Hereditary spherocytosis - Revision history

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2026-03-22T09:34:53Z

Add References

2019-09-01T14:58:07Z

Clinical Features

← Older revision		Revision as of 14:58, 1 September 2019
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	==Clinical Features==		==Clinical Features==
	*Classic triad: [[anemia, [[jaundice]], splenomegaly		*Classic triad: [[anemia]], [[jaundice]], splenomegaly

	===Mild===		===Mild===

2019-09-01T14:57:58Z

← Older revision		Revision as of 14:57, 1 September 2019
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	==Clinical Features==		==Clinical Features==
	*Classic triad: ~~Anemia~~, ~~Jaundice~~, ~~Splenomegaly~~		*Classic triad: [[anemia, [[jaundice]], splenomegaly

	===Mild===		===Mild===
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	===Severe===		===Severe===
	*Marked hemolysis, anemia, ~~hyperbili~~, splenomegaly		*Marked [[hemolytic anemia\|hemolysis]], [[anemia]], [[hyperbilirubinemia]], splenomegaly
	*Require regular RBC transfusions		*Require regular RBC transfusions
	*Dx’d in infancy		*Dx’d in infancy
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	==Management==		==Management==
	*Folic acid		*[[Folic acid]]
	*~~RBC~~ transfusion		*[[pRBC]] transfusion
	*EPO		*EPO
	*Splenectomy		*[[Splenectomy]]
	**As late as possible, preferably >6 yo		**As late as possible, preferably >6 yo
	**Post-op need encapsulated bacteria prophylaxis: Strep Pneumo, H Influenza, Neiserria Meningitidis		**Post-op need encapsulated bacteria prophylaxis: Strep Pneumo, H Influenza, Neiserria Meningitidis
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	==Complications==		==Complications==
	*Cholelithiasis secondary to intravascular hemolysis causing development of bilirubin gallstones		*[[Cholelithiasis]] secondary to intravascular hemolysis causing development of bilirubin gallstones
	*Pseudohyperkalemia: K+ leaks out of RBCs after blood draw		*Pseudohyperkalemia: K+ leaks out of RBCs after blood draw

2016-09-05T16:07:01Z

Management

← Older revision		Revision as of 16:07, 5 September 2016
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	*Splenectomy		*Splenectomy
	**As late as possible, preferably >6 yo		**As late as possible, preferably >6 yo
	**Post-op need encapsulated bacteria ~~prophy~~: Strep Pneumo, H Influenza, Neiserria Meningitidis		**Post-op need encapsulated bacteria prophylaxis: Strep Pneumo, H Influenza, Neiserria Meningitidis
	**Possible higher risk of arterial/venous thrombosis in HS after splenectomy		**Possible higher risk of arterial/venous thrombosis in HS after splenectomy
	*Hematopoietic cell transplant (most agree risks outweigh benefits)		*Hematopoietic cell transplant (most agree risks outweigh benefits)

2016-08-01T17:04:41Z

Text replacement - "Hb" to "hemoglobin"

← Older revision		Revision as of 17:04, 1 August 2016
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	==Evaluation==		==Evaluation==
	*Low Hb, elevated retic count, spherocytes on peripheral smear		*Low hemoglobin, elevated retic count, spherocytes on peripheral smear
	*Elevated MCHC (RBC membrane leaky causing RBC dehydration)		*Elevated MCHC (RBC membrane leaky causing RBC dehydration)
	*Osmotic fragility test, AGLT, EMA binding test, cryohemolysis		*Osmotic fragility test, AGLT, EMA binding test, cryohemolysis

2016-07-24T03:18:50Z

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

← Older revision		Revision as of 03:18, 24 July 2016
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	{{Hemolytic anemia DDX}}		{{Hemolytic anemia DDX}}

	==~~Diagnosis~~==		==Evaluation==
	*Low Hb, elevated retic count, spherocytes on peripheral smear		*Low Hb, elevated retic count, spherocytes on peripheral smear
	*Elevated MCHC (RBC membrane leaky causing RBC dehydration)		*Elevated MCHC (RBC membrane leaky causing RBC dehydration)

2016-07-08T10:15:25Z

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

2016-06-21T16:50:05Z

Text replacement - " pts" to " patients"

← Older revision		Revision as of 16:50, 21 June 2016
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	*Elevated MCHC (RBC membrane leaky causing RBC dehydration)		*Elevated MCHC (RBC membrane leaky causing RBC dehydration)
	*Osmotic fragility test, AGLT, EMA binding test, cryohemolysis		*Osmotic fragility test, AGLT, EMA binding test, cryohemolysis
	**One study showed AGLT + EMA binding test identified all HS ~~pts~~		**One study showed AGLT + EMA binding test identified all HS patients
	*Negative Coombs test		*Negative Coombs test

2015-06-29T12:50:18Z

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)]]
	*[[Paroxysmal nocturnal hemoglobinuria]]

	==Diagnosis==		==Diagnosis==

2015-06-29T12:49:52Z

Background

← Older revision		Revision as of 12:49, 29 June 2015
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	==Background==		==Background==
	*Hereditary ~~Spherocytosis (HS) = hereditary~~ hemolytic anemia due to defect in RBC cell membrane (mainly spectrin, ankyrin)		*Hereditary hemolytic anemia due to defect in RBC cell membrane (mainly spectrin, ankyrin)
	**75% Autosomal Dominant, 25% Autosomal Recessive		**75% Autosomal Dominant, 25% Autosomal Recessive