Hemophilia - Revision history

Danbot: Add MedicationDose entry (desmopressin) with SMW annotations

2026-03-20T18:54:13Z

Add MedicationDose entry (desmopressin) with SMW annotations

@@ Line 159: / Line 159: @@
 **270 mcg/kg dosing
 **Separate further dosing by at least 6 hours
 ==Disposition==

Elcatracho: /* Background */

2021-02-09T03:35:07Z

Background

← Older revision		Revision as of 03:35, 9 February 2021
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	*Two types (clinically indistinguishable):		*Two types (clinically indistinguishable):
	**Hemophilia A: Factor VIII deficiency		**Hemophilia A: Factor VIII deficiency
	**Hemophilia B: Factor IX deficiency		**Hemophilia B (Christmas disease): Factor IX deficiency
	*Substantial proportion (~1/3) of both types arise from spontaneous mutations		*Substantial proportion (~1/3) of both types arise from spontaneous mutations
	*Most common X-linked disorder<ref>[http://emedicine.medscape.com/article/779322-overview#a1 Medscape: Hemophilia A]</ref>(overwhelmingly a disease of men)		*Most common X-linked disorder<ref>[http://emedicine.medscape.com/article/779322-overview#a1 Medscape: Hemophilia A]</ref>(overwhelmingly a disease of men)

Josgood: /* Desired Level RepletionDiMichele D. Hemophilia 1996. New approach to an old disease. Pediatr Clin North Am. 1996 Jun;43(3): 709-36. */

2021-01-25T18:20:00Z

Desired Level RepletionDiMichele D. Hemophilia 1996. New approach to an old disease. Pediatr Clin North Am. 1996 Jun;43(3): 709-36.

← Older revision		Revision as of 18:20, 25 January 2021
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	\|-		\|-
	\|}		\|}
	*For isolated oral mucosal bleeding consider antifibrinolytic agents		*For isolated oral mucosal bleeding consider antifibrinolytic agents (e.g. - [[TXA]])

	===Inhibitors to Factors===		===Inhibitors to Factors===

Elcatracho: /* TreatmentBitting RL, Bent S, Li Y, Kohlwes J. The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis. Blood Coagul Fibrinolysis. Oct 2009;20(7):517-23 */

2021-01-11T02:58:53Z

TreatmentBitting RL, Bent S, Li Y, Kohlwes J. The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis. Blood Coagul Fibrinolysis. Oct 2009;20(7):517-23

← Older revision		Revision as of 02:58, 11 January 2021
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	*Always inquire whether patient has known inhibitors - may be refractory to conventional treatment		*Always inquire whether patient has known inhibitors - may be refractory to conventional treatment
	**If so, obtain hematology consult before treatment		**If so, obtain hematology consult before treatment
	**If no ~~know~~ inhibitors, and patient not improving after replacement, order mixing study		**If no known inhibitors, and patient not improving after replacement, order mixing study
	***PTT will not correct if inhibitors present		***PTT will not correct if inhibitors present

ClaireLewis: /* Consider */

2019-10-01T23:46:35Z

Consider

← Older revision		Revision as of 23:46, 1 October 2019
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	*[[Head CT]]		*[[Head CT]]
	**If [[headache]], [[altered mental status]], blunt [[head trauma\|head injury]]		**If [[headache]], [[altered mental status]], blunt [[head trauma\|head injury]]
	**MRI is preferred to CT if available (better visualization of posterior fossa)		**[[Brain MRI\|MRI]] is preferred to CT if available (better visualization of posterior fossa)
	*CT A/P		*CT A/P
	**Back, thigh, groin, or abdominal pain		**Back, thigh, groin, or abdominal pain

ClaireLewis at 00:55, 1 October 2019

2019-10-01T00:55:19Z

← Older revision		Revision as of 00:55, 1 October 2019
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	*Substantial proportion (~1/3) of both types arise from spontaneous mutations		*Substantial proportion (~1/3) of both types arise from spontaneous mutations
	*Most common X-linked disorder<ref>[http://emedicine.medscape.com/article/779322-overview#a1 Medscape: Hemophilia A]</ref>(overwhelmingly a disease of men)		*Most common X-linked disorder<ref>[http://emedicine.medscape.com/article/779322-overview#a1 Medscape: Hemophilia A]</ref>(overwhelmingly a disease of men)
	*ICH is most common cause of hemorrhagic death		*[[ICH]] is most common cause of hemorrhagic death
	*Do not give NSAIDs or IM injections		*Do ''not'' give [[NSAIDs]] or IM injections
	*Avoid invasive procedures (e.g. central lines, LP)		*Avoid invasive procedures (e.g. central lines, LP)

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	**Neck (airway compromise)		**Neck (airway compromise)
	**Limbs (compartment syndromes)		**Limbs (compartment syndromes)
	**Eye (retro-orbital hematoma)		**Eye ([[retrobulbar hematoma\|retro-orbital hematoma]])
	**Spine (epidural hematoma)		**Spine ([[epidural hematoma]])
	**Retroperitoneum (iliopsoas bleeds and massive blood loss)		**[[retroperitoneal bleed\|Retroperitoneum]] (iliopsoas bleeds and massive blood loss)
	*Most common initial complaint		*Most common initial complaint
	===Mucocutaneous bleeding===		===Mucocutaneous bleeding===
	*Spontaneous bleeding uncommon from oropharynx, GI tract, epistaxis, or hemoptysis		*Spontaneous bleeding uncommon from oropharynx, [[GI bleed\|GI tract]], [[epistaxis]], or [[hemoptysis]]
	===CNS===		===CNS===
	*Intracranial bleeding is most common cause of hemorrhagic death		*[[ICH\|Intracranial bleeding]] is most common cause of hemorrhagic death
	*Subdural ~~hematomas~~ occur spontaneously or with minimal trauma		*[[Subdural hematoma]]s occur spontaneously or with minimal trauma
	===Hematuria===		===[[Hematuria]]===
	*Common, usually not serious, source is rarely found		*Common, usually not serious, source is rarely found

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	====Consider====		====Consider====
	*Head CT		*[[Head CT]]
	**If headache, altered mental status, ~~significant~~ blunt head injury		**If [[headache]], [[altered mental status]], blunt [[head trauma\|head injury]]
	**MRI is preferred to CT if available (better visualization of posterior fossa)		**MRI is preferred to CT if available (better visualization of posterior fossa)
	*CT A/P		*CT A/P
	**Back, thigh, groin, or abdominal pain		**Back, thigh, groin, or abdominal pain
	*[[LP]]		*If [[LP]] required
	**replete factor before attempting		**replete factor before attempting

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	===Indications for Factor Replacement===		===Indications for Factor Replacement===
	* Suspected bleeding into muscles or joints		* Suspected bleeding into muscles or joints
	* Trauma to head or neck		* Trauma to [[head trauma\|head]] or [[neck trauma\|neck]]
	* Any new or concerning headache (spontaneous or traumatic)		* Any new or concerning [[headache]] (spontaneous or traumatic)
	* Trauma with potential for internal bleeding		* [[Trauma]] with potential for internal bleeding
	* Prior to any planned invasive procedure or surgery		* Prior to any planned invasive procedure or surgery
	* Gastrointestinal bleeding		* [[GI bleed\|Gastrointestinal]] bleeding
	* Acute fractures, dislocations and sprains		* Acute [[fractures]], [[joint dislocations\|dislocations]] and sprains

	===Factor Replacement===		===Factor Replacement===
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	*Moderate bleeding (soft tissue, small muscle, joint) requires 30-50%		*Moderate bleeding (soft tissue, small muscle, joint) requires 30-50%
	*Diagnosis unknown		*Diagnosis unknown
	**Give FFP (contains VIII and IX)		**Give [[FFP]] (contains VIII and IX)
	**Each bag raises factor levels by 3-5%		**Each bag raises factor levels by 3-5%
	*Severe mechanism of injury (head, neck trauma), even without evidence of bleeding		*Severe mechanism of injury (head, neck trauma), even without evidence of bleeding
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	**For severe bleeding, desired factor level is 80-100%.		**For severe bleeding, desired factor level is 80-100%.
	**For less severe bleeding, the desired factor level is 30-50%		**For less severe bleeding, the desired factor level is 30-50%
	*Desmopressin		*[[Desmopressin]]
	**May be sufficient in patients with mild bleeding		**May be sufficient in patients with mild bleeding
	**0.3mcg/kg IV over 15-30min		**0.3mcg/kg IV over 15-30min

Aganapathy: /* TreatmentBitting RL, Bent S, Li Y, Kohlwes J. The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis. Blood Coagul Fibrinolysis. Oct 2009;20(7):517-23 */

2017-09-18T20:19:07Z

TreatmentBitting RL, Bent S, Li Y, Kohlwes J. The prognosis and treatment of acquired hemophilia: a systematic review and meta-analysis. Blood Coagul Fibrinolysis. Oct 2009;20(7):517-23

← Older revision		Revision as of 20:19, 18 September 2017
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	**If no know inhibitors, and patient not improving after replacement, order mixing study		**If no know inhibitors, and patient not improving after replacement, order mixing study
	***PTT will not correct if inhibitors present		***PTT will not correct if inhibitors present

			===Indications for Factor Replacement===
			* Suspected bleeding into muscles or joints
			* Trauma to head or neck
			* Any new or concerning headache (spontaneous or traumatic)
			* Trauma with potential for internal bleeding
			* Prior to any planned invasive procedure or surgery
			* Gastrointestinal bleeding
			* Acute fractures, dislocations and sprains

	===Factor Replacement===		===Factor Replacement===
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	*Patients requiring multiple factor replacement doses		*Patients requiring multiple factor replacement doses
	*Bleeding in head, neck, pharynx, retropharynx, or retroperitoneum		*Bleeding in head, neck, pharynx, retropharynx, or retroperitoneum
			*Potential for compartment syndrome
	===Discharge===		===Discharge===
	*If close follow-up and mild hemarthrosis		*If close follow-up and mild hemarthrosis

Alvarez13 at 23:34, 26 November 2016

2016-11-26T23:34:10Z

← Older revision		Revision as of 23:34, 26 November 2016
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	==See Also==		==See Also==
	*[[Coagulopathy (Main)]]		*[[Coagulopathy (Main)]]
			*[[Von Willebrand Disease (vWD)]]

	==References==		==References==

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

2016-08-03T20:46:33Z

Text replacement - " ==" to "=="

← Older revision		Revision as of 20:46, 3 August 2016
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	==Background ==		==Background==
	*TREAT FIRST, Diagnose second. Assume bleeding until proven otherwise.		*TREAT FIRST, Diagnose second. Assume bleeding until proven otherwise.
	*Two types (clinically indistinguishable):		*Two types (clinically indistinguishable):
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	*For hemarthrosis; elevate, ice, and protect joint (crutches for knee/ankle)		*For hemarthrosis; elevate, ice, and protect joint (crutches for knee/ankle)

	==Clinical Features ==		==Clinical Features==
	''Patient does not need objective exam finding to treat. Subjective complaints are a harbinger of serious issues.''		''Patient does not need objective exam finding to treat. Subjective complaints are a harbinger of serious issues.''
	===Hemarthroses===		===Hemarthroses===
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	==Evaluation==		==Evaluation==
	===Work-Up ===		===Work-Up===
	*Coags		*Coags
	**Only helpful in making the diagnosis; once established unlikely to yield new information		**Only helpful in making the diagnosis; once established unlikely to yield new information
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	***PTT will not correct if inhibitors present		***PTT will not correct if inhibitors present

	===Factor Replacement ===		===Factor Replacement===
	*Major bleeding (GI, CNS, large muscle, trauma) requires factor replacement level 80-100%		*Major bleeding (GI, CNS, large muscle, trauma) requires factor replacement level 80-100%
	*Moderate bleeding (soft tissue, small muscle, joint) requires 30-50%		*Moderate bleeding (soft tissue, small muscle, joint) requires 30-50%
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	*[[Coagulopathy (Main)]]		*[[Coagulopathy (Main)]]

	==References ==		==References==
	<references/>		<references/>

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

Rossdonaldson1: Text replacement - "AMS" to "altered mental status"

2016-07-31T00:52:17Z

Text replacement - "AMS" to "altered mental status"

← Older revision		Revision as of 00:52, 31 July 2016
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	====Consider====		====Consider====
	*Head CT		*Head CT
	**If headache, ~~AMS~~, significant blunt head injury		**If headache, altered mental status, significant blunt head injury
	**MRI is preferred to CT if available (better visualization of posterior fossa)		**MRI is preferred to CT if available (better visualization of posterior fossa)
	*CT A/P		*CT A/P