Myelofibrosis diagnostic study of choice: Difference between revisions

	Myelofibrosis Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Myelofibrosis from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Myelofibrosis diagnostic study of choice On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Myelofibrosis diagnostic study of choice All Images X-rays Echo and Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Myelofibrosis diagnostic study of choice
CDC on Myelofibrosis diagnostic study of choice
Myelofibrosis diagnostic study of choice in the news
Blogs on Myelofibrosis diagnostic study of choice
Directions to Hospitals Treating Myelofibrosis
Risk calculators and risk factors for Myelofibrosis diagnostic study of choice

← Older edit Newer edit →

VisualWikitext

Revision as of 20:01, 11 December 2018

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Overview

Diagnosis of myelofibrosis may be made based upon a thorough clinical evaluation, detailed patient history, and specialized tests. The World Health Organization (WHO) has set the criteria for diagnosing primary myelofibrosis (PMF). It has determined set rules for distinguishing the prefibrotic/early (pre-primary myelofibrosis) phase and the overtly fibrotic (overt primary myelofibrosis) phase. The World Health Organization (WHO) has also introduced a proposed revised criteria for primary myelofibrosis (PMF).

Diagnostic Criteria

2001 World Health Organization (WHO) criteria for prefibrotic/early (pre-primary myelofibrosis) phase


Clinical findings	Morphological findings

Spleen and liver No or mild splenomegaly or hepatomegaly	Blood No or mild leukoerythroblastosis No or mild red blood cell poikilocytosis Few if any dacryocytes
Hematology (variable) Mild anemia Mild to moderate leukocytosis Mild to marked thrombocytosis	Bone marrow Hypercellularity Neutrophilic proliferation Megakaryocytic proliferation

^[1]^[2]

2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase


Clinical findings	Morphological findings

Spleen and liver Moderate to marked splenomegaly or hepatomegaly	Blood Leukoerythroblastosis Prominent red blood cell poikilocytosis Prominent dacryocytosis
Hematology (variable) Leukoerythroblastosis White blood cells decreased to elevated Platelet count decreased to elevated	Bone marrow Reticulin and/or collagen fibrosis Decreased cellularity Dilated marrow sinuses Intraluminal hematopoiesis Neutrophilic proliferation Prominent megakaryocytic proliferation Megakaryocytic atypia* New bone formation (osteosclerosis) _{*Clustering of megakaryocytes, abnormally lobulated megakaryocytic nuclei, naked megakaryocytic nuclei}

^[1]^[2]

Proposed revised World Health Organization (WHO) criteria for primary myelofibrosis (PMF)


Clinical findings

Major criteria Presence of megakaryocyte proliferation and atypia,* usually accompanied by either reticulin and/or collagen fibrosis, or, in the absence of significant reticulin fibrosis, the megakaryocyte changes must be accompanied by an increased bone marrow cellularity characterized by granulocytic proliferation and often decreased erythropoiesis (ie, prefibrotic cellular-phase disease) Not meeting WHO criteria for PV, †CML, ‡ MDS, § or other myeloid neoplasm Demonstration of JAK2617V>F or other clonal marker (eg, MPL515W>L/K), or in the absence of a clonal marker, no evidence of bone marrow fibrosis due to underlying inflammatory or other neoplastic diseases¶
Minor criteria Leukoerythroblastosis∥ Increase in serum lactate dehydrogenase level∥ Anemia∥ Palpable splenomegaly∥ _{*Small to large megakaryocytes with an aberrant nuclear/cytoplasmic ratio and hyperchromatic, bulbous, or irregularly folded nuclei and dense clustering.} _{†Requires the failure of iron replacement therapy to increase hemoglobin level to the polycythemia vera range in the presence of decreased serum ferritin. Exclusion of polycythemia vera is based on hemoglobin and hematocrit levels. Red cell mass measurement is not required.} _{‡Requires the absence of BCR-ABL.} _{§Requires the absence of dyserythropoiesis and dysgranulopoiesis.} _{¶Secondary to infection, autoimmune disorder or other chronic inflammatory condition, hairy cell leukemia or other lymphoid neoplasm, metastatic malignancy, or toxic (chronic) myelopathies. It should be noted that patients with conditions associated with reactive myelofibrosis are not immune to primary myelofibrosis and the diagnosis should be considered in such cases if other criteria are met.} _{∥Degree of abnormality could be borderline or marked.}

Diagnosis requires meeting all 3 major criteria and 2 minor criteria.^[1]^[2]^[3]

References

↑ ^1.0 ^1.1 ^1.2 Mesa RA, Verstovsek S, Cervantes F, Barosi G, Reilly JT, Dupriez B, Levine R, Le Bousse-Kerdiles MC, Wadleigh M, Campbell PJ, Silver RT, Vannucchi AM, Deeg HJ, Gisslinger H, Thomas D, Odenike O, Solberg LA, Gotlib J, Hexner E, Nimer SD, Kantarjian H, Orazi A, Vardiman JW, Thiele J, Tefferi A (June 2007). "Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT)". Leuk. Res. 31 (6): 737–40. doi:10.1016/j.leukres.2006.12.002. PMID 17210175.
↑ ^2.0 ^2.1 ^2.2 Tefferi, A.; Thiele, J.; Orazi, A.; Kvasnicka, H. M.; Barbui, T.; Hanson, C. A.; Barosi, G.; Verstovsek, S.; Birgegard, G.; Mesa, R.; Reilly, J. T.; Gisslinger, H.; Vannucchi, A. M.; Cervantes, F.; Finazzi, G.; Hoffman, R.; Gilliland, D. G.; Bloomfield, C. D.; Vardiman, J. W. (2007). "Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel". Blood. 110 (4): 1092–1097. doi:10.1182/blood-2007-04-083501. ISSN 0006-4971.
↑ Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

Template:WH Template:WS

[pmid17210175-1] 1.0 ^1.1 ^1.2 Mesa RA, Verstovsek S, Cervantes F, Barosi G, Reilly JT, Dupriez B, Levine R, Le Bousse-Kerdiles MC, Wadleigh M, Campbell PJ, Silver RT, Vannucchi AM, Deeg HJ, Gisslinger H, Thomas D, Odenike O, Solberg LA, Gotlib J, Hexner E, Nimer SD, Kantarjian H, Orazi A, Vardiman JW, Thiele J, Tefferi A (June 2007). "Primary myelofibrosis (PMF), post polycythemia vera myelofibrosis (post-PV MF), post essential thrombocythemia myelofibrosis (post-ET MF), blast phase PMF (PMF-BP): Consensus on terminology by the international working group for myelofibrosis research and treatment (IWG-MRT)". Leuk. Res. 31 (6): 737–40. doi:10.1016/j.leukres.2006.12.002. PMID 17210175.

[TefferiThiele2007-2] 2.0 ^2.1 ^2.2 Tefferi, A.; Thiele, J.; Orazi, A.; Kvasnicka, H. M.; Barbui, T.; Hanson, C. A.; Barosi, G.; Verstovsek, S.; Birgegard, G.; Mesa, R.; Reilly, J. T.; Gisslinger, H.; Vannucchi, A. M.; Cervantes, F.; Finazzi, G.; Hoffman, R.; Gilliland, D. G.; Bloomfield, C. D.; Vardiman, J. W. (2007). "Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendations from an ad hoc international expert panel". Blood. 110 (4): 1092–1097. doi:10.1182/blood-2007-04-083501. ISSN 0006-4971.

[3] Hoffman, Ronald (2018). Hematology : basic principles and practice. Philadelphia, PA: Elsevier. ISBN 9780323357623.

[1]

[2]

[3]

@@ Line 4: / Line 4: @@
 ==Overview==
-Diagnosis of myelofibrosis may be made based upon a thorough clinical evaluation, detailed patient history, and specialized tests. The World Health Organization (WHO) has set the criteria for diagnosing primary myelofibrosis (PMF). It has determined set rules for distinguishing the prefibrotic/early (pre-primary myelofibrosis) phase and the overtly fibrotic (overt primary myelofibrosis) phase. The World Health Organization (WHO) has also introduced a proposed revised criteria for primary myelofibrosis (PMF).
+[[Diagnosis]] of [[myelofibrosis]] may be made based upon a thorough clinical evaluation, detailed [[patient history]], and specialized tests. The World Health Organization (WHO) has set the criteria for diagnosing primary myelofibrosis (PMF). It has determined set rules for distinguishing the prefibrotic/early (pre-primary myelofibrosis) phase and the overtly fibrotic (overt primary myelofibrosis) phase. The World Health Organization (WHO) has also introduced a proposed revised criteria for primary myelofibrosis (PMF).
 ==Diagnostic Criteria==
 ===2001 World Health Organization (WHO) criteria for prefibrotic/early (pre-primary myelofibrosis) phase===
 {| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
-|valign=top|
+| valign="top" |
 |+
 ! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
 ! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Morphological findings}}
 |-
-| style="padding: 5px 5px; background: #DCDCDC;|'''Spleen and liver'''
+| style="padding: 5px 5px; background: #DCDCDC;" |'''Spleen and liver'''
 *No or mild splenomegaly or hepatomegaly
 | style="padding: 5px 5px; background: #DCDCDC;" |'''Blood'''
@@ Line 21: / Line 21: @@
 *Few if any dacryocytes
 |-
-| style="padding: 5px 5px; background: #DCDCDC;|'''Hematology (variable)'''
+| style="padding: 5px 5px; background: #DCDCDC;" |'''Hematology (variable)'''
 *Mild anemia
 *Mild to moderate leukocytosis
@@ Line 33: / Line 33: @@
 ===2001 World Health Organization (WHO) criteria for overtly fibrotic (overt primary myelofibrosis) phase===
 {| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
-|valign=top|
+| valign="top" |
 |+
 ! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
 ! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Morphological findings}}
 |-
-| style="padding: 5px 5px; background: #DCDCDC;|'''Spleen and liver'''
+| style="padding: 5px 5px; background: #DCDCDC;" |'''Spleen and liver'''
 *Moderate to marked splenomegaly or hepatomegaly
 | style="padding: 5px 5px; background: #DCDCDC;" |'''Blood'''
@@ Line 45: / Line 45: @@
 *Prominent dacryocytosis
 |-
-| style="padding: 5px 5px; background: #DCDCDC;|'''Hematology (variable)'''
+| style="padding: 5px 5px; background: #DCDCDC;" |'''Hematology (variable)'''
 *Leukoerythroblastosis
 *White blood cells decreased to elevated
@@ Line 63: / Line 63: @@
 ===Proposed revised World Health Organization (WHO) criteria for primary myelofibrosis (PMF)===
 {| style="border: 0px; font-size: 90%; margin: 3px; width:650px"
-|valign=top|
+| valign="top" |
 |+
 ! style="background: #4479BA; width: 340px;" | {{fontcolor|#FFF|Clinical findings}}
 |-
-| style="padding: 5px 5px; background: #DCDCDC;|'''Major criteria'''
+| style="padding: 5px 5px; background: #DCDCDC;" |'''Major criteria'''
 #Presence of megakaryocyte proliferation and atypia,* usually accompanied by either reticulin and/or collagen fibrosis, or, in the absence of significant reticulin fibrosis, the megakaryocyte changes must be accompanied by an increased bone marrow cellularity characterized by granulocytic proliferation and often decreased erythropoiesis (ie, prefibrotic cellular-phase disease)
 #Not meeting WHO criteria for PV, †CML, ‡ MDS, § or other myeloid neoplasm
 #Demonstration of JAK2617V>F or other clonal marker (eg, MPL515W>L/K), or in the absence of a clonal marker, no evidence of bone marrow fibrosis due to underlying inflammatory or other neoplastic diseases¶
 |-
-| style="padding: 5px 5px; background: #DCDCDC;|'''Minor criteria'''
+| style="padding: 5px 5px; background: #DCDCDC;" |'''Minor criteria'''
 #Leukoerythroblastosis∥
 #Increase in serum lactate dehydrogenase level∥