Posts: 3,675,934
Threads: 734,342
Joined:
Sep 2021
Reputation:
5
A 37-year-old woman who has a clinical picture of fever, splenomegaly, varying neurologic manifestations, and purplish ecchymoses of the skin is found to have a hemoglobin level of 10.0 g/dL, a mean corpuscular hemoglobin concentration (MCHC) of 48, peripheral blood polychromasia with stippled macrocytes, and spherocytes, with a blood urea nitrogen level of 68 mg/dL. The findings of coagulation studies and the patient™s fibrin-degraded products are not overtly abnormal. Which of the following is most closely identified with these findings?
A. Idiopathic thrombocytopenic purpura
B. Thrombotic thrombocytopenic purpura
C. Disseminated intravascular coagulopathy
D. Submassive hepatic necrosis
E. Waterhouse-Friderichsen syndrome
Posts: 3,675,934
Threads: 734,342
Joined:
Sep 2021
Reputation:
5
what is the main difference between ITP and TTP?
Posts: 3,675,934
Threads: 734,342
Joined:
Sep 2021
Reputation:
5
TTP involves CNS, has neurologic manifestations, while ITP doesn't
Posts: 3,675,934
Threads: 734,342
Joined:
Sep 2021
Reputation:
5
Correct answer is B
Here is the explanation
A fulminating septic state should always be considered whenever the constellation of fever, deteriorating mental status, skin hemorrhages, and shock develops. Such conditions can be seen in gram-negative rod septicemia caused by any of the coliforms (gram-negative endotoxic shock) or fulminant meningococcemia (Waterhouse-Friderichsen syndrome).
However, a form of nonbacterial vasculitis termed thrombotic thrombocytopenic purpura (TTP) is notorious for producing a clinical syndrome very similar to fulminating infective states.
TTP is characterized by arteriole and capillary occlusions by fibrin and platelet microthrombi and is usually unassociated with any of the predisposing states seen in disseminated intravascular coagulopathy (DIC), such as malignancy, infection, retained fetus, and amniotic fluid embolism. Macrocytic hemolytic anemia, variable jaundice, renal failure, skin hemorrhages, and central nervous system dysfunction are all seen in TTP and are related to the fibrin thrombi, which can be demonstrated with biopsies of skin, bone marrow, and lymph node.
There is less coagulopathy in TTP than is found in DIC, and hemolytic anemia is generally not found in idiopathic or autoimmune thrombocytopenic purpura. The condition of patients with TTP may be improved by plasmapheresis, with 80% survival.
Posts: 3,675,934
Threads: 734,342
Joined:
Sep 2021
Reputation:
5
Mechanistically,
ITP-- antibody against IIb/IIIa, acute (more in children), chronic (more in 40~ women)
TTP-- deficiency of ADAMTS13 in many cases.
