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Diagnosis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in adults
Descripción
Mapa Mental sobre Diagnosis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in adults, creado por Erick Quirola el 12/02/2022.
Mapa Mental por
Erick Quirola
, actualizado hace más de 1 año
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Creado por
Erick Quirola
hace más de 2 años
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Resumen del Recurso
Diagnosis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in adults
Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS)
Are both acute syndromes with abnormalities in multiple organ systems and evidencing microangiopathic hemolytic anemia and thrombocytopenia
CLINICAL AND LABORATORY MANIFESTATIONS
Pentad
Microangiopathic hemolytic anemia
Hemolysis
> serum indirect bilirubin concentration
> serum LDH
< serum haptoglobin concentration
Thrombocytopenia
Renal disease
For renal thrombotic microangiopathy
Mild proteinuria
1-2 g/day
Acute renal insufficiency
With Anuria
Requiring acute dialysis.
Neurologic abnormalities
Confusion, severe headache
Transient aphasia, transient ischemic attack, stroke
High fever with chills
Diagnosis of sepsis/infection and DIC rather than TTP-HUS
Now the diagnosis is with
Thrombocytopenia and microangiopathic hemolytic anemia
Cardiac involvement
Diffuse platelet thrombi and associated hemorrhage and patches of necrosis in cardiac tissues
Arrhythmia, sudden cardiac death, myocardial infarction, cardiogenic shock, and/or heart failure
ADAMTS13 deficiency
Understanding pathogenesis of congenital and acquired causes of this disorder and for anticipating the long term risk for relapse
Congenital TTP or Upshaw-Schulman syndrome
Absent or very low levels of ADAMTS13 activity
Factors are infection (eg, influenza), pregnancy, surgery, and pancreatitis
Causes: TTP occurring during an initial pregnancy
Diagnosis
Repeatedly low to absent levels of ADAMTS13 during clinical remission
Absence of an inhibitor to ADAMTS13
Homozygosity or compound heterozygosity for mutation in the ADAMTS13 gene
Differential Diagnosis
Bloody diarrhea: Shiga toxin-producing bacteria
Pregnancy: Symptoms near term or early in the postpartum period.
Drugs: Dose-dependent toxicity (mitomycin C, gemcitabine , cyclosporine)
Presence of quinine
Systemic disorders to be considered
Thrombocytopenia, microangiopathic hemolytic anemia, and renal failure
Vasculitis and other connective tissue disease
Severe hypertension and renal involvement
Catastrophic antiphospholipid syndrome
Widespread microvascular and macrovascular changes due to intravascular thrombosis
Scleroderma renal crisis
Acute onset of renal failure
Hypertension
Headache, blurred vision, seizures, microscopic hematuria and granular casts
Malignant hypertension
History of uncontrolled hypertension
Diastolic pressure that is typically above 130 mmHg, and hemorrhages and papilledema on retinal examination
TTP-HUS versus DIC
Sepsis, shock, or an obstetrical complication including severe preeclampsia
Postpartum acute renal failure
Disseminated malignancy
Mucin-producing adenocarcinomas
Treatment
Curative plasma exchange therapy
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