Thrombotic thrombocytopenic purpura: the masquerader.
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder with a mortality rate of up to 90% if left untreated, and is characterized by microvascular thrombi, mainly in small arteries and capillaries, thrombocytopenia, hemolysis, and neurological abnormalities. Malignant hypertension (HTN) is a clinical syndrome characterized by severe hypertension associated with end-organ damage; including encephalopathy, renal dysfunction, and retinal hemorrhage due to platelet aggregation in the microcirculation leading to thrombotic microangiopathy (TMA). These are similar to TTP. Malignant hypertension can cause endothelial injury and fibrinoid necrosis in the vessel wall which results in its clinical manifestations. Therefore, it is difficult to differentiate malignant hypertension from TTP. However, it is critical to differentiate these two entities, as early initiation of plasmapheresis in TTP can be life saving. TTP is considered a masquerader as it can present with few clinical features and, if the index of suspicion is not very high, lead to mortality.
Faxton-St. Lukes Hospital, Utica, NY, USA. dranpatel@gmail.com
Anil
A
Patel
Harsh
H
Patel
Anush
A
Patel
D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D058186:Acute Kidney Injury / Q000188:drug therapy; D000328:Adult; D003937:Diagnosis, Differential; D004913:Erythrocytes, Abnormal; D006801:Humans; D006974:Hypertension, Malignant / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology*; D007676:Kidney Failure, Chronic; D008297:Male; D055806:Microvessels / Q000473:pathology / Q000503:physiopathology; D009400:Nephrosclerosis / Q000473:pathology / Q000503:physiopathology; D009529:Nicardipine / Q000627:therapeutic use; D010956:Plasmapheresis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000503:physiopathology* / Q000628:therapy; D014665:Vasodilator Agents / Q000627:therapeutic use
D014665:Vasodilator Agents; D009529:Nicardipine; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human