Diferencia entre revisiones de «Thrombotic thrombocytopenic purpura»
Sin resumen de edición |
(Clarified diagnosis and minor aditions to pathophys) |
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| Línea 3: | Línea 3: | ||
**Insufficient ADAMTS-13 activity allows vWF multimers to accumulate in microcirculation | **Insufficient ADAMTS-13 activity allows vWF multimers to accumulate in microcirculation | ||
***Leads to platelet aggregation/thrombocytopenia and hemolysis of RBCs | ***Leads to platelet aggregation/thrombocytopenia and hemolysis of RBCs | ||
****Platelet clots are transient, symptoms, especially neuro, can be in flux | |||
*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 (MAHA)]] + low plts is TTP until proven otherwise | *[[Microangiopathic Hemolytic Anemia (MAHA)]] + low plts is TTP until proven otherwise | ||
| Línea 15: | Línea 16: | ||
==Clinical Features== | ==Clinical Features== | ||
*Pentad | *Pentad (rarely all present) | ||
#[[Microangiopathic Hemolytic Anemia (MAHA)]] | #[[Microangiopathic Hemolytic Anemia (MAHA)]] | ||
#Thrombocytopenia | #Thrombocytopenia | ||
| Línea 21: | Línea 22: | ||
#Renal pathology | #Renal pathology | ||
#CNS abnormalities (seizure, AMS, CVA, coma) | #CNS abnormalities (seizure, AMS, CVA, coma) | ||
*Neuro symptoms are often transient, may not be present in ED | |||
*TTP pentad mnemonic = FAT RN | *TTP pentad mnemonic = FAT RN | ||
**Fever, Anemia, Thrombocytopenia, Renal, Neuro sx | **Fever, Anemia, Thrombocytopenia, Renal, Neuro sx | ||
***All features do not need to be present at the same time | |||
*Consider diagnosis without the full pentad | |||
**MAHA + Thrombocytopenia + any other feature | |||
==DDX== | ==DDX== | ||
| Línea 53: | Línea 59: | ||
**Indicated if plasma exchange cannot be performed immediately | **Indicated if plasma exchange cannot be performed immediately | ||
**FFP | **FFP | ||
***Contains ADAMTS-13 | |||
**Platelet (only for life-threatening bleeding or intracranial hemorrhage) | **Platelet (only for life-threatening bleeding or intracranial hemorrhage) | ||
***Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death | ***Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death | ||
| Línea 68: | Línea 75: | ||
*Tintinalli | *Tintinalli | ||
*Harwood Nuss | *Harwood Nuss | ||
*Rosens | |||
[[Category:Heme/Onc]] | [[Category:Heme/Onc]] | ||
Revisión del 14:45 25 mar 2014
Background
- Pathophysiology
- Insufficient ADAMTS-13 activity allows vWF multimers to accumulate in microcirculation
- Leads to platelet aggregation/thrombocytopenia and hemolysis of RBCs
- Platelet clots are transient, symptoms, especially neuro, can be in flux
- 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 (MAHA) + 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 (rarely all present)
- Microangiopathic Hemolytic Anemia (MAHA)
- Thrombocytopenia
- Fever
- Renal pathology
- CNS abnormalities (seizure, AMS, CVA, coma)
- Neuro symptoms are often transient, may not be present in ED
- TTP pentad mnemonic = FAT RN
- Fever, Anemia, Thrombocytopenia, Renal, Neuro sx
- All features do not need to be present at the same time
- Fever, Anemia, Thrombocytopenia, Renal, Neuro sx
- Consider diagnosis without the full pentad
- MAHA + Thrombocytopenia + any other feature
DDX
- Other MAHAs (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
- Contains ADAMTS-13
- 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
See Also
Source
- Tintinalli
- Harwood Nuss
- Rosens