Myocardial infarction in a patient with thrombotic thrombocytopenic purpura

A 63-year-old male with a prior history of thrombotic thrombocytopenic purpura was admitted with sudden onset of syncope. He denied chest pain. His initial blood chemistries were consistent with acute relapse of thrombotic thrombocytopenic purpura as indicated by microangiopathic hemolytic anemia and thrombocytopenia. The patient had evidence of myocardial injury as indicated by elevation of cardiac enzymes. A 12-lead electrocardiogram demonstrated ST elevation (up to 5 mm) in leads V(2) to V(6). The patient was treated with plasma exchange with fresh frozen plasma in addition to nitroglycerin, metoprolol and prednisone in a tapering dose. After reviewing the literature, we believe that the etiology of myocardial damage remains elusive, but may be secondary to an autoimmune phenomenon resulting in microthrombosis and myocarditis. We were unable to find any documentation about any specific treatment in such patients. Further studies are awaited regarding appropriate treatment of patients with thrombotic thrombocytopenic purpura and acute electrocardiographic changes.     

Studies of a patient with recurring thrombotic thrombocytopenic purpura

A young woman with recurring bouts of thrombotic thrombocytopenic purpura presented an opportunity to study a single patient serially and in depth. This patient underwent 12 remissions in response to fresh frozen plasma, and as little as 250 ml of plasma or plasma from which cryoprecipitate had been extracted was efficacious. Neither the Lian factor nor alterations in either cold-insoluble globulin or protein C could be demonstrated. Evidence in support of the concept of “exhausted” platelets was generated, in that immediately before a thrombocytopenic relapse, platelet function was altered as manifested by bruising, prolongation of the bleeding time, and decreased platelet aggregation. The in vitro addition of normal plasma to such platelets did not improve aggregation. The antiplatelet agent sulfinpyrazone did not seem to add efficacy. Serial observations support the hypothesis that the progression of pathophysiologic events in this patient is: exhausted platelets, thrombocytopenia, hemolysis, renal perturbations, and central nervous system dysfunction.     

Fatal cardiac arrhythmia in a patient with thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP), a well-recognized syndrome with typical diagnostic features, has been implicated in rare causes of sudden death. Involvement of TTP on the myocardium and cardiac conduction system has been characterized, but the consequences related to these effects are infrequently reported. The outcome is almost always death, thus underscoring the need for early detection and therapy. A case is reported of a 36-year-old man who suffered a fatal cardiac arrhythmia related to intramyocardial hemorrhage with a diagnosis of TTP established by postmortem examination.     

Refractory thrombotic thrombocytopenic purpura in a lupus nephritis patient

Thrombotic thrombocytopenic purpura is an uncommon disease and is rare in systemic lupus erythematosus. Rarely, patients do not respond to plasma exchange therapy, and treatment options are limited and often disappointing, thus it is a therapeutic challenge to clinicians. We report a patient with lupus nephritis who developed refractory thrombotic thrombocytopenic purpura and was subsequently treated with cyclosporin A. An immediate and sustained response was demonstrated, and she remained well; eventually she was able to be cyclosporin free after the acute episode. Cyclosporin A can be a safe and effective therapeutic option in patients with refractory thrombotic thrombocytopenic purpura.     

Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

Abstract

We encountered a 39-year-old female patient with systemic lupus erythematosus (SLE) in whom thrombotic thrombocytopenic purpura (TTP) recurred. The patient was successfully treated with corticosteroid in combination with immunosuppressive agents. Because TTP complicating SLE is more resistant to treatment than idiopathic TTP, prompt diagnosis and efficacious initial treatment are critical.     

Recurrent thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

We encountered a 39-year-old female patient with systemic lupus erythematosus (SLE) in whom thrombotic thrombocytopenic purpura (TTP) recurred. The patient was successfully treated with corticosteroid in combination with immunosuppressive agents. Because TTP complicating SLE is more resistant to treatment than idiopathic TTP, prompt diagnosis and efficacious initial treatment are critical.     

Fatal thrombotic thrombocytopenic purpura in a patient with systemic lupus erythematosus

An immunologic mechanism, possibly immune complex mediated, has been suggested as the basis for the pathogenesis of thrombotic thrombocytopenic purpura (TTP). The evidence supporting this concept has been the association of TTP with systemic lupus erythematosus and the successful therapy of TTP by plasmapheresis. However, most investigators have failed to demonstrate elevated circulating immune complexes during the course of TTP. This report describes a young woman with systemic lupus who developed TTP as a terminal event. Elevated levels of immune complexes were associated with periods of active lupus but were not detectable at the time she developed TTP.     

Life-threatening thrombotic thrombocytopenic purpura (TTP) in a patient with sickle cell-hemoglobin C disease

We report a patient with hemoglobin sickle cell-hemoglobin C disease who developed the clinical syndrome of thrombotic thrombocytopenic purpura (TTP) during admission for typical acute pain crisis. The potential for multiorgan involvement secondary to vaso-occlusive crisis complicated the diagnosis and overlapped with the patient's clinical presentation of chronic bone pain and hemolytic anemia. Clinical improvement and normalization of laboratory parameters followed rapidly in response to plasma exchange therapy.     

Effects of intravenous immunoglobulin in a patient with intermittent thrombotic thrombocytopenic purpura

We describe a patient with a 9-year history of thrombotic thrombocytopenic purpura (TTP) who exhibited four relapses. Intravenous immunoglobulin (IVIg) was effective for these four episodes. The patient was well and the laboratory findings were within normal ranges between each episode, although unusually large von Willebrand factor multimers were observed during remission. Our results suggest the usefulness of IVIg at the time of relapse in the treatment of patients with TTP who have multiple relapses over a long period.     

Thrombocytopenia and hemolytic anemia in a patient with mixed connective tissue disease due to thrombotic thrombocytopenic purpura

A 33-year-old woman with mixed connective tissue disease (MCTD) presented with headache, fever, thrombocytopenia, hemolytic anemia, and renal involvement due to thrombotic thrombocytopenic purpura (TTP). She did not improve after treatment with prednisolone, fresh frozen plasma, antiplatelet agents, and prostacyclin, but a trial with vincristine resulted in a longlasting complete remission. TTP in autoimmune diseases probably results from immune mediated vasculopathy, which was demonstrated in our patient using nailfold capillary microscopy. Though TTP has many clinical and laboratory features in common with active MCTD, recognition of differences between the 2 conditions, i.e., microangiopathic hemolytic anemia and a negative Coombs' test in the former, is important because treatment in the 2 conditions differs.     

Dermatopathic lymphadenopathy in a patient with adult onset Still's disease

Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown cause characterized by high fever accompanied by systemic manifestations. Since AOSD consists of heterogeneous symptoms and has no definite diagnostic tool, the diagnosis is based upon exclusive criteria. Dermatopathic lymphadenopathy (DL) is characterized by a localized paracortical proliferation of histiocytes and deposition of melanin in the lymph nodes. DL is not only a reactive hyperplasia of the lymph nodes, but has also been reported to be associated with hematological malignancies such as cutaneous T cell lymphoma (CTCL) and Hodgkin's lymphoma. It is therefore important to evaluate CTCL or Hodgkin's lymphoma in a patient with DL, in order to both rule out hematological malignancy and diagnose AOSD. In this report, we first describe a 37-year-old patient with AOSD whose biopsy of lymph node was proved to be DL.     

Reversible posterior leukoencephalopathy in an HIV-infected patient with thrombotic thrombocytopenic purpura

We describe a 37-y-old male with advanced HIV disease who was diagnosed with thrombotic thrombocytopenic purpura after MRI of the brain revealed reversible posterior leukoencephalopathy. We discuss reversible posterior leukoencephalopathy as a diagnostic clue in HIV-infected patients with multi-organ system disease.     

Delayed diagnosis of congenital thrombotic thrombocytopenic purpura in a patient with recurrent strokes

Journal of Thrombosis and Thrombolysis - Congenital thrombotic thrombocytopenic purpura (cTTP) is caused by ADAMTS13 mutations and associated with high risk of microvascular thrombosis. A...