Myeloproliferative neoplasms - Revision history

Mark at 16:44, 26 May 2018

2018-05-26T16:44:50Z

← Older revision		Revision as of 16:44, 26 May 2018
Line 11:		Line 11:

	==Clinic==		==Clinic==
	* ~~moderate to severe~~ anemia (in ~~cases where the erythrocytosis is not a feature)~~		* increase in blood count(s), dependent on type of MPN
			* anemia may occur in late stage disease or due leukemic transformation
	* pale skin		* pale skin
	* fatigue		* fatigue
	* prolonged bleeding
	* enlarged spleen		* enlarged spleen
	* increased clotting		* increased clotting (i.e. thrombosis)

	==Polycythemia vera==		==Polycythemia vera==
Line 24:		Line 24:
	*Typical course:		*Typical course:
	**Prodromal stadium: Mild erythrocytosis.		**Prodromal stadium: Mild erythrocytosis.
	**Polycythemic phase: ~~Incereased~~ RBC count.		**Polycythemic phase: Increased RBC count.
	**Post-polycythemic ("spent") phase: Ineffective hematopoiesis, bone marrow fibrosis.		**Post-polycythemic ("spent") phase: Ineffective hematopoiesis, bone marrow fibrosis.
	*Transformation in [[AML]] possible (2-3%).		*Transformation to [[AML]] possible (2-3%).

	===Morphology===		===Morphology===
Line 32:		Line 32:
	*Many megakaryocytes in bone marrow section.		*Many megakaryocytes in bone marrow section.
	*Increased cellularity of bone marrow (35-100%).		*Increased cellularity of bone marrow (35-100%).
	*Teardrop-RBC cells (~~dacryocytes~~) in late stage.		*Teardrop-RBC cells (dacrocytes) in late stage.
	*Fibrosis (reticulin stain).		*Fibrosis (reticulin stain).

Line 39:		Line 39:
	*Median age: 55 years.		*Median age: 55 years.
	*Mostly asymptomatic.		*Mostly asymptomatic.
	*Indolent course, long-term survival. Life-threatening ~~thromboembolitic~~ epsisodes.		*Indolent course, long-term survival. Life-threatening thromboembolic epsisodes.

	===Morphology===		===Morphology===
	*Thrombocytosis.		*Thrombocytosis.
	*Bone marrow normo- to slightly hypercellular.		*Bone marrow normo- to slightly hypercellular.
	*Large megakaryocytes in bone-marrow.		*Large hypersegmented megakaryocytes in bone-marrow.
	*Usually no BM fibrosis.		*Usually no BM fibrosis.

Mark: MPN vs MDS

2018-05-26T16:25:30Z

MPN vs MDS

← Older revision		Revision as of 16:25, 26 May 2018
Line 1:		Line 1:
	'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms characterized by clonal expansion of pluripotent hematopoietic progenitor.		'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms characterized by clonal expansion of pluripotent hematopoietic progenitor.

	These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS).		These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS), the main difference is that MPNs produce functional haematological cells detectable on a complete blood count, whereas in MDS, hematopoisis is ineffective and is characterised by cytopenias.

	==Grouping==		==Grouping==
	They include the following:<ref name=pmid15781101>{{cite journal \|author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' \|title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders \|journal=Lancet \|volume=365 \|issue=9464 \|pages=1054–61 \|year=2005 \|pmid=15781101 \|doi=10.1016/S0140-6736(05)71142-9 \|url=}}</ref>		They include the following:<ref name=pmid15781101>{{cite journal \|author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' \|title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders \|journal=Lancet \|volume=365 \|issue=9464 \|pages=1054–61 \|year=2005 \|pmid=15781101 \|doi=10.1016/S0140-6736(05)71142-9 \|url=}}</ref>
	*Polycythemia vera.		*[[Chronic myeloid leukemia]]
	*Essential thrombocythemia.		*[[Polycythemia vera]]
	*Idiopathic myelofibrosis.		*[[Essential thrombocythemia]].
			*[[Idiopathic myelofibrosis]].

	==Clinic==		==Clinic==
	* moderate to severe anemia		* moderate to severe anemia (in cases where the erythrocytosis is not a feature)
	* pale skin		* pale skin
	* fatigue		* fatigue

Jensflorian: /* Essential thrombocythaemia */ added

2015-12-09T14:00:42Z

Essential thrombocythaemia: added

← Older revision		Revision as of 14:00, 9 December 2015
Line 33:		Line 33:
	*Teardrop-RBC cells (dacryocytes) in late stage.		*Teardrop-RBC cells (dacryocytes) in late stage.
	*Fibrosis (reticulin stain).		*Fibrosis (reticulin stain).

			==Essential thrombocythaemia==
			*ICD-O: 9962/3
			*Median age: 55 years.
			*Mostly asymptomatic.
			*Indolent course, long-term survival. Life-threatening thromboembolitic epsisodes.

			===Morphology===
			*Thrombocytosis.
			*Bone marrow normo- to slightly hypercellular.
			*Large megakaryocytes in bone-marrow.
			*Usually no BM fibrosis.

	==Images==		==Images==

Jensflorian: polycythemia + image

2015-12-09T13:41:46Z

polycythemia + image

← Older revision		Revision as of 13:41, 9 December 2015
Line 16:		Line 16:
	* enlarged spleen		* enlarged spleen
	* increased clotting		* increased clotting

			==Polycythemia vera==
			*ICD-O: 9950/3
			*Median age: 60 years.
			*Median survival: >10years.
			*Typical course:
			**Prodromal stadium: Mild erythrocytosis.
			**Polycythemic phase: Incereased RBC count.
			**Post-polycythemic ("spent") phase: Ineffective hematopoiesis, bone marrow fibrosis.
			*Transformation in [[AML]] possible (2-3%).

			===Morphology===
			*Panmyelosis.
			*Many megakaryocytes in bone marrow section.
			*Increased cellularity of bone marrow (35-100%).
			*Teardrop-RBC cells (dacryocytes) in late stage.
			*Fibrosis (reticulin stain).

	==Images==		==Images==
	<gallery>		<gallery>
	File:Thrombocytosis.jpg \| Thrombocythemia. Peripheral blood (WC/Erhabor Osario)		File:Thrombocytosis.jpg \| Thrombocythemia. Peripheral blood (WC/Erhabor Osario)
	File:Polycythemia_vera,_blood_smear.jpg \| Polycythemia vera. Peripheral blood (WC/AFIP)		File:Polycythemia_vera,_blood_smear.jpg \| Polycythemia vera. Peripheral blood (WC/Orlandi Mourao)
			File:Teardrop Cells smear 2009-09-22.JPG \| Teardrop RBC cells in polycythemia vera. Peripheral blood (WC/AFIP)
	File:Essential Thrombocythemia, Peripheral Blood (10189570483).jpg \| Essential thrombocythemia. Peripheral blood (WC/Ed Uthman)		File:Essential Thrombocythemia, Peripheral Blood (10189570483).jpg \| Essential thrombocythemia. Peripheral blood (WC/Ed Uthman)
	File:Essential thrombocythemia (2).jpg \| Essential thrombocythemia. Bone marrow (WC/KGH)		File:Essential thrombocythemia (2).jpg \| Essential thrombocythemia. Bone marrow (WC/KGH)

Jensflorian: +images

2015-12-09T13:25:36Z

+images

← Older revision		Revision as of 13:25, 9 December 2015
Line 16:		Line 16:
	* enlarged spleen		* enlarged spleen
	* increased clotting		* increased clotting

			==Images==
			<gallery>
			File:Thrombocytosis.jpg \| Thrombocythemia. Peripheral blood (WC/Erhabor Osario)
			File:Polycythemia_vera,_blood_smear.jpg \| Polycythemia vera. Peripheral blood (WC/AFIP)
			File:Essential Thrombocythemia, Peripheral Blood (10189570483).jpg \| Essential thrombocythemia. Peripheral blood (WC/Ed Uthman)
			File:Essential thrombocythemia (2).jpg \| Essential thrombocythemia. Bone marrow (WC/KGH)
			</gallery>

	==Molecular==		==Molecular==

Jensflorian: /* Molecular */ JAk2 negative

2015-12-09T08:38:44Z

Molecular: JAk2 negative

← Older revision		Revision as of 08:38, 9 December 2015
Line 20:		Line 20:
	* BCR-ABL1 negative (DDx: [[Leukemia]] (BCR-ABL1 positive))		* BCR-ABL1 negative (DDx: [[Leukemia]] (BCR-ABL1 positive))
	* A specific JAK2 mutation (Val617Phe) in seen in a group of patients.<ref name=pmid15781101>{{cite journal \|author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' \|title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders \|journal=Lancet \|volume=365 \|issue=9464 \|pages=1054–61 \|year=2005 \|pmid=15781101 \|doi=10.1016/S0140-6736(05)71142-9 \|url=}}</ref><ref name=pmid15860661>{{cite journal \|author=Steensma DP, Dewald GW, Lasho TL, ''et al.'' \|title=The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both "atypical" myeloproliferative disorders and myelodysplastic syndromes \|journal=Blood \|volume=106 \|issue=4 \|pages=1207–9 \|year=2005 \|month=August \|pmid=15860661 \|pmc=1895198 \|doi=10.1182/blood-2005-03-1183 \|url=}}</ref>		* A specific JAK2 mutation (Val617Phe) in seen in a group of patients.<ref name=pmid15781101>{{cite journal \|author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' \|title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders \|journal=Lancet \|volume=365 \|issue=9464 \|pages=1054–61 \|year=2005 \|pmid=15781101 \|doi=10.1016/S0140-6736(05)71142-9 \|url=}}</ref><ref name=pmid15860661>{{cite journal \|author=Steensma DP, Dewald GW, Lasho TL, ''et al.'' \|title=The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both "atypical" myeloproliferative disorders and myelodysplastic syndromes \|journal=Blood \|volume=106 \|issue=4 \|pages=1207–9 \|year=2005 \|month=August \|pmid=15860661 \|pmc=1895198 \|doi=10.1182/blood-2005-03-1183 \|url=}}</ref>
			**In JAK2-negative cases, CALR or MPL-Mutations are often found.<ref>{{Cite journal \| last1 = Nangalia \| first1 = J. \| last2 = Green \| first2 = TR. \| title = The evolving genomic landscape of myeloproliferative neoplasms. \| journal = Hematology Am Soc Hematol Educ Program \| volume = 2014 \| issue = 1 \| pages = 287-96 \| month = Dec \| year = 2014 \| doi = 10.1182/asheducation-2014.1.287 \| PMID = 25696868 }}</ref>

	==See also==		==See also==

Jensflorian: update

2015-12-09T08:30:27Z

update

← Older revision		Revision as of 08:30, 9 December 2015
Line 1:		Line 1:
	'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms.		'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms characterized by clonal expansion of pluripotent hematopoietic progenitor.

	These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS).		These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS).
Line 8:		Line 8:
	*Essential thrombocythemia.		*Essential thrombocythemia.
	*Idiopathic myelofibrosis.		*Idiopathic myelofibrosis.

			==Clinic==
			* moderate to severe anemia
			* pale skin
			* fatigue
			* prolonged bleeding
			* enlarged spleen
			* increased clotting

	==Molecular==		==Molecular==
	A specific JAK2 mutation (Val617Phe) in seen in a group of patients.<ref name=pmid15781101>{{cite journal \|author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' \|title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders \|journal=Lancet \|volume=365 \|issue=9464 \|pages=1054–61 \|year=2005 \|pmid=15781101 \|doi=10.1016/S0140-6736(05)71142-9 \|url=}}</ref><ref name=pmid15860661>{{cite journal \|author=Steensma DP, Dewald GW, Lasho TL, ''et al.'' \|title=The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both "atypical" myeloproliferative disorders and myelodysplastic syndromes \|journal=Blood \|volume=106 \|issue=4 \|pages=1207–9 \|year=2005 \|month=August \|pmid=15860661 \|pmc=1895198 \|doi=10.1182/blood-2005-03-1183 \|url=}}</ref>		* BCR-ABL1 negative (DDx: [[Leukemia]] (BCR-ABL1 positive))
			* A specific JAK2 mutation (Val617Phe) in seen in a group of patients.<ref name=pmid15781101>{{cite journal \|author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' \|title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders \|journal=Lancet \|volume=365 \|issue=9464 \|pages=1054–61 \|year=2005 \|pmid=15781101 \|doi=10.1016/S0140-6736(05)71142-9 \|url=}}</ref><ref name=pmid15860661>{{cite journal \|author=Steensma DP, Dewald GW, Lasho TL, ''et al.'' \|title=The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both "atypical" myeloproliferative disorders and myelodysplastic syndromes \|journal=Blood \|volume=106 \|issue=4 \|pages=1207–9 \|year=2005 \|month=August \|pmid=15860661 \|pmc=1895198 \|doi=10.1182/blood-2005-03-1183 \|url=}}</ref>

	==See also==		==See also==

Michael: fix sp.

2011-11-29T03:46:32Z

fix sp.

← Older revision		Revision as of 03:46, 29 November 2011
Line 6:		Line 6:
	They include the following:<ref name=pmid15781101>{{cite journal \|author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' \|title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders \|journal=Lancet \|volume=365 \|issue=9464 \|pages=1054–61 \|year=2005 \|pmid=15781101 \|doi=10.1016/S0140-6736(05)71142-9 \|url=}}</ref>		They include the following:<ref name=pmid15781101>{{cite journal \|author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' \|title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders \|journal=Lancet \|volume=365 \|issue=9464 \|pages=1054–61 \|year=2005 \|pmid=15781101 \|doi=10.1016/S0140-6736(05)71142-9 \|url=}}</ref>
	*Polycythemia vera.		*Polycythemia vera.
	*Essential ~~thrombocythaemia~~.		*Essential thrombocythemia.
	*Idiopathic myelofibrosis.		*Idiopathic myelofibrosis.

Michael: wikify

2011-05-12T17:22:40Z

wikify

← Older revision		Revision as of 17:22, 12 May 2011
Line 1:		Line 1:
	'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms.		'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms.

	These should '''not''' be confused with ''myelodysplastic syndromes'' (MDS).		These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS).

	==Grouping==		==Grouping==

Michael: vs. MDS

2011-05-12T17:20:23Z

vs. MDS

← Older revision		Revision as of 17:20, 12 May 2011
Line 1:		Line 1:
	'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms.		'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms.

			These should '''not''' be confused with ''myelodysplastic syndromes'' (MDS).

	==Grouping==		==Grouping==