Diferencia entre revisiones de «Thrombotic thrombocytopenic purpura»
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===Risk Factors=== | ===Risk Factors=== | ||
*Congenitally deficient ADAMTS-13 activity and: | |||
#Pregnancy | |||
#Infection | |||
#Inflammation | |||
#Medication use (quinolones, ticlopidine, clopidogrel) | |||
==Clinical Features== | ==Clinical Features== | ||
*Pentad | |||
## | #Microangiopathic hemolytic anemia | ||
### | #Thrombocytopenia | ||
#Fever | |||
#Renal pathology | |||
#CNS abnormalities (seizure, AMS, CVA, coma) | |||
*TTP pentad mnemonic = FAT RN | |||
**Fever, Anemia, Thrombocytopenia, Renal, Neuro sx | |||
==DDX== | ==DDX== | ||
*Other microangiopathic hemolytic anemias (HUS, DIC, malignant HTN) | |||
*ITP | |||
*Sepsis | |||
*SLE | |||
*HELLP syndrome | |||
**Difficult to distinguish | |||
**Anemia, plt count, and LDH tend to be more severe in TTP; LFTs more severe in HELLP | |||
== Work-Up == | == Work-Up == | ||
*CBC with peripheral smear (anemia, '''schistocytes''', thrombocytopenia) | |||
*LDH (elevated) | |||
*Haptoglobin (decreased) | |||
*Reticulocyte count (appropriate) | |||
*UA (hemoglobinuria) | |||
*Creatinine (possibly elevated) | |||
*LFTs (increased bilirubin) | |||
*PT/PTT/INR (normal; differentiates from DIC) | |||
*Urine pregnancy (significant association between pregnancy and TTP) | |||
== Treatment == | == Treatment == | ||
*Plasma exchange (plasmapheresis) | |||
**Replaces defective or insufficient ADAMTS-13 and clears vWF multimers | |||
*Transfusion | |||
**Indicated if plasma exchange cannot be performed immediately | |||
**FFP | |||
**Platelet (only for life-threatening bleeding or intracranial hemorrhage) | |||
***Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death | |||
*Splenectomy - 2nd line therapy | |||
==Disposition== | ==Disposition== | ||
Revisión del 17:46 4 oct 2013
Background
- Pathophysiology
- Insufficient ADAMTS-13 activity allows vWF multimers to accumulate in microcirculation
- Leads to platelet aggregation/thrombocytopenia and hemolysis of RBCs
- Insufficient ADAMTS-13 activity allows vWF multimers to accumulate in microcirculation
- Similar to but different from HUS (which is more common in peds)
- Microangiopathic hemolytic anemia + low plts is TTP until proven otherwise
Risk Factors
- Congenitally deficient ADAMTS-13 activity and:
- Pregnancy
- Infection
- Inflammation
- Medication use (quinolones, ticlopidine, clopidogrel)
Clinical Features
- Pentad
- Microangiopathic hemolytic anemia
- Thrombocytopenia
- Fever
- Renal pathology
- CNS abnormalities (seizure, AMS, CVA, coma)
- TTP pentad mnemonic = FAT RN
- Fever, Anemia, Thrombocytopenia, Renal, Neuro sx
DDX
- Other microangiopathic hemolytic anemias (HUS, DIC, malignant HTN)
- ITP
- Sepsis
- SLE
- HELLP syndrome
- Difficult to distinguish
- Anemia, plt count, and LDH tend to be more severe in TTP; LFTs more severe in HELLP
Work-Up
- CBC with peripheral smear (anemia, schistocytes, thrombocytopenia)
- LDH (elevated)
- Haptoglobin (decreased)
- Reticulocyte count (appropriate)
- UA (hemoglobinuria)
- Creatinine (possibly elevated)
- LFTs (increased bilirubin)
- PT/PTT/INR (normal; differentiates from DIC)
- Urine pregnancy (significant association between pregnancy and TTP)
Treatment
- Plasma exchange (plasmapheresis)
- Replaces defective or insufficient ADAMTS-13 and clears vWF multimers
- Transfusion
- Indicated if plasma exchange cannot be performed immediately
- FFP
- Platelet (only for life-threatening bleeding or intracranial hemorrhage)
- Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death
- Splenectomy - 2nd line therapy
Disposition
- Admit for plasma exchange
Source
- Tintinalli
- Harwood Nuss