Clinical manifestations of paroxysmal nocturnal hemoglobinuria (PNH).
Due to intravascular hemolysis: |
Anemia, hemoglobinuria, fatigue, acute/chronic renal failure, recurrent urinary tract infection, |
Abdominal pain, bloating, back pain, headache, |
Esophagospasms, erectile dysfunction |
Cholelithiasis |
Rare: |
Choledocho dyskinesia, acute pancreatitis |
Ischemia and ulceration of duodenum or colon |
Due to thrombosis: |
Venous thrombosis: |
Abdominal vein thrombosis: Budd-Chiari, splenic, mesenteric, renal vein thrombosis |
Portal hypertension, esophageal varices, caput medusae (dilated abdominal veins) |
Cerebral vein thrombosis: headache, hemorrhagic infarct |
Retinal vein thrombosis, loss of vision |
Deep vein thrombosis, pulmonary emboli |
Rare: |
Cutaneous vein thrombosis, pyoderma gangrenosum |
Arterial thrombosis (less common): |
Stroke, myocardial infarction |
Due to bone marrow failure: |
Anemia, infections, bleeding |
Myelodysplastic syndrome |
Bone pain |
Rare: |
Transformation to acute myeloid leukemia (AML) |
Due to intravascular hemolysis: |
Anemia, hemoglobinuria, fatigue, acute/chronic renal failure, recurrent urinary tract infection, |
Abdominal pain, bloating, back pain, headache, |
Esophagospasms, erectile dysfunction |
Cholelithiasis |
Rare: |
Choledocho dyskinesia, acute pancreatitis |
Ischemia and ulceration of duodenum or colon |
Due to thrombosis: |
Venous thrombosis: |
Abdominal vein thrombosis: Budd-Chiari, splenic, mesenteric, renal vein thrombosis |
Portal hypertension, esophageal varices, caput medusae (dilated abdominal veins) |
Cerebral vein thrombosis: headache, hemorrhagic infarct |
Retinal vein thrombosis, loss of vision |
Deep vein thrombosis, pulmonary emboli |
Rare: |
Cutaneous vein thrombosis, pyoderma gangrenosum |
Arterial thrombosis (less common): |
Stroke, myocardial infarction |
Due to bone marrow failure: |
Anemia, infections, bleeding |
Myelodysplastic syndrome |
Bone pain |
Rare: |
Transformation to acute myeloid leukemia (AML) |