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What are the key clinical features that should raise suspicion for Thrombotic Thrombocytopenic Purpura (TTP) in a patient?
Answer
Suspicion for Thrombotic Thrombocytopenic Purpura (TTP) should be raised by a combination of key clinical features, which are often described as a pentad, though not all features may be present 1 (Patel et al., 2009).
The hallmark features include microangiopathic haemolytic anaemia (MAHA) and severe thrombocytopenia 1 (Thompson et al., 1992). MAHA is indicated by evidence of red blood cell fragmentation (schistocytes) on a blood film, elevated lactate dehydrogenase (LDH), and undetectable haptoglobin 1. Thrombocytopenia refers to a low platelet count 1.
Additional features that raise suspicion for TTP include neurological abnormalities, such as confusion, seizures, or focal neurological deficits 3 (Thompson et al., 1992). Renal impairment, indicated by elevated creatinine or proteinuria, is another important sign 1 (Thompson et al., 1992). Fever may also be present 1 (Thompson et al., 1992).
It is important to note that TTP can present as a “masquerader,” meaning its symptoms can be varied and non-specific, making diagnosis challenging 1 (Patel et al., 2009). Therefore, a high index of suspicion is crucial, especially when thrombocytopenia and MAHA are present 1. In pregnant women, TTP or similar thrombotic microangiopathies should be considered when evaluating suspected cases of pre-eclampsia or HELLP syndrome, as their presentations can overlap 2 (McMinn and George, 2001). Abnormal platelet counts, including thrombocytopenia, warrant investigation to rule out serious underlying conditions like TTP 1.
Key References
- CKS - Platelets - abnormal counts and cancer
- NG121 - Intrapartum care for women with existing medical conditions or obstetric complications and their babies
- CKS - Stroke and TIA
- CKS - Parvovirus B19 infection
- (Thompson et al., 1992): Thrombotic microangiopathies in the 1980s: clinical features, response to treatment, and the impact of the human immunodeficiency virus epidemic.
- (McMinn and George, 2001): Evaluation of women with clinically suspected thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy.
- (Patel et al., 2009): Thrombotic thrombocytopenic purpura: the masquerader.
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