Pnh - High Yield Topic Series

phosphatidylinositol glycan A (PIGA) is needed to make glycosylphosphatidylinositol (GPI) anchor.

Pancytopenia with cellular marrow is seen in PNH (Paroxysmal Nocturnal Hemoglobinuria). PNH is a rare acquired genetic disorder characterized by the destruction of red blood cells, white blood cells, and platelets. This leads to a decrease in all three cell types in the blood, resulting in pancytopenia. The bone marrow, however, shows normal or increased cellularity, indicating that the problem lies in the destruction of cells rather than a decrease in production.

PNH causes INTRA vascular Hemolysis

These are the features of all Hemolytic anemias

CD55 is the correct answer because Decay-accelerating factor (DAF) is also known as CD55. CD55 is a protein that is found on the surface of cells and helps to protect them from being attacked by the body's immune system. It does this by preventing the complement system, which is part of the immune system, from damaging the cells. CD59, CD5b, and CD35 are not alternative names for Decay-accelerating factor (DAF).

Flow cytometry is a diagnostic test for PNH (Paroxysmal Nocturnal Hemoglobinuria). It is a laboratory technique that uses laser technology to analyze and measure the physical and chemical characteristics of cells in a fluid sample. In the case of PNH, flow cytometry is used to detect and quantify the presence of abnormal red blood cells that lack certain proteins on their surface, which is a characteristic feature of PNH. This test is highly sensitive and specific, making it a reliable method for diagnosing PNH. Other tests mentioned, such as the Sugar Water Test, Hams test, and FISH, may have different applications but are not specifically used for diagnosing PNH.

Patients with Paroxysmal Nocturnal Hemoglobinuria (PNH) have an increased risk of developing Acute Myeloid Leukemia (AML). PNH is a rare blood disorder characterized by the destruction of red blood cells, leading to symptoms such as anemia, fatigue, and blood clots. PNH is caused by a mutation in the PIG-A gene, which results in the deficiency of certain proteins on the surface of blood cells. This deficiency increases the risk of abnormal cell growth and the development of AML, a type of cancer that affects the bone marrow and blood. Therefore, AML is the correct answer.

The correct answer is "coomb's test positive". This means that in the case of PNH, the Coombs test, which is used to detect antibodies against red blood cells, is positive. This indicates that there is an immune-mediated destruction of red blood cells in PNH. The other statements mentioned, such as thrombotic episodes, impotence, eculizumab treatment, and refractory/aplastic anemia, are not associated with PNH.

Eculizumab is a monoclonal antibody that specifically targets the C5 protein. By binding to C5, eculizumab prevents its cleavage into C5a and C5b, which are important components of the complement system. This inhibition of C5 prevents the formation of the membrane attack complex (MAC) and the release of pro-inflammatory mediators, ultimately reducing the destruction of red blood cells in conditions such as paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS).

PNH is an ACQUIRED STEM CELL DISORDER !!