Pathology - Paroxysmal Nocturnal Hemoglobinuria (PNH)
Resulting from a genetic alteration on the X chromosome. This leads to the incapacity of hematopoietic stem cells to generate a GPI surface protein, which plays a crucial role in interacting with complement molecules. This results in flawed interactions with complement, leading to the destruction of the erythrocyte by complement. Pathology Peripheral blood smear reveals indications of hemolytic anemia. Patients exhibit intermittent reddish-brown urine in the mornings as a result of elevated levels of urine hemosiderin. Indications of hemolytic anemia and blood clotting in veins. Symptoms and signs Additionally, certain people may exhibit infections and bleeding as a result of a pancytopenic condition. Laboratory results: The results indicate a positive Ham test, positive urine hemosiderin, negative Coombs test, and flow cytometry showing the absence of CD55 or CD59, which are both GPI surface proteins. Therapy Supportive therapy include transfusions and iron supplementation as necessary; corticosteroids for mild cases, while severe cases may necessitate a bone marrow transplant or immunosuppression using eculizumab.
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