Question

Patient with hemolytic anemia, cytopenias, and hypercoagulable state (i.e. hepatic vein thrombosis)? Dx? pathophys?

a) Paroxysmal nocturnal hemoglobinuria (PNH); Intrinsic defect in the complement system
b) Hereditary spherocytosis; RBC membrane defect
c) Sickle cell disease; Abnormal hemoglobin structure
d) Thalassemia; Abnormal hemoglobin synthesis

Answer 1

Paroxysmal Nocturnal Hemoglobinuria (PNH) is the likely diagnosis for this patient. PNH is characterized by hemolytic anemia, cytopenias, and a hypercoagulable state.

Step-by-step explanation:

The patient in this case has a condition characterized by hemolytic anemia, cytopenias, and a hypercoagulable state, specifically hepatic vein thrombosis. This presentation is consistent with Paroxysmal Nocturnal Hemoglobinuria (PNH).

In PNH, there is an intrinsic defect in the complement system, which leads to the destruction of red blood cells by the complement system. The excessive hemolysis can cause anemia, as well as other complications such as cytopenias and hypercoagulability.