Thrombotic Thrombocytopenic Purpura/Hemolytic Uremic Syndrome and Disseminated Intravascular Coagulation
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Abstract:
Thrombotic microangiopathies (TMA) are a group of conditions characterized by excessive platelet activation, resulting in microvascular thrombi and platelet consumption (thrombocytopenia). The thrombosed microvasculature shears red blood cells (RBCs), causing microangiopathic hemolytic anemia. Primary TMAs include thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), while secondary TMAs result from infection, pregnancy, malignancy, drugs and toxins, among other causes.Keywords:
Schistocyte
Microangiopathic hemolytic anemia
Purpura (gastropod)
A hallmark of the clinical syndrome of thrombotic thrombocytopenic purpura (TTP) is evidence of microangiopathic hemolytic anemia. The presence of schistocytes on the peripheral blood smear, elevated plasma lactic dehydrogenase, and decreased haptoglobin concentration are used as evidence of microangiopathic hemolytic anemia to make a diagnosis of TTP. This report describes a case of recurrence of TTP in the absence of schistocytes in the peripheral blood smear during the recurrent episode. Although careful attention should be paid to microscopic examination of a blood smear in any patient presenting with acute renal failure and thrombocytopenia, this case emphasizes the need to consider TTP-hemolytic uremic syndrome in the differential diagnosis, even in the absence of peripheral schistocytosis.
Schistocyte
Microangiopathic hemolytic anemia
Haptoglobin
Purpura (gastropod)
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Objective:To investigate clinical features,outcome and laboratory characteristics of thrombotic thrombocytopenic purpura(TTP) and improve diagnostic ability for physician in TTP management. Methods:Thirty-seven TTP patients admitted to our hospital from 1998 to 2009 were analyzed. The number of schistocytes per 5,000 red cells was counted at 1000-power magnification. The results were expressed as number of schistocytes per 100 red cells. Results:There were 30 patients(81.1%) with the triad of TTP,including hemolytic anemia,thrombocytopenia and neurologic abnormalities;7(18.9%) had the classical pentad of TTP. Major etiologic factors were acquired autoimmunological abnormalities(35.14%) or idiopathic TTP(56.76%) . The schistocytes of peripheral blood smears were present in all cases with a mean of 4.4% and a range of 0.3%-13.4%. In the studies,plasmapheresis in combination with plasma infusion is the main therapeutic method. 23of 26 patients(88.46%) accepting plasmapheresis achieved complete remission;those patients who only underwent plasma infusion had low remission rate(18%) and high mortality(9/11;82%) . 4 patients with packed RBC infusion manifested transient exacerbation of neurologic or psychiatric symptoms. Conclusions:In conclusion,the diagnosis of TTP is still based on clinical features including evidence of microangiopathic haemolysis and an increased schistocytes count is more valuable and simpler method for the diagnosis of TTP. Plasmapheresis is still the optimal treatment method for TTP patients.
Schistocyte
Plasmapheresis
Microangiopathic hemolytic anemia
Haemolysis
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Microangiopathic hemolytic anemia
Eculizumab
Thrombotic microangiopathy
Schistocyte
Abnormality
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Microangiopathic hemolytic anemia (MAHA) may occur as a paraneoplastic syndrome in some solid tumors, but MAHA accompanied by signet ring cell carcinoma (SRCC) of an unknown origin is very rare. We report a patient who presented with an acute onset of Coombs negative hemolytic anemia and frequent schistocytes in the peripheral blood smear which are typical for MAHA as initial presentation of metastatic SRCC. Our patients fulfilled the criteria of thrombotic thrombocytopenic purpura (TTP) and received the specific treatment for TTP without improvement.
Microangiopathic hemolytic anemia
Schistocyte
Purpura (gastropod)
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Schistocyte
Microangiopathic hemolytic anemia
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A 33-year-old woman was seen with fever, confusion, and respiratory distress. A diagnosis of thrombotic thrombocytopenic purpura (TTP) was made when renal failure, microangiopathic hemolytic anemia, and thrombocytopenia subsequently developed. An open kidney biopsy confirmed the clinical diagnosis. The patient initially was seen with the roentgenographic and clinical appearance of pulmonary hemorrhage, which was confirmed histologically and, to our knowledge, previously has not been associated with TTP.
Microangiopathic hemolytic anemia
Schistocyte
Pulmonary hemorrhage
Confusion
Purpura (gastropod)
Thrombocytopenic purpura
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Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP/HUS) is a multisystem disorder characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal function abnormalities, and fever. Coexistence of TTP/HUS and adult onset Still's disease (ASD) is extremely rare. We report the case of a 46-year-old woman who presented with fever, arthritis, myalgias, petechia on skin and confusion five years after the onset of ASD. Thrombocytopenia, renal failure, marked elevation lactate dehydrogenase, and red cell fragmentation on peripheral blood smear were observed. We made a diagnosis of TTP/HUS associated with ASD, according to physical examination and characteristic laboratory data. She recovered from the TTP/HUS following daily sessions of therapeutic plasma exchange with fresh frozen plasma replacement and glucocorticoid therapy. Awareness of the possible development of TTP/HUS in ASD is important for early diagnosis and treatment.
Microangiopathic hemolytic anemia
Schistocyte
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Thrombotic thrombocytopenic purpura (TTP) or Moschovitz' syndrome is rare and is even rarer in childhood. Clinically, it is characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. The etiology is still unknown, although different factors such as large von Willebrand factor multimers and prostacyclin have been implicated. The acute form is more frequent, and in most cases the course is fulminant if treatment is not initiated. Laboratory data typically reveal hemolytic anemia, with schistocytes on the peripheral smear, diminished serum haptoglobin, and thrombocytopenia.We present the clinical cases of two children, aged 4 and 7 respectively, with TTP, but with different evolution and treatment. Evolution was favorable in both patients. The first child recovered spontaneously. In the second plasmapheresis was required and produced remission of all the symptomatology. Normality has been maintained for 36 and 24 months respectively, and the children have presented no clinico-biological alterations.
Microangiopathic hemolytic anemia
Schistocyte
Plasmapheresis
Haptoglobin
Purpura (gastropod)
Fulminant
Etiology
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Microangiopathic hemolytic anemia
Schistocyte
Thrombotic microangiopathy
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Microangiopathic hemolytic anemia
Eculizumab
Thrombotic microangiopathy
Plasmapheresis
Schistocyte
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