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Old 02-26-2011
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Question 40-year-old woman with thrombocytopenia

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A 40-year-old woman has had a week long course of fever and mental confusion. Physical examination shows T 38.2 C, P 100/minute, RR 20/minute, and BP 100/60 mm Hg. She has widespread petechiae of skin and mucosal surfaces. Laboratory studies show her serum urea nitrogen is 52 mg/dL with creatinine 5.3 mg/dL. She has a hemoglobin of 12.2 g/dL, hematocrit 36.8%, MCV 93 fL, platelet count 19,000/microliter, and WBC count 8180/microliter. Schistocytes are seen on her peripheral blood smear. Her condition deteriorates rapidly following platelet transfusion. At autopsy, pink hyaline thrombi are found in small myocardial arteries. Which of the following is the most likely diagnosis?

A Disseminated intravascular coagulopathy
B Idiopathic thrombocytopenic purpura
C Thrombotic thrombocytopenic purpura
D Trousseau syndrome
E Warm autoimmune hemolytic anemia
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Old 02-26-2011
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I like C.) TTP, there are neurological and renal symptoms, schistocytes and of course thrombocytopenia.
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Old 02-26-2011
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It is TTP/HUS secondary to sepsis.

The hyaline thrombi are typical for TTP, as is the pentad of fever, mental changes, renal failure, thrombocytopenia, and microangiopathic hemolytic anemia. Platelet transfusion is contraindicated.
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Old 02-26-2011
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Arrow C) Thrombotic thrombocytopenic purpura

My ans is C) Thrombotic thrombocytopenic purpura

Red blood cells passing the microscopic clots are subjected to shear stress which damages their membranes, leading to intravascular hemolysis and schistocyte formation.

Sx

Neurologic symptoms - mental confusion
Kidney failure - urea nitrogen is 52 mg/dL with creatinine 5.3 mg/dL
Fever - 38.2 C
Thrombocytopenia - platelet count 19,000/microliter and widespread petechiae
Microangiopathic hemolytic anemia - hemoglobin of 12.2 g/dL, hematocrit 36.8%

Contraindication : Platelet transfusion (Usually these pts will have spontaneous aggregation of platelets and activation of coagulation in the small blood vessels, once you transfuse the pts with more platelets, will lead to more clot and die.
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You all got it right .It's TTP
The classic TTP “pentad” of signs and symptoms includes
(1) thrombocytopenia, which is usually moderate or severe with an associated increase in bone marrow megakaryocytes;
(2) microangiopathic hemolytic anemia with a blood smear showing schistocyte as well as an extremely high serum lactic dehydrogenase (LDH) level;
(3) renal insufficiency, which is generally mild with moderate increases in serum creatinine and urine protein levels;
(4) fever; and
(5) neurologic abnormalities, which range from mild headache and disorientation to severe symptoms such as hemiparesis, seizures, focal neurologic deficits, coma, and death

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