35404042
Description
Mind Map by Erick Quirola, updated more than 1 year ago
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Created by Erick Quirola
about 2 years ago
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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
- 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
- Hemolysis
- Thrombocytopenia
- Renal disease
- For renal thrombotic microangiopathy
- Mild proteinuria
- 1-2 g/day
- 1-2 g/day
- Acute renal insufficiency
- With Anuria
- Requiring acute dialysis.
- Requiring acute dialysis.
- With Anuria
- Mild proteinuria
- For renal thrombotic microangiopathy
- Neurologic abnormalities
- Confusion, severe
headache
- Transient aphasia,
transient ischemic
attack, stroke
- Confusion, severe
headache
- High fever with chills
- Diagnosis of sepsis/infection and
DIC rather than TTP-HUS
- Diagnosis of sepsis/infection and
DIC rather than TTP-HUS
- Microangiopathic
hemolytic anemia
- Now the diagnosis is with
- Thrombocytopenia and
microangiopathic hemolytic anemia
- 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
- Arrhythmia, sudden cardiac death, myocardial
infarction, cardiogenic shock, and/or heart failure
- Diffuse platelet thrombi and associated hemorrhage
and patches of necrosis in cardiac tissues
- ADAMTS13 deficiency
- Understanding pathogenesis of congenital and
acquired causes of this disorder and for
anticipating the long term risk for relapse
- Understanding pathogenesis of congenital and
acquired causes of this disorder and for
anticipating the long term risk for relapse
- Pentad
- Congenital TTP or Upshaw-Schulman syndrome
- Absent or very low levels of ADAMTS13 activity
- Factors are infection (eg, influenza), pregnancy, surgery, and pancreatitis
- 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
- Repeatedly low to absent levels of
ADAMTS13 during clinical remission
- Absent or very low levels of ADAMTS13 activity
- 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
- Bloody diarrhea: Shiga toxin-producing bacteria
- Systemic disorders
to be considered
- Thrombocytopenia, microangiopathic
hemolytic anemia, and renal failure
- Vasculitis and other connective
tissue disease
- Severe hypertension
and renal involvement
- Severe hypertension
and renal involvement
- Catastrophic antiphospholipid
syndrome
- Widespread microvascular
and macrovascular
changes due to
intravascular thrombosis
- Widespread microvascular
and macrovascular
changes due to
intravascular thrombosis
- Scleroderma renal crisis
- Acute onset of renal failure
- Hypertension
- Hypertension
- Headache, blurred vision, seizures,
microscopic hematuria and granular casts
- Acute onset of renal failure
- Malignant hypertension
- History of uncontrolled
hypertension
- Diastolic pressure that is typically above 130 mmHg, and
hemorrhages and papilledema on retinal examination
- Diastolic pressure that is typically above 130 mmHg, and
hemorrhages and papilledema on retinal examination
- History of uncontrolled
hypertension
- TTP-HUS versus DIC
- Sepsis, shock, or an obstetrical complication
including severe preeclampsia
- Sepsis, shock, or an obstetrical complication
including severe preeclampsia
- Postpartum acute renal failure
- Disseminated malignancy
- Mucin-producing adenocarcinomas
- Mucin-producing adenocarcinomas
- Thrombocytopenia, microangiopathic
hemolytic anemia, and renal failure
- Treatment
- Curative plasma exchange therapy
- Curative plasma exchange therapy
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