Intravascular heamolysis occurs in
**Core Concept**
Intravascular hemolysis refers to the breakdown of red blood cells within the blood vessels. This process can be caused by various factors, including enzyme deficiencies, autoimmune disorders, or complement system dysregulation.
**Why the Correct Answer is Right**
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease characterized by the destruction of red blood cells within the blood vessels, leading to hemoglobinuria. This is due to the deficiency of CD55 and CD59 on the surface of red blood cells, which normally protect against complement-mediated lysis. As a result, the complement system is activated, leading to the formation of the membrane attack complex and subsequent cell lysis. PNH is also associated with bone marrow failure and thrombosis.
**Why Each Wrong Option is Incorrect**
**Option A:** Hereditary spherocytosis is a genetic disorder characterized by the production of abnormal, sphere-shaped red blood cells that are prone to premature destruction in the spleen, primarily through extravascular hemolysis.
**Option B:** Autoimmune hemolytic anemia (AIHA) is an acquired condition where the immune system produces antibodies against the red blood cells, leading to their destruction. While AIHA can cause hemolysis, it is primarily extravascular, occurring in the spleen and liver.
**Option D:** Thalassemia is a genetic disorder affecting the production of the beta-globin chains of hemoglobin, leading to anemia and hemolysis. However, the hemolysis in thalassemia is primarily extravascular, occurring in the spleen.
**Clinical Pearl / High-Yield Fact**
PNH is often diagnosed by flow cytometry, which detects the absence of CD55 and CD59 on the surface of red blood cells. Additionally, the presence of hemoglobinuria, especially in the absence of other obvious causes, should raise suspicion for PNH.
**Correct Answer:**
β Correct Answer: C. Paroxysmal nocturnal hemoglobinuria