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Fragmented Red Blood Cells ( Schistocytes)
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Decreased Fibrinogen and Increased D-dimer
Elevated LDH
Decreased Reticulocyte Count
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The patient presents with fever and possible sepsis. Cloudy urine indicates that UTI is the possible source of sepsis. Recognize that severe sepsis can lead to multi-organ dysfunction such as hypotension, encephalopathy. Disseminated… intravascular coagulation and renal insufficiency. The latter three are seen in this patient. Recognize that the important difference between DIC and TTP is that DIC is a consumption coagulopathy i.e; it consumes the entire coagulation factors along with platelets. Hence, PT and PTT are elevated and fibrinogen is decreased in DIC but not in TTP. The intravascular thrombi in DIC are fibrin thrombi – the lysis of these lead to increased D-Dimer and Fibrin Split products. TTP is a consumption thrombocytopenia and is composed of platelet thrombi not fibrin – so, D-dimer is usually normal in TTP. Increased LDH suggests hemolysis here and is non-specific. MAHA is associated with increased reticulocyte count not decreased retic. The distractors in the question are typical TTP like pentad and scistocytes on the smear. However, realize that severe sepsis can have all these features ( Fever, thrombocytopenia, DIC leading to MAHA, altered mental status and renal failure). So, the entire clinical scenario should be put together in arriving at the diagnosis. The peripheral blood smear shows Schistocytes. Recognize that schistocytes are not specific for TTP. Schistocytes can occur in any condition that is associated with Microangiopathic Hemolysis (MAHA). MAHA can occur in conditions where intravascular thrombi rub against RBC in tiny capillaries leading to RBC fragmentation and hemolysis eg: MAHA can be seen in TTP, HUS, DIC, HELLP Syndrome and Malignant Hypertension. Key Concepts: 1. DIC is a consumptive coagulopathy-thrombocytopenia and occurs secondary to several causes. 2. TTP is non-immune consumptive thrombocytopenia. PT and PTT are usually normal. 3. Severe sepsis can resemble TTP. Full clinical picture should be considered in decision making. Source of sepsis should be sought and ruled out in suspected cases before making a diagnosis of TTP 4. MAHA is not specific for TTP. Recognize other causes of MAHA are DIC, HUS, HELLP and Malignant Hypertension.
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