement [25][26][27][28][29], It is to be expected that the kidney, as the most vascu lar organ in the body, should be involved in a disease process the major pathologic feature of which was recog nized from the outset as widespread microvascular thrombi. In fact, in the initial case report by Moschcowitz [1], the urine was noted to have shown 'marked traces of albumin with hyaline and granular casts', the kidneys were reported to have 'marked parenchymatous degener ation in the tubules and congestion of the parenchyma', and 'many of the arterioles and capillaries in the middle zone, in the region of the vasa recta, showed hyaline thrombi of the same morphology as those in the heart'.…”