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11/552. Use of cyclophosphamide in the treatment of thrombotic thrombocytopenic purpura complicating systemic lupus erythematosus: report of two cases.

    Thrombotic thrombocytopenic purpura (TTP) is an unusual complication of systemic lupus erythematosus (SLE). There are no randomized, prospective studies of its treatment. The association of plasma infusions and (or) plasmapheresis with steroids improves survival when compared with steroid treatment alone, but these patients still have a higher mortality than those with "classic" TTP. The role of immunosuppressive drugs in the management of this disorder remains uncertain. We report two cases of TTP in SLE which presumably benefited from the addition of cyclophosphamide to the treatment with plasmapheresis and steroids. ( info)

12/552. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) following treatment with deoxycoformycin in a patient with cutaneous T-cell lymphoma (sezary syndrome): A case report.

    We present a case of a patient who developed all manifestations of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) acutely following treatment of cutaneous T-cell lymphoma (CTCL, sezary syndrome) with deoxycoformycin (pentostatin). Symptoms and signs included severe thrombocytopenia and microangiopathic hemolytic anemia; hallucinations, confusion and disorientation; oliguric acute renal failure requiring hemodialysis; and fever. No other etiology for these symptoms and signs was present. Complete recovery followed treatment for one month with plasma exchange and glucocorticoids. During the succeeding 20 months she has remained well and her CTCL remains stable on no further treatment. This case and two previously published cases suggest that acute and severe TTP-HUS may be a dose-dependent toxicity of deoxycoformycin (pentostatin). ( info)

13/552. Successful treatment of resistant thrombotic thrombocytopenic purpura/hemolytic uremic syndrome with autologous peripheral blood stem and progenitor (CD34 ) cell transplantation.

    The first-line treatment of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS syndrome) induces a response and survival rate of approximately 85%, even if a considerable number of patients relapse; nevertheless, a number of these patients are resistant to conventional management. Immunoablation followed by stem cell transplantation has been shown to be capable of inducing remissions in a large spectrum of experimental autoimmune disorders. We report here the case of a 20-year-old male patient with the TTP-HUS syndrome who was resistant to conventional treatment and was transplanted with autologous immunoselected CD34 PBPC after conditioning with cyclosphosphamide, anti-T lymphocyte globulin and prednisone. Seven months after transplant the patient is alive and well, without any further treatment being given. ( info)

14/552. Thrombocytopenic thrombotic purpura: severe clinic with no CT, minor MRI, but a SPECT correlate.

    A 28-year-old woman with primarily therapy refractory TTP was followed neuroradiologically over 6 months. Despite pronounced neurological and neuropsychiatric symptoms including hemiparesis and aphasia she had unremarkable CT scans on two occasions. Three MRI exams showed no correlate for her neurological symptoms except a small petechial cortical hemorrhage in the right parietooccipital gyrus which may account for her TTP-related anxiety disorder. A cerebral HMPAO-SPECT showed long-standing right-sided hypoperfusion compatible with residual vasculature changes. The possible causes for the clinico-neuroradiological discrepancies are discussed in view of the literature. ( info)

15/552. Relapsing catastrophic antiphospholipid antibody syndrome: a mimic for thrombotic thrombocytopenic purpura?

    SUMMARY: The catastrophic antiphospholipid antibody syndrome (CAPS) is an uncommon disorder characterized by widespread micro- and macrovascular changes due to intravascular thrombosis. This complication of the antiphospholipid antibody syndrome is often fatal and recurrences are very rare. The differential diagnosis of CAPS includes thrombotic thrombocytopenic purpura (TTP) and this distinction may be difficult, but essential, for appropriate therapy. plasmapheresis is effective in both conditions, but anticoagulation, a mainstay in the treatment of CAPS, could be disastrous in TTP. We present the case of an elderly woman who survived two episodes of CAPS four years apart and whose clinical findings were also suggestive of TTP. The characteristics of TTP and CAPS are compared and the importance of accurate diagnosis is emphasized. ( info)

16/552. Treatment of thrombotic thrombocytopenic purpura with the cryosupernatant fraction of plasma: a case report and review of the literature.

    We report a case of thrombotic thrombocytopenic purpura (TTP) that did not respond to extensive plasma exchange with fresh frozen plasma (FFP) but responded to plasma exchange with cryosupernatant. Several reports indicate that responses to cryosupernatant may be seen in patients refractory to FFP, and this approach may be appropriate in these patients before one recommends a splenectomy. The literature on refractory TTP treated with cryosupernatant exchange is reviewed. ( info)

17/552. Thrombotic thrombocytopenic purpura associated with ticlopidine therapy.

    A patient who developed thrombotic microangiopathy while on ticlopidine therapy is reported. Thrombotic microangiopathy resolved with discontinuation of the drug and treatment with plasma exchange and has not recurred during 10 months of follow-up. The emerging data on the risk of developing thrombotic microangiopathy while on ticlopidine and the possible mechanisms underlying this association are reviewed. The need for careful monitoring of the platelet count and hematocrit in addition to the white cell count during the first 3 months of therapy with this drug is emphasized. It is important that nephrologists, who are frequently called upon to diagnose thrombotic microangiopathy, be aware of its association with ticlopidine. Other drug-induced syndromes of thrombotic microangiography are also considered and compared, with respect to possible mechanisms of disease in each case. ( info)

18/552. Renal involvement of thrombotic thrombocytopenic purpura: special reference to the glomeruloid structures.

    We report the case of a 9-year-old girl with biopsy-proven renal thrombotic microangiopathy in thrombotic thrombocytopenic purpura (TTP), with particular reference to the glomeruloid structures. The renal biopsy sample from this TTP patient revealed platelet thrombus deposition, a glomeruloid structure and aneurysm with relative sparing of the glomeruli. The glomeruloid structure displayed a proliferation of mainly capillary-sized channels lined by factor viii-related, antigen-positive plump endothelial cells embedded in the edematous connective tissue. These glomeruloid vessels communicated with the aneurysmal segment at the end portion of the arteriolar branch. We believe that the glomeruloid structures in TTP represent not merely organization or recanalization of thrombus but rather active angiogenesis through aneurysmal dilation in the arteriolized vessel, probably initiated by platelet agglutination. ( info)

19/552. Massive myocardial necrosis in thrombotic thrombocytopenic purpura: a case report and review of the literature.

    Thrombotic thrombocytopenic purpura (TTP) is an uncommon syndrome resulting from diffuse occlusion of small arterioles and capillaries by hyaline microthrombi. It is characterized by fever, thrombocytopenic purpura, microangiopathic hemolytic anemia, and neurologic and renal dysfunction. While cardiac pathology in TTP is commonly seen at autopsy, clinical cardiac dysfunction is rare and typically results from conduction system involvement. While 3% to 8% of patients with TTP report chest pain on admission, reports of fatal ventricular pump failure are extremely rare. We now report a case of TTP resulting in death from widespread myocardial necrosis. This patient presented with elevated cardiac enzymes and electrocardiographic disturbances that mimicked viral myocarditis, as well as a profound thrombocytopenia. Such a case may represent the extreme of a distribution of cardiac involvement in TTP or the consequence of an unidentified autoimmune process capable of precipitating severe myocardial TTP. ( info)

20/552. New strategies in diagnosis and treatment of thrombotic thrombocytopenic purpura: case report and review.

    The pentad of thrombocytopenia, haemolytic anaemia, mild renal dysfunction, neurological signs and fever, classically characterizes the syndrome of thrombotic thrombocytopenic purpura (TTP). TTP usually occurs in adults but also children have been described with this condition. The disorder may take a relapsing course, termed chronic relapsing TTP (CRTTP), which although very rare, may also begin in childhood. Deficiency of a recently identified enzyme, the von Willebrand factor (vWF)-cleaving protease, seems to play a major role in the development of TTP. We report on a 3-year-old boy with a dramatic but typical clinical course of CRTTP. At the time of diagnosis, neurological deficits and multiple cerebral infarctions had already occurred. In plasma, vWF-cleaving protease was completely absent, both during acute TTP and in remission. There was no protease inhibitor detected. Regular infusions of fresh frozen plasma were successfully given for replacement on a prophylactic basis. CONCLUSION: Assay of von willebrand factor-cleaving protease helps to diagnose a form of thrombotic thrombocytopenic purpura which may be managed by prophylactic treatment with fresh frozen plasma. ( info)
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