TTP-HUS

TTP-HUS

Abbreviation for thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. See: thrombotic thrombocytopenic purpura, hemolytic uremic syndrome.

TTP-HUS

The combination of thrombotic thrombocytopenic purpura–TTP and hemolytic-uremic syndrome–HUS, polar expressions of the same disease defined by a pentad of TTP features: Thrombocytopenia, microangiopathic hemolytic anemia, neurologic defects, fever, renal disease Clinical Abrupt onset in children after a viral URI; may be associated with a verotoxin in E coli-induced gastroenteritis; it may occur in pregnancy, at parturition, or during chemotherapy; spontaneously resolving renal failure–RF occurs in 60%–10% progress to chronic RF; RF is more common in E coli O157:H7 infections, which are linked to 16% of cases with HUS Lab Reticulocytosis, ↑ BR, ↑ FDPs without DIC, ↓ haptoglobin Management Most resolve spontaneously, others require high-dose corticosteroids Prognosis with prednisone and plasma exchange, 91% survival
References in periodicals archive ?
The incidence in the region of the Oklahoma TTP-HUS Registry for all patients who are clinically diagnosed with TTP is 11.
4,9,12) Finally, some patients in the Oklahoma TTP-HUS Registry have had an established SLE diagnosis; we describe these patients as having both SLE and TTP, (4,9) but we recognize that accurate diagnosis of these patients is difficult.
We previously reported 17 patients with quinine-associated TTP-HUS (18).
The patients described here are from the Oklahoma TTP-HUS registry (20) and were included in a previous report describing quinine-associated TTP-HUS (18).
The increased LDH level was considered to be due to both hemolysis and muscle ischemia, as both typically occur in TTP-HUS (22).
However, when the previous history of TTP-HUS was recognized, plasma exchange was begun.
In 1995, quinine-associated TTP-HUS had been recently reported (16, 23), and it was during this episode that the association of her episodes with quinine was recognized.
This presumably occurs because of severe systemic muscle ischemia caused by systemic microvascular thrombosis (22), the principal pathogenic feature of TTP-HUS.
Because of the acute hematologic and renal abnormalities, TTP-HUS was suspected, and plasma exchange and hemodialysis were begun the day after admission.
TTP-HUS is a relatively uncommon, life-threatening cause of thrombocytopenia.
It is important to consider TTP-HUS as a differential.
Pathophysiologic classification of thrombocytopenia Decreased Production Increased Destruction * Hematologic malignancies Immune * Aplastic anemia * ITP * Myelodysplasia * HIT * Drugs: chemotherapy, alcohol * Drug-induced antibodies * Radiation * HIV * HIV * Post transfusion purpura * Vitamin D deficiencies * Connective tissue diseases * Hereditary thrombocytopenias Nonimmune * Metastatic cancer to bone marrow * DIC * Sepsis * Cardiac valves * TTP-HUS * Kasabach Merrit syndrome Splenic Sequestration * Hypersplenism HIV, human immunodeficiency virus; ITP, idiopathic thrombocytopenic purpura; HIT, heparin-induced thrombocytopenia; DIC, disseminated intravascular coagulation; TTP-HUS, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.