Humans ; Purpura, Thrombotic Thrombocytopenic ; diagnosis ; therapy

Thrombotic microangiopathy (TMA) is a group of highly heterogeneous, acute and severe clinicopathological syndromes, characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and ischemic injury of end organs. TMA has the characteristics of dangerous condition, multiple organ involvement and high mortality. Patients with severe TMA need to be admitted to intensive care unit (ICU) for organ function support therapy. Early and rapid evaluation, differential diagnosis, and timely and effective treatment are the key to improve the prognosis of TMA patients. Here, we review the pathophysiological changes, diagnosis differential diagnosis, and treatment of the severe TMA in adult.
Adult ; Humans ; Thrombotic Microangiopathies/therapy* ; Purpura, Thrombotic Thrombocytopenic/therapy* ; Anemia, Hemolytic/therapy* ; Treatment Outcome ; Diagnosis, Differential

Thrombotic thrombocytopenic purpura(TTP) is a clinical syndrome of unknown etiology and characterized by microangiopathic hemolytic anemia, thrombocytopenia, fluctuating neurological status, renal dysfunction and fever. Systemic lupus erythromatosus(SLE) is also multisystemic disease that some of clinical features may mimic TTP. Therefore both diseases have led to diagnostic confusion. We experienced two cases with SLE who subsequently or initially developed TTP. In case 1, a 44-year old woman had 1-year previous history of SLE and presented with dyspnea. After diagnosis of thrombotic microangiopathy by renal biopsy, she was managed with steroid, cyclophosphamide pulse therapy, fresh frozen plasma infusion and plasmapheresis. She was treated by aggressive treatment; nevertheless, she died on 15th admission day. In case 2, a 22-year old man was admitted because of nausea and vomiting. SLE with TTP was diagnosed by ARA criteria and the finding of microangiopathic hemolytic anemia. He was treated with plasmapheresis, fresh frozen plasma infusion and steroid therapy. He showed clinical response to the therapy, and has shown no recurrence of disease until now on. In conclusion, we suggest that early diagnosis and prompt therapy such as plasmapheresis and plasma infusion are very important in SLE with TTP.
Adult ; Anemia, Hemolytic ; Biopsy ; Cyclophosphamide ; Diagnosis ; Dyspnea ; Early Diagnosis ; Female ; Fever ; Humans ; Nausea ; Plasma ; Plasmapheresis ; Purpura, Thrombocytopenic* ; Purpura, Thrombotic Thrombocytopenic ; Recurrence ; Thrombocytopenia ; Thrombotic Microangiopathies ; Vomiting ; Young Adult

Anemia, Hemolytic, Congenital ; diagnosis ; pathology ; therapy ; Humans ; Infant ; Male ; Plasma ; Purpura, Thrombotic Thrombocytopenic ; diagnosis ; pathology ; therapy

Thrombotic thrombocytopenic purpura (TTP), a fatal disease, is mostly idiopathic but can occur secondary to cancer, infection, transplantation, pregnancy, surgery, or drugs. The mechanism of TTP is still unknown, however, and detection is difficult because of unclear diagnosis criteria. Colonic stent insertion is commonly used in management of malignant colon obstruction. This is a very safe procedure with a low procedure-related mortality rate, but serious complications can develop. The authors first experienced a patient with TTP when the phenomenon occurred after stent insertion for palliation of obstructive colon cancer and therefore would like to report the case.
Colon ; Colonic Neoplasms* ; Diagnosis ; Humans ; Mortality ; Pregnancy ; Purpura, Thrombotic Thrombocytopenic* ; Stents*

Thrombotic thrombocytopenic purpura (TTP) rarely may be seen in association with autoimmune processes such as scleroderma, rheumatoid arthritis, polyarteritis nodosa, Sj gren's syndrome, and systemic lupus erythematosusus (SLE). The diagnosis of TTP as a syndrome distinct from SLE may be challenging, because both processes may present with some or all elements of the classic pentad considered pathognomonic of the former: microangiopathic hemolytic anemia, fever, thrombocytopenia, neurological deficits, and renal abnormalities. We describe a patient with synchronous TTP and SLE, and review the literature.
Anemia, Hemolytic ; Arthritis, Rheumatoid ; Diagnosis ; Fever ; Humans ; Lupus Erythematosus, Systemic* ; Polyarteritis Nodosa ; Purpura, Thrombotic Thrombocytopenic* ; Thrombocytopenia

BACKGROUND: Ever since medical professionals have recognized the important role of ADAMTS-13 in the pathogenesis of TTP, several methods to diagnosis the activity of ADAMTS-13 in the plasma of TTP patients haves been developed. However these assays have not been widely used in practice because they are cumbersome and they require several days to complete. In this study we examine the new, rapid ADAMTS-13 activity assay that uses fluorescence resonance energy transfer and we compared it with the conventional assay to determine its diagnostic advantage. METHODS: Seven TTP patients were compared with 60 healthy controls. The plasma ADAMTS-13 activity was measured using the fluorescence-quenching substrate assay method. The results were compared with the results of performing multimer analysis of SDS-agarose gel electrophoresis. RESULTS: It took only 2 hour to complete the fluorescence-quenching substrate assay. The median ADAMTS-13 activity using the fluorescence-quenching substrate was 5.9% (range: 0~29.9%) for the patient group and 99.1% (range: 74.4~143.3%) for the healthy group, respectively. The median ADAMTS-13 activity using multimer analysis of SDS-agarose gel electrophoresis was 5.6% (range: 1.6~28.8%) for the patients group and 87.7% (range: 44.1~120.9%) for the healthy group, respectively. The ADAMTS-13 activities of the two assays were well correlated (correlation coefficient: 0.69). CONCLUSION: The quantification of ADAMTS-13 activity with using the fluorescence-quenching substrate is rapid and highly specific for the diagnosis of TTP and it is expected to be used widely in the diagnosis of TTP.
Diagnosis* ; Electrophoresis ; Fluorescence Resonance Energy Transfer ; Humans ; Plasma ; Purpura, Thrombotic Thrombocytopenic*

ADAMTS13, a plasma metalloprotease, specifically cleaves von Willebrand factor (vWF). Severe deficiency of plasma ADAMTS13 activity results in thrombotic thrombocytopenic purpura (TTP). In this review, the structure and function of ADAMTS13 protease and its relationship with TTP are summarized.
ADAM Proteins ; metabolism ; ADAMTS13 Protein ; Humans ; Purpura, Thrombotic Thrombocytopenic ; diagnosis ; pathology ; therapy

Adolescent ; Adult ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; Male ; Purpura, Thrombotic Thrombocytopenic ; diagnosis ; etiology ; therapy

Thrombotic thrombocytopenic purpura is a rare but fatal complication of systemic lupus erythematosus. The diagnosis of thrombotic thrombocytopenic purpura as a syndrome distinct from systemic lupus erythematosus may be challenging particularly when thrombotic thrombocytopenic purpura is presented concomitantly with systemic lupus erythematosus. Early diagnosis and aggressive treatment including plasmapheresis would be required. However, recent reports have suggested that the use of cyclophosphamide may have a role. We describe a patient with systemic lupus erythematosus who was first presented with severe thrombotic thrombocytopenic purpura. Diagnosis was based on typical clinical features of thrombotic thrombocytopenic purpura and laboratory findings of active lupus nephritis. Renal biopsy also confirmed the coexistence of thrombotic thrombocytopenic purpura and diffuse proliferative lupus nephritis. Although prompt extensive plasmapheresis and high dose steroid therapy were performed, oliguric renal failure and thrombocytopenia persisted. After addition of cyclophosphamide to the treatment with plasmapheresis and steroid, clinical manifestations of thrombotic thrombocytopenic purpura and lupus nephritis were markedly improved.
Biopsy ; Cyclophosphamide* ; Diagnosis ; Early Diagnosis ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Plasmapheresis ; Purpura ; Purpura, Thrombotic Thrombocytopenic* ; Renal Insufficiency ; Thrombocytopenia