Diferencia entre revisiones de «Thrombotic thrombocytopenic purpura»
Sin resumen de edición |
(edit) |
||
| Línea 4: | Línea 4: | ||
***Leads to platelet aggregation/thrombocytopenia and hemolysis of RBCs | ***Leads to platelet aggregation/thrombocytopenia and hemolysis of RBCs | ||
*Similar to but different from [[HUS]] (which is more common in peds) | *Similar to but different from [[HUS]] (which is more common in peds) | ||
*Microangiopathic hemolytic anemia + low plts is TTP until proven otherwise | *[[Microangiopathic hemolytic anemia]] + low plts is TTP until proven otherwise | ||
*Can occur as a result of [[Plavix]] (clopidogrel) use (usually within first 2 wk of starting) | |||
===Risk Factors=== | ===Risk Factors=== | ||
| Línea 15: | Línea 16: | ||
==Clinical Features== | ==Clinical Features== | ||
*Pentad | *Pentad | ||
#Microangiopathic hemolytic anemia | #[[Microangiopathic hemolytic anemia]] | ||
#Thrombocytopenia | #Thrombocytopenia | ||
#Fever | #Fever | ||
| Línea 25: | Línea 26: | ||
==DDX== | ==DDX== | ||
*Other microangiopathic hemolytic anemias (HUS, DIC, malignant | *Other microangiopathic hemolytic anemias (eg, [[HUS]], [[DIC]], [[malignant hypertension]]) | ||
*ITP | *[[ITP]] | ||
*Sepsis | *[[Sepsis]] | ||
*SLE | *[[SLE]] | ||
*HELLP syndrome | *[[HELLP]] syndrome | ||
**Difficult to distinguish | **Difficult to distinguish | ||
**Anemia, plt count, and LDH tend to be more severe in TTP; LFTs more severe in HELLP | **Anemia, plt count, and LDH tend to be more severe in TTP; LFTs more severe in HELLP | ||
| Línea 42: | Línea 43: | ||
*Creatinine (possibly elevated) | *Creatinine (possibly elevated) | ||
*LFTs (increased bilirubin) | *LFTs (increased bilirubin) | ||
*PT/PTT/INR (normal; differentiates from DIC) | *PT/PTT/INR (normal; differentiates from [[DIC]]) | ||
*Urine pregnancy (significant association between pregnancy and TTP) | *Urine pregnancy (significant association between pregnancy and TTP) | ||
Revisión del 16:42 9 nov 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
- Can occur as a result of Plavix (clopidogrel) use (usually within first 2 wk of starting)
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 (eg, HUS, DIC, malignant hypertension)
- 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