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Paroxysmal nocturnal hemoglobinuria (PNH) is chronic intravascular hemolysis caused by an acquired RBC-membrane defect; it often worsens during sleep and may present with episodic hemoglobinuria and pancytopenia. Though rare, incidence has been increasing recently; it is more common in northern China.
Hemoglobinuria; anemia; pancytopenia; abdominal pain; thrombosis; fatigue
Acquired PIG-A gene mutation in hematopoietic stem cells causes deficiency of GPI-anchored complement-regulatory proteins (CD55, CD59) on blood-cell membranes, making RBCs abnormally sensitive to complement lysis.
Eculizumab; bone-marrow transplant; supportive care; transfusion; anticoagulation
Monitor thrombosis risk; treat with complement inhibitor; vaccination; stem-cell transplant for severe cases.
It is recommended to visit the Hematology department; an appointment can be booked on this platform before the visit.
Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.
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