Paroxysmal nocturnal hemoglobinuria - Revision history

Danbot: Add References

2026-03-22T09:35:00Z

Add References

← Older revision		Revision as of 09:35, 22 March 2026
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	==See Also==		==See Also==


			==References==
			<references/>

	[[Category:Heme/Onc]]		[[Category:Heme/Onc]]

ClaireLewis at 17:21, 1 October 2019

2019-10-01T17:21:19Z

← Older revision		Revision as of 17:21, 1 October 2019
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	==Background==		==Background==
	*Intravascular hemolysis caused by increased sensitivity of RBCs to hemolytic action of complement		*Intravascular [[hemolytic anemia\|hemolysis]] caused by increased sensitivity of RBCs to hemolytic action of complement

	===Pathophysiology===		===Pathophysiology===
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	==Clinical Features==		==Clinical Features==
	*[[Abdominal pain]] or [[back pain]]		*[[Abdominal pain]] or [[back pain]]
	*Intravascular hemolysis		*Intravascular [[hemolytic anemia\|hemolysis]]
	*[[Renal failure]] (both from intravascular hemolysis causing AKI and chronic hemolysis causing iron deposition in kidneys)		*[[Renal failure]] (both from intravascular hemolysis causing AKI and chronic hemolysis causing iron deposition in kidneys)
	*[[DVT]], thrombosis of hepatic/intraabdominal/cerebral veins, arterial thrombosis		*[[DVT]], thrombosis of hepatic/intraabdominal/cerebral veins, [[arterial thrombosis]]
	*Bone marrow failure leading to aplastic anemia		*Bone marrow failure leading to [[aplastic anemia]]
	*[[Myelodysplastic syndrome]] (MDS) or [[myeloproliferative disorders]]		*[[Myelodysplastic syndrome]] (MDS) or [[myeloproliferative disorders]]
	*[[Acute leukemia]]		*[[Acute leukemia]]
	*Esophageal spasm		*Esophageal spasm
	*Crampy abdominal pain		*Crampy [[abdominal pain]]
	*Impotence/ED		*Impotence/erectile dysfunction
	*May progress to [[aplastic anemia]]		*May progress to [[aplastic anemia]]

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	==Evaluation==		==Evaluation==
	*Anemia		*[[Anemia]]
	*Negative Coombs test		*Negative Coombs test
	*Hemoglobinuria		*Hemoglobinuria
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	==Management==		==Management==
	*Eculizumab (C5 antibody): stops hemolysis in PNH		*Eculizumab (C5 antibody): stops hemolysis in PNH
	*Iron/folate supplements		*[[iron supplementation\|Iron]]/[[folate]] supplements
	*RBC transfusion PRN		*[[pRBCs\|RBC transfusion]] PRN
	*Prednisone may decrease RBC destruction		*[[Prednisone]] may decrease RBC destruction
	*For severe hematopoietic dysfunction with marked cytopenias: hematopoietic cell transplant or immunosuppression (cyclosporine or antithymocyte globulin)		*For severe hematopoietic dysfunction with marked cytopenias: hematopoietic cell transplant or immunosuppression (cyclosporine or antithymocyte globulin)
	*Anticoagulation for thrombosis		*[[Anticoagulation]] for thrombosis

	==Disposition==		==Disposition==

ClaireLewis: /* Clinical Features */

2016-10-05T04:57:03Z

Clinical Features

← Older revision		Revision as of 04:57, 5 October 2016
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	==Clinical Features==		==Clinical Features==
	*Abdominal or back pain		*[[Abdominal pain]] or [[back pain]]
	*Intravascular hemolysis		*Intravascular hemolysis
	*Renal failure (both from intravascular hemolysis causing AKI and chronic hemolysis causing iron deposition in kidneys)		*[[Renal failure]] (both from intravascular hemolysis causing AKI and chronic hemolysis causing iron deposition in kidneys)
	*DVT, thrombosis of hepatic/intraabdominal/cerebral veins, arterial thrombosis		*[[DVT]], thrombosis of hepatic/intraabdominal/cerebral veins, arterial thrombosis
	*Bone marrow failure leading to aplastic anemia		*Bone marrow failure leading to aplastic anemia
	*Myelodysplastic (MDS) or myeloproliferative disorders		*[[Myelodysplastic syndrome]] (MDS) or [[myeloproliferative disorders]]
	*Acute leukemia		*[[Acute leukemia]]
	*Esophageal spasm		*Esophageal spasm
	*Crampy abdominal pain		*Crampy abdominal pain
	*Impotence/ED		*Impotence/ED
	*May progress to aplastic anemia		*May progress to [[aplastic anemia]]

	==Differential Diagnosis==		==Differential Diagnosis==

Rossdonaldson1: /* Clinical Features */

2016-09-08T11:21:46Z

Clinical Features

← Older revision		Revision as of 11:21, 8 September 2016
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	*Esophageal spasm		*Esophageal spasm
	*Crampy abdominal pain		*Crampy abdominal pain
	*Impotence/ED~~<br />~~		*Impotence/ED
	*May progress to aplastic anemia		*May progress to aplastic anemia

AnthonyLucero: Added evaluation bullets

2016-09-02T04:11:20Z

Added evaluation bullets

← Older revision		Revision as of 04:11, 2 September 2016
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	*Elevated LDH		*Elevated LDH
	*Decreased haptoglobin		*Decreased haptoglobin
	*Positive sucrose lysis test or Ham test (both test sensitivity of RBCs to lysis by complement)		*Positive sucrose lysis test or Ham test (both test sensitivity of RBCs to lysis by complement). Ham's test is when the patient's cells are incubated in acidified serum, which triggers the alternative compliment pathway, resulting in lysis of PNH cells. Sucrose lysis test is when the patient's serum is mixed in sucrose, which causes hemolysis of PNH cells.
	*Other clues: granulocytopenia/thrombocytopenia, venous thrombosis, aplastic anemia, MDS, dysphagia or abdominal pain		*Other clues: granulocytopenia/thrombocytopenia, venous thrombosis, aplastic anemia, MDS, dysphagia or abdominal pain
			*Flow cytometry of cell membrane proteins (CD55 and CD59) is a sensitive test for PNH

	==Management==		==Management==

AnthonyLucero: Added clinical features

2016-09-02T04:05:31Z

Added clinical features

← Older revision		Revision as of 04:05, 2 September 2016
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	==Clinical Features==		==Clinical Features==
			*Abdominal or back pain
	*Intravascular hemolysis		*Intravascular hemolysis
	*Renal failure (both from intravascular hemolysis causing AKI and chronic hemolysis causing iron deposition in kidneys)		*Renal failure (both from intravascular hemolysis causing AKI and chronic hemolysis causing iron deposition in kidneys)
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	*Esophageal spasm		*Esophageal spasm
	*Crampy abdominal pain		*Crampy abdominal pain
	*Impotence/ED		*Impotence/ED<br />
			*May progress to aplastic anemia

	==Differential Diagnosis==		==Differential Diagnosis==

Neil.m.young: Text replacement - "==Diagnosis==" to "==Evaluation=="

2016-07-24T19:26:17Z

Text replacement - "==Diagnosis==" to "==Evaluation=="

← Older revision		Revision as of 19:26, 24 July 2016
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	{{Hemolytic anemia DDX}}		{{Hemolytic anemia DDX}}

	==~~Diagnosis~~==		==Evaluation==
	*Anemia		*Anemia
	*Negative Coombs test		*Negative Coombs test

Neil.m.young: Text replacement - " w/ " to " with "

2016-07-12T04:52:29Z

Text replacement - " w/ " to " with "

← Older revision		Revision as of 04:52, 12 July 2016
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	*RBC transfusion PRN		*RBC transfusion PRN
	*Prednisone may decrease RBC destruction		*Prednisone may decrease RBC destruction
	*For severe hematopoietic dysfunction w/ marked cytopenias: hematopoietic cell transplant or immunosuppression (cyclosporine or antithymocyte globulin)		*For severe hematopoietic dysfunction with marked cytopenias: hematopoietic cell transplant or immunosuppression (cyclosporine or antithymocyte globulin)
	*Anticoagulation for thrombosis		*Anticoagulation for thrombosis

Neil.m.young: Text replacement - "==Treatment==" to "==Management=="

2016-07-08T01:42:30Z

Text replacement - "==Treatment==" to "==Management=="

← Older revision		Revision as of 01:42, 8 July 2016
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	*Other clues: granulocytopenia/thrombocytopenia, venous thrombosis, aplastic anemia, MDS, dysphagia or abdominal pain		*Other clues: granulocytopenia/thrombocytopenia, venous thrombosis, aplastic anemia, MDS, dysphagia or abdominal pain

	==~~Treatment~~==		==Management==
	*Eculizumab (C5 antibody): stops hemolysis in PNH		*Eculizumab (C5 antibody): stops hemolysis in PNH
	*Iron/folate supplements		*Iron/folate supplements

Rossdonaldson1: /* Differential Diagnosis */

2015-06-29T12:50:46Z

Differential Diagnosis

← Older revision		Revision as of 12:50, 29 June 2015
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	==Differential Diagnosis==		==Differential Diagnosis==
	*[[DIC]]		{{Hemolytic anemia DDX}}
	*[[TTP]]
	*[[HUS]]
	*[[Microangiopathic Hemolytic ~~Anemia (MAHA)]]~~
	*[[HELLP]]
	*[[HIT]]
	*[[Hereditary Spherocytosis (HS)]]

	==Diagnosis==		==Diagnosis==