A pregnant female presents with fever. On lab investigation her Hb was decreased (7 mg%), TLC was normal and platelet count was also decreased. Peripheral smear shows fragmented RBCs. Which is least probable diagnosis?

Correct Answer: Evans syndrome
Description: The clinical scenario of the patient shows the following signs and symptoms: Fever Anemia Thrombocytopenia Normal total leukocyte count Fragmented RBCs (Schistocytes) on peripheral smear. Now let us review each option one by one Option (a): DIC Harrison 20/e, p 979 DIC may present with sudden onset of fever (as the M/c cause of D/c is sepsis) Excessive bleeding may lead to anemia Platelet consumption may lead to thrombocytopenia Leukocyte count is not affected Intravascular microangiopathic hemolysis can lead to schistocytes on peripheral smear. Williams Obs 23/e, p 786 Option (b): TTP i.e Thrombotic thrombocytopenic purpura. TTP presents with a pentad of: Fever Microangiopathic haemolytic anemia, leading to anemia and fragmentation of RBCs Thrombocytopenia Neurologic symptoms Renal failure. Option (c): HELLP syndrome HELLP syndrome presents with the combination of: Hemolysis because of which fragmented RBC’s may be seen Elevated liver enzymes and Low platelet count Fever may or may not be present. Oprion (d): Evans syndrome Hoffman: Hematology: Basic Principle and Practice, 5/e Evans syndrome is an autoimmune disease in which an individual’s antibodies attack their own red blood cells and platelets. Its overall pathology resembles a combination of autoimmune haemolytic anemia and idiopathic thrombocytopenic purpura. Autoimmune hemolysis leads to the formation of spherocytes and not schistocytes. Schistocytes are fragmented RBCs that are the result of microangiopathic hemolysis. Autoiminune destruction of RBCs leads to the formation of spherocytes. Hence, Evans syndrome is the least likely possibility in this clinical scenario.
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