Thrombotic thrombocytopenic purpura - Revision history

Danbot: Strip excess bold

2026-03-22T09:34:57Z

Strip excess bold

← Revisión anterior		Revisión del 09:34 22 mar 2026
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	*Similar clinical symptoms but different pathophysiology than [[HUS]]		*Similar clinical symptoms but different pathophysiology than [[HUS]]
	**[[HUS]] more common in pediatrics		**[[HUS]] more common in pediatrics
	*~~'''~~[[Microangiopathic Hemolytic Anemia (MAHA)]] + [[thrombocytopenia\|low Platelets]] is TTP until proven otherwise~~'''~~		*[[Microangiopathic Hemolytic Anemia (MAHA)]] + [[thrombocytopenia\|low Platelets]] is TTP until proven otherwise
	**MAHA is non-immune hemolysis (Coomb's-negative hemolysis) causing RBC fragmentation producing schistocytes		**MAHA is non-immune hemolysis (Coomb's-negative hemolysis) causing RBC fragmentation producing schistocytes
	*Can occur as a result of [[Plavix]] (clopidogrel) use, especially within the first 2 weeks		*Can occur as a result of [[Plavix]] (clopidogrel) use, especially within the first 2 weeks
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	===Risk Factors===		===Risk Factors===
	*Congenitally deficient ADAMTS-13 activity AND:		*Congenitally deficient ADAMTS-13 activity AND:
	**[[Pregnancy]] ~~'''~~OR~~'''~~		**[[Pregnancy]] OR
	**[[Infection]] ~~'''~~OR~~'''~~		**[[Infection]] OR
	**Inflammation ~~'''~~OR~~'''~~		**Inflammation OR
	**Medication use ([[quinolones]], [[ticlopidine]], [[clopidogrel]])		**Medication use ([[quinolones]], [[ticlopidine]], [[clopidogrel]])

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	#*Neuro symptoms are often transient, may not be present in ED		#*Neuro symptoms are often transient, may not be present in ED

	;TTP pentad mnemonic = ~~'''~~FAT RN~~'''~~		;TTP pentad mnemonic = FAT RN
	*Fever, Anemia, Thrombocytopenia, Renal, Neuro Symptoms		*Fever, Anemia, Thrombocytopenia, Renal, Neuro Symptoms

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	==Evaluation==		==Evaluation==
	===Workup===		===Workup===
	*CBC with peripheral smear (anemia, ~~'''~~microspherocytes~~'''~~, thrombocytopenia are suggestive findings)		*CBC with peripheral smear (anemia, microspherocytes, thrombocytopenia are suggestive findings)
	**Microangiopathic hemolytic anemia produces ~~'''~~schistocytes~~'''~~		**Microangiopathic hemolytic anemia produces schistocytes
	*LDH (elevated)		*LDH (elevated)
	*Haptoglobin (decreased)		*Haptoglobin (decreased)
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	''Management ideally done in consultation with heme/onc''<ref>George J. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood 2010; 116:4060</ref>		''Management ideally done in consultation with heme/onc''<ref>George J. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood 2010; 116:4060</ref>

	*~~'''~~[[Plasma exchange]] (plasmapheresis)~~'''~~		*[[Plasma exchange]] (plasmapheresis)
	**Replaces defective or insufficient ADAMTS-13 and clears vWF multimers		**Replaces defective or insufficient ADAMTS-13 and clears vWF multimers
	*~~'''~~[[FFP]] Transfusion~~'''~~		*[[FFP]] Transfusion
	**Contains ADAMTS-13		**Contains ADAMTS-13
	**Should only be initiated if delay in plasmapheresis		**Should only be initiated if delay in plasmapheresis
	*~~'''~~[[Glucocorticoids]]~~'''~~		*[[Glucocorticoids]]
	**Consider 1 mg/kg [[prednisone]] PO or [[methylprednisolone]] 125 mg IV<ref>Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 1991; 325:398.</ref><ref>		**Consider 1 mg/kg [[prednisone]] PO or [[methylprednisolone]] 125 mg IV<ref>Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 1991; 325:398.</ref><ref>
	Balduini CL, Gugliotta L, Luppi M, et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 2010; 89:591.</ref>		Balduini CL, Gugliotta L, Luppi M, et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 2010; 89:591.</ref>
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	***Caplacizumab may be withheld in patients with whom no ADAMTS-13 can be obtained as there are concerns for increased bleeding risk with this therapy		***Caplacizumab may be withheld in patients with whom no ADAMTS-13 can be obtained as there are concerns for increased bleeding risk with this therapy
	**In patients with low pretest probability, consider empiric plasma exchange and steroids and withhold these biologics pending ADAMTS-13 results.		**In patients with low pretest probability, consider empiric plasma exchange and steroids and withhold these biologics pending ADAMTS-13 results.
	*~~'''~~Transfusion of [[pRBCs]]~~'''~~ (only severe bleeding)		*Transfusion of [[pRBCs]] (only severe bleeding)
	**Generally only indicated if plasma exchange cannot be performed immediately		**Generally only indicated if plasma exchange cannot be performed immediately
	*~~'''~~[[Splenectomy]]~~'''~~ - 2nd line therapy after stabilization		*[[Splenectomy]] - 2nd line therapy after stabilization
	**Inhibitor antibody is made in the spleen		**Inhibitor antibody is made in the spleen
	*[[Platelet transfusion]] is ~~'''~~AVOIDED~~'''~~		*[[Platelet transfusion]] is AVOIDED
	**Only used for life-threatening bleeding or intracranial hemorrhage under guidance from hematologist		**Only used for life-threatening bleeding or intracranial hemorrhage under guidance from hematologist
	**Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death		**Platelet infusion may lead to acutely worsened thrombosis, renal failure, and death

Danbot: Add MedicationDose SMW entry for methylprednisolone/prednisone; doses verified against hematology guidelines

2026-03-20T17:58:28Z

Add MedicationDose SMW entry for methylprednisolone/prednisone; doses verified against hematology guidelines

← Revisión anterior		Revisión del 17:58 20 mar 2026
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	==Disposition==		==Disposition==
	*Admit for plasma exchange		*Admit for plasma exchange


			==Medication Dosing==
			*{{MedicationDose\|drug=Methylprednisolone\|dose=125 mg IV (or prednisone 1 mg/kg PO)\|route=IV/PO\|context=Glucocorticoid (adjunct to plasmapheresis)\|indication=Thrombotic thrombocytopenic purpura\|population=Adult}}

	==See Also==		==See Also==

Rossdonaldson1: /* Clinical Features */

2023-06-07T21:16:33Z

Clinical Features

← Revisión anterior		Revisión del 21:16 7 jun 2023
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	==Clinical Features==		==Clinical Features==
			[[File:Echymosis.jpg\|thumb\|Spontaneous bruising in a woman with critically low platelets.]]
	===Pentad (rarely all present)<ref>George J: Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927</ref>===		===Pentad (rarely all present)<ref>George J: Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927</ref>===
	#[[Microangiopathic Hemolytic Anemia (MAHA)]]		#[[Microangiopathic Hemolytic Anemia (MAHA)]]

Rossdonaldson1: /* Evaluation */

2023-06-07T21:13:51Z

Evaluation

← Revisión anterior		Revisión del 21:13 7 jun 2023
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	==Evaluation==		==Evaluation==
			===Workup===
	*CBC with peripheral smear (anemia, '''microspherocytes''', thrombocytopenia are suggestive findings)		*CBC with peripheral smear (anemia, '''microspherocytes''', thrombocytopenia are suggestive findings)
	**Microangiopathic hemolytic anemia produces '''schistocytes'''		**Microangiopathic hemolytic anemia produces '''schistocytes'''
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	*PT/PTT/INR (normal; differentiates from [[DIC]])		*PT/PTT/INR (normal; differentiates from [[DIC]])
	*Urine pregnancy (significant association between pregnancy and TTP)		*Urine pregnancy (significant association between pregnancy and TTP)

			===Diagnosis===
	*Plasma ADAMTS-13 Level:		*Plasma ADAMTS-13 Level:
	**Ideally drawn prior to initiation of treatment, but results rarely available in the ER.		**Ideally drawn prior to initiation of treatment, but results rarely available in the ER.

Rossdonaldson1: /* Clinical FeaturesGeorge J: Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927 */

2023-05-31T18:06:54Z

Clinical FeaturesGeorge J: Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927

← Revisión anterior		Revisión del 18:06 31 may 2023
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	**Medication use ([[quinolones]], [[ticlopidine]], [[clopidogrel]])		**Medication use ([[quinolones]], [[ticlopidine]], [[clopidogrel]])

	==Clinical Features<ref>George J: Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927</ref>==		==Clinical Features==
	~~===Pentad (rarely all present)~~===		===Pentad (rarely all present)<ref>George J: Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med 2006; 354:1927</ref>===
	#[[Microangiopathic Hemolytic Anemia (MAHA)]]		#[[Microangiopathic Hemolytic Anemia (MAHA)]]
	#[[Thrombocytopenia]]		#[[Thrombocytopenia]]

Athorne: /* Management */

2023-05-20T08:46:53Z

Management

← Revisión anterior		Revisión del 08:46 20 may 2023
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	**Consider 1 mg/kg [[prednisone]] PO or [[methylprednisolone]] 125 mg IV<ref>Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 1991; 325:398.</ref><ref>		**Consider 1 mg/kg [[prednisone]] PO or [[methylprednisolone]] 125 mg IV<ref>Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 1991; 325:398.</ref><ref>
	Balduini CL, Gugliotta L, Luppi M, et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 2010; 89:591.</ref>		Balduini CL, Gugliotta L, Luppi M, et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 2010; 89:591.</ref>
	*'''Biologic Therapies:''' ISTH guidelines now recommend adding both empiric rituximab and caplacizumab for patients with high pretest probability while awaiting ADAMTS-13 levels.		*'''Biologic Therapies:''' ISTH guidelines now recommend adding both empiric rituximab and caplacizumab for patients with high pretest probability while awaiting ADAMTS-13 levels.<ref>Zheng XL, Vesely SK, Cataland SR, Coppo P, Geldziler B, Iorio A, Matsumoto M, Mustafa RA, Pai M, Rock G, Russell L, Tarawneh R, Valdes J, Peyvandi F. ISTH guidelines for treatment of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020 Oct;18(10):2496-2502. doi: 10.1111/jth.15010. Epub 2020 Sep 11. PMID: 32914526; PMCID: PMC8091490.</ref>
	**Rituximab is a monoclonal antibody against CD20 and is used in many autoimmune disorders		**Rituximab is a monoclonal antibody against CD20 and is used in many autoimmune disorders
	**Caplacizumab is a monoclonal antibody blocking platelet-vWF interactions and thus formation of microvascular thrombosis.		**Caplacizumab is a monoclonal antibody blocking platelet-vWF interactions and thus formation of microvascular thrombosis.

Athorne: /* Management */

2023-05-20T08:45:01Z

Management

← Revisión anterior		Revisión del 08:45 20 may 2023
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	*'''[[Plasma exchange]] (plasmapheresis)'''		*'''[[Plasma exchange]] (plasmapheresis)'''
	**Replaces defective or insufficient ADAMTS-13 and clears vWF multimers		**Replaces defective or insufficient ADAMTS-13 and clears vWF multimers
	*'''Transfusion of [[pRBCs]]''' (only severe bleeding)
	**Generally only indicated if plasma exchange cannot be performed immediately
	*'''[[FFP]] Transfusion'''		*'''[[FFP]] Transfusion'''
	**Contains ADAMTS-13		**Contains ADAMTS-13
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	**Consider 1 mg/kg [[prednisone]] PO or [[methylprednisolone]] 125 mg IV<ref>Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 1991; 325:398.</ref><ref>		**Consider 1 mg/kg [[prednisone]] PO or [[methylprednisolone]] 125 mg IV<ref>Bell WR, Braine HG, Ness PM, Kickler TS. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med 1991; 325:398.</ref><ref>
	Balduini CL, Gugliotta L, Luppi M, et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 2010; 89:591.</ref>		Balduini CL, Gugliotta L, Luppi M, et al. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study. Ann Hematol 2010; 89:591.</ref>
			*'''Biologic Therapies:''' ISTH guidelines now recommend adding both empiric rituximab and caplacizumab for patients with high pretest probability while awaiting ADAMTS-13 levels.
			**Rituximab is a monoclonal antibody against CD20 and is used in many autoimmune disorders
			**Caplacizumab is a monoclonal antibody blocking platelet-vWF interactions and thus formation of microvascular thrombosis.
			***Caplacizumab may be withheld in patients with whom no ADAMTS-13 can be obtained as there are concerns for increased bleeding risk with this therapy
			**In patients with low pretest probability, consider empiric plasma exchange and steroids and withhold these biologics pending ADAMTS-13 results.
			*'''Transfusion of [[pRBCs]]''' (only severe bleeding)
			**Generally only indicated if plasma exchange cannot be performed immediately
	*'''[[Splenectomy]]''' - 2nd line therapy after stabilization		*'''[[Splenectomy]]''' - 2nd line therapy after stabilization
	**Inhibitor antibody is made in the spleen		**Inhibitor antibody is made in the spleen

Athorne: /* Evaluation */

2023-05-20T08:38:15Z

Evaluation

← Revisión anterior		Revisión del 08:38 20 may 2023
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	***>20 IU/dL within normal, consider other diagnoses		***>20 IU/dL within normal, consider other diagnoses

	*PLASMIC Score: <5 features effectively rules out TTP (99% NPV)<ref>Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta-analysis. Transfusion. 2020 Sep;60(9):2047-2057. doi: 10.1111/trf.15954. Epub 2020 Aug 5. PMID: 32757237.<~~ref><~~ref>		*PLASMIC Score: <5 features effectively rules out TTP (99% NPV)<ref>Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta-analysis. Transfusion. 2020 Sep;60(9):2047-2057. doi: 10.1111/trf.15954. Epub 2020 Aug 5. PMID: 32757237.</ref>
	**Platelets <30 x 10<sup>9</sup>/L		**Platelets <30 x 10<sup>9</sup>/L
	**Hemolysis with reticulocye count >2.5%, indirect bilirubin >2mg/dL, or low haptoglobin		**Hemolysis with reticulocye count >2.5%, indirect bilirubin >2mg/dL, or low haptoglobin

Athorne: /* Evaluation */

2023-05-20T07:34:27Z

Evaluation

← Revisión anterior		Revisión del 07:34 20 may 2023
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	***>20 IU/dL within normal, consider other diagnoses		***>20 IU/dL within normal, consider other diagnoses

	*PLASMIC Score: <5 features effectively rules out TTP (99% NPV) <ref>Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta-analysis. Transfusion. 2020 Sep;60(9):2047-2057. doi: 10.1111/trf.15954. Epub 2020 Aug 5. PMID: 32757237.<ref>		*PLASMIC Score: <5 features effectively rules out TTP (99% NPV)<ref>Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta-analysis. Transfusion. 2020 Sep;60(9):2047-2057. doi: 10.1111/trf.15954. Epub 2020 Aug 5. PMID: 32757237.<ref><ref>
	**Platelets <30 x 10<sup>9</sup>/L		**Platelets <30 x 10<sup>9</sup>/L
	**Hemolysis with reticulocye count >2.5%, indirect bilirubin >2mg/dL, or low haptoglobin		**Hemolysis with reticulocye count >2.5%, indirect bilirubin >2mg/dL, or low haptoglobin

Athorne: /* Evaluation */

2023-05-20T07:33:23Z

Evaluation

← Revisión anterior		Revisión del 07:33 20 may 2023
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	***>20 IU/dL within normal, consider other diagnoses		***>20 IU/dL within normal, consider other diagnoses

	*PLASMIC Score: <5 features effectively rules out TTP (99% NPV)		*PLASMIC Score: <5 features effectively rules out TTP (99% NPV) <ref>Paydary K, Banwell E, Tong J, Chen Y, Cuker A. Diagnostic accuracy of the PLASMIC score in patients with suspected thrombotic thrombocytopenic purpura: A systematic review and meta-analysis. Transfusion. 2020 Sep;60(9):2047-2057. doi: 10.1111/trf.15954. Epub 2020 Aug 5. PMID: 32757237.<ref>
	**Platelets <30 x 10<sup>9</sup>/L		**Platelets <30 x 10<sup>9</sup>/L
	**Hemolysis with reticulocye count >2.5%, indirect bilirubin >2mg/dL, or low haptoglobin		**Hemolysis with reticulocye count >2.5%, indirect bilirubin >2mg/dL, or low haptoglobin