Hemophagocytic lymphohistiocytosis - Revision history

Aidan at 17:11, 14 November 2025

2025-11-14T17:11:45Z

← Older revision		Revision as of 17:11, 14 November 2025
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	*** [[SARS-CoV-2]], possibly		*** [[SARS-CoV-2]], possibly
	** After anti-TNF therapy: [[tuberculosis]], [[CMV]], [[EBV]], or [[histoplasmosis]]		** After anti-TNF therapy: [[tuberculosis]], [[CMV]], [[EBV]], or [[histoplasmosis]]
	** Rare: [[Brucella]], gram-negative bacilli, [[tuberculosis]], [[~~Leishmaniasis~~]], [[malaria]], and fungal infections		** Rare: [[Brucella]], gram-negative bacilli, [[tuberculosis]], [[leishmaniasis]], [[malaria]], and fungal infections
	* Malignancy, most commonly lymphoid cancers and leukemias, but also solid cancers		* Malignancy, most commonly lymphoid cancers and leukemias, but also solid cancers
	* Rheumatologic disease		* Rheumatologic disease

Aidan: /* Differential Diagnosis */

2023-06-07T16:28:23Z

Differential Diagnosis

← Older revision		Revision as of 16:28, 7 June 2023
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	== Differential Diagnosis ==		== Differential Diagnosis ==

	* Macrophage activation syndrome		* [[Macrophage activation syndrome]]
	* Severe sepsis		* Severe [[sepsis]]
	* Liver failure		* Liver failure
	* Multiple organ dysfunction syndrome		* [[Multiple organ dysfunction syndrome]]
	* [[Encephalitis]]		* [[Encephalitis]]
	* Autoimmune lymphoproliferative syndrome		* [[Autoimmune lymphoproliferative syndrome]]
	* [[Drug reaction with eosinophilia and systemic symptoms]] (DRESS)		* [[Drug reaction with eosinophilia and systemic symptoms]] (DRESS)
	* [[Kawasaki disease]]		* [[Kawasaki disease]]
	* [[Cytophagic histiocytic panniculitis]]		* [[Cytophagic histiocytic panniculitis]]
	* [[TTP]]/[[HUS]]/[[DITMA]]		* [[TTP]]/[[HUS]]/[[DITMA]]
	* Transfusion-associated graft-versus-host disease		* [[Transfusion-associated graft-versus-host disease]]

	==Diagnostic Criteria (HLH-2004)==		==Diagnostic Criteria (HLH-2004)==

Aidan: /* Etiologies */ linkified

2023-01-19T15:24:01Z

Etiologies: linkified

← Older revision		Revision as of 15:24, 19 January 2023
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	* Infection		* Infection
	** Viral		** Viral
	*** EBV (most common)		*** [[EBV]] (most common)
	*** CMV, parvovirus, HSV, VZV, measles, HHV-8, H1N1 influenza virus, parechovirus, and HIV		*** [[CMV]], [[parvovirus]], [[HSV]], [[VZV]], [[measles]], [[HHV-8]], H1N1 [[influenza virus]], [[parechovirus]], and [[HIV]]
	*** SARS-CoV-2, possibly		*** [[SARS-CoV-2]], possibly
	** After anti-TNF therapy: tuberculosis, CMV, EBV, or histoplasmosis		** After anti-TNF therapy: [[tuberculosis]], [[CMV]], [[EBV]], or [[histoplasmosis]]
	** Rare: Brucella, gram-negative bacilli, tuberculosis, Leishmaniasis, malaria, and fungal infections		** Rare: [[Brucella]], gram-negative bacilli, [[tuberculosis]], [[Leishmaniasis]], [[malaria]], and fungal infections
	* Malignancy, most commonly lymphoid cancers and leukemias, but also solid cancers		* Malignancy, most commonly lymphoid cancers and leukemias, but also solid cancers
	* Rheumatologic disease		* Rheumatologic disease
	** Systemic juvenile idiopathic arthritis (Still disease)		** [[Systemic juvenile idiopathic arthritis]] ([[Still disease]])
	** Rheumatoid arthritis, dermatomyositis, systemic sclerosis, mixed connective tissue disease, antiphospholipid syndrome, ~~Sjogren~~ syndrome, ankylosing spondylitis, vasculitis, and sarcoidosis		** [[Rheumatoid arthritis]], [[dermatomyositis]], [[systemic sclerosis]], [[mixed connective tissue disease]], [[antiphospholipid syndrome]], [[Sjögren syndrome]], [[ankylosing spondylitis]], [[vasculitis]], and [[sarcoidosis]]
	* Immunodeficiency		* Immunodeficiency
	** [[Primary immunodeficiency]]		** [[Primary immunodeficiency]]
	** HIV~~/AIDS~~, stem cell transplant, kidney transplant, liver transplant		** [[HIV]], [[stem cell transplant]], [[kidney transplant]], [[liver transplant]]

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

Aidan at 18:00, 22 January 2021

2021-01-22T18:00:17Z

← Older revision		Revision as of 18:00, 22 January 2021
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			== Etiologies ==
⚫	== Diagnostic Criteria (HLH-2004) ==

			* Infection
			** Viral
			*** EBV (most common)
			*** CMV, parvovirus, HSV, VZV, measles, HHV-8, H1N1 influenza virus, parechovirus, and HIV
			*** SARS-CoV-2, possibly
			** After anti-TNF therapy: tuberculosis, CMV, EBV, or histoplasmosis
			** Rare: Brucella, gram-negative bacilli, tuberculosis, Leishmaniasis, malaria, and fungal infections
			* Malignancy, most commonly lymphoid cancers and leukemias, but also solid cancers
			* Rheumatologic disease
			** Systemic juvenile idiopathic arthritis (Still disease)
			** Rheumatoid arthritis, dermatomyositis, systemic sclerosis, mixed connective tissue disease, antiphospholipid syndrome, Sjogren syndrome, ankylosing spondylitis, vasculitis, and sarcoidosis
			* Immunodeficiency
			** [[Primary immunodeficiency]]
			** HIV/AIDS, stem cell transplant, kidney transplant, liver transplant

			== Differential Diagnosis ==

			* Macrophage activation syndrome
			* Severe sepsis
			* Liver failure
			* Multiple organ dysfunction syndrome
			* [[Encephalitis]]
			* Autoimmune lymphoproliferative syndrome
			* [[Drug reaction with eosinophilia and systemic symptoms]] (DRESS)
			* [[Kawasaki disease]]
			* [[Cytophagic histiocytic panniculitis]]
			* [[TTP]]/[[HUS]]/[[DITMA]]
			* Transfusion-associated graft-versus-host disease

		⚫	==Diagnostic Criteria (HLH-2004)==

	The diagnosis of HLH can be established if one of either 1 or 2 below is fulfilled:		The diagnosis of HLH can be established if one of either 1 or 2 below is fulfilled:

	# A molecular diagnosis consistent with HLH is made.		#A molecular diagnosis consistent with HLH is made.
	# Diagnostic criteria for HLH are fulfilled (5 of the 8 criteria below):*		#Diagnostic criteria for HLH are fulfilled (5 of the 8 criteria below):*
	#* Fever		#*Fever
	#* Splenomegaly		#*Splenomegaly
	#* Cytopenias (affecting ≥ 2-3 lineages in the peripheral blood):		#*Cytopenias (affecting ≥ 2-3 lineages in the peripheral blood):
	#** hemoglobin < 90 g/L (in infants < 4 weeks of age, hemoglobin < 100 g/L)		#**hemoglobin < 90 g/L (in infants < 4 weeks of age, hemoglobin < 100 g/L)
	#** platelets < 100x10^9^/L		#**platelets < 100x10^9^/L
	#** neutrophils < 1.0x10^9^/L		#**neutrophils < 1.0x10^9^/L
	#* Hypertriglyceridemia and/or hypofibrinogenemia:		#*Hypertriglyceridemia and/or hypofibrinogenemia:
	#** fasting triglycerides ≥ 3.0 mmol/L (ie, ≥ 265 mg/dL)		#**fasting triglycerides ≥ 3.0 mmol/L (ie, ≥ 265 mg/dL)
	#** fibrinogen ≤ 1.5 g/L		#**fibrinogen ≤ 1.5 g/L
	#* Hemophagocytosis in BM, spleen, or lymph nodes		#*Hemophagocytosis in BM, spleen, or lymph nodes
	#* Low or absent NK-cell activity (according to local laboratory reference)		#*Low or absent NK-cell activity (according to local laboratory reference)
	#* Ferritin ≥ 500 g/L		#*Ferritin ≥ 500 g/L
	#* Soluble CD25 (ie, sIL2r) 2400 U/mL†		#*Soluble CD25 (ie, sIL2r) 2400 U/mL†

	* Supportive criteria include neurologic symptoms, cerebrospinal fluid pleocytosis, conjugated hyperbilirubinemia and transaminitis, hypoalbuminemia, hyponatremia, elevated D-dimers, and lactate dehydrogenase (see text for details). The absence of hemophagocytosis in the BM does not exclude a diagnosis of HLH.		*Supportive criteria include neurologic symptoms, cerebrospinal fluid pleocytosis, conjugated hyperbilirubinemia and transaminitis, hypoalbuminemia, hyponatremia, elevated D-dimers, and lactate dehydrogenase (see text for details). The absence of hemophagocytosis in the BM does not exclude a diagnosis of HLH.

	† New data show normal variation by age. Level should be compared with ~~agerelated~~ norms.		† New data show normal variation by age. Level should be compared with age-related norms.

	[[Category:Hematology]]		[[Category:Hematology]]

Maintenance script: Imported from text file

2020-07-04T01:17:37Z

Imported from text file

New page

== Diagnostic Criteria (HLH-2004) ==

The diagnosis of HLH can be established if one of either 1 or 2 below is fulfilled:

# A molecular diagnosis consistent with HLH is made.
# Diagnostic criteria for HLH are fulfilled (5 of the 8 criteria below):*
#* Fever
#* Splenomegaly
#* Cytopenias (affecting ≥ 2-3 lineages in the peripheral blood):
#** hemoglobin < 90 g/L (in infants < 4 weeks of age, hemoglobin < 100 g/L)
#** platelets < 100x10^9^/L
#** neutrophils < 1.0x10^9^/L
#* Hypertriglyceridemia and/or hypofibrinogenemia:
#** fasting triglycerides ≥ 3.0 mmol/L (ie, ≥ 265 mg/dL)
#** fibrinogen ≤ 1.5 g/L
#* Hemophagocytosis in BM, spleen, or lymph nodes
#* Low or absent NK-cell activity (according to local laboratory reference)
#* Ferritin ≥ 500 g/L
#* Soluble CD25 (ie, sIL2r) 2400 U/mL†

* Supportive criteria include neurologic symptoms, cerebrospinal fluid pleocytosis, conjugated hyperbilirubinemia and transaminitis, hypoalbuminemia, hyponatremia, elevated D-dimers, and lactate dehydrogenase (see text for details). The absence of hemophagocytosis in the BM does not exclude a diagnosis of HLH.

† New data show normal variation by age. Level should be compared with agerelated norms.

[[Category:Hematology]]
[[Category:Rheumatology]]
[[Category:Infectious diseases]]