Match each clinical presentation with its most likely underlying pathophysiological mechanism.
- A 35-year-old woman with fever, confusion, schistocytes on peripheral smear, and normal coagulation timesSeverely reduced ADAMTS13 metalloprotease activity
- A 65-year-old man with massive splenomegaly, fatigue, and teardrop red blood cells on peripheral smearMegakaryocyte proliferation causing marrow collagen fibrosis
- A 70-year-old man with painless cervical lymphadenopathy, marked lymphocytosis, and smudge cells on smearMonoclonal expansion of CD5-positive mature B lymphocytes
- A 28-year-old man developing jaundice and dark urine after trimethoprim-sulfamethoxazole use, with bite cellsDefective generation of reduced glutathione in red blood cells
- A 45-year-old woman with recurrent deep vein thromboses, pregnancy losses, and a prolonged PTTAutoantibodies directed against phospholipid-binding proteins
Answer
Each clinical presentation is matched to its corresponding pathognomonic mechanism.
Each clinical vignette provides pathognomonic clues for a specific hematologic disorder. TTP is caused by ADAMTS13 deficiency; Primary Myelofibrosis involves marrow fibrosis from abnormal megakaryocytes; CLL is a CD5+ B cell neoplasm; G6PD deficiency involves defective generation of reduced glutathione; and Antiphospholipid Syndrome is driven by autoantibodies against phospholipid-binding proteins.
Step-by-Step Solution
Key Concept
Pathophysiological mechanisms of classic hematologic and oncologic disorders based on their peripheral smear and clinical presentations.