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Difference between revisions of "Myelodysplastic syndromes"
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Myelodysplastic syndromes (view source)
Revision as of 15:42, 18 February 2016
, 15:42, 18 February 2016→Dysmegakaryocytopoiesis
(→Dysmegakaryocytopoiesis: +IHC) |
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=Overview= | =Overview= | ||
==Lab | ==Lab findings== | ||
*+/-[[Anemia]]. | *+/-[[Anemia]]. | ||
*+/-Neutropenia. | *+/-Neutropenia. | ||
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#*Congenital hematopoietic disorder. | #*Congenital hematopoietic disorder. | ||
#**Congenital dyserythropoietic anemia. | #**Congenital dyserythropoietic anemia. | ||
=Bone marrow specimens= | =Bone marrow specimens= | ||
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=Histologic features= | =Histologic features= | ||
*Auer rods - '''used to be''' diagnostic of MDS regardless of blast count. | *[[Auer rods]] - '''used to be''' diagnostic of MDS regardless of blast count. | ||
==Dyserythropoiesis== | ==Dyserythropoiesis== | ||
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===Histology=== | ===Histology=== | ||
Features:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref> | Features:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref> | ||
*Nuclear hypolobation (pseudo | *Nuclear hypolobation (pseudo Pelger-Huët). | ||
*Hypersegmentation. | *Hypersegmentation. | ||
**May be seen in vitamin B12 deficiency, Rx. | **May be seen in vitamin B12 deficiency, Rx. | ||
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===Histology=== | ===Histology=== | ||
Features:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref> | Features:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref> | ||
*Micromegakaryoctes with | *Micromegakaryoctes with hypolobated nuclei. | ||
*Non-lobated nuclei of any size. | *Non-lobated nuclei of any size. | ||
*Multiple widely separated nuclear lobes. | *Multiple widely separated nuclear lobes. | ||
Image: | |||
*[http://www.atlasbloodcells.es/ficha.php?codigo=410&codigo2=499&indice=410 Dysmegakaryocytopoiesis (atlasbloodcells.es)]. | |||
=IHC= | =IHC= | ||
Typical [[IHC]] in work-up of MDS: | Typical [[IHC]] in work-up of MDS: | ||
*CD34 - (myeloid) progenitor/precursor cells. | *CD34 - (myeloid) progenitor/precursor cells. | ||
*CD117 - (myeloid) progenitor/precursor cells, mast | *CD117 - (myeloid) progenitor/precursor cells, [[mast cell]]s. | ||
* | *Tryptase - mast cells, immature basophils. | ||
**Uncommonly done. | **Uncommonly done. | ||
*CD61 - megakaryocytes. | *CD61 - megakaryocytes. | ||
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Notes: | Notes: | ||
*Other | *Other markers: | ||
**Factor VIII. | |||
**vWF. | |||
*Blasts - the gold standard: histomorphology. | |||
**Most blasts are CD34 +ve. | |||
***[[Flow cytometry]] not useful (yet) for MDS -- as CD34 +ve != blast; may change with more multiplexing. | |||
**CD117 marks some blasts that are CD34 -ve. | |||
=[[Cytogenetics]]= | |||
*Important in the Dx and prognostication of MDS; however, only ~50% have a cytogenetic abnormality based on karyotyping. | |||
Common changes: | |||
#Isolated del(5q). | |||
#Isolated del(17p). | |||
#*Assoc. with pseudo-Pelger-Huet. (???) | |||
#Monosomy 7. | |||
#Monosomy 8. | |||
There is a scoring system for cytogenetic abnormalities ''International Prognostic Scoring System'',<ref name=pmid21594886>{{Cite journal | last1 = Garcia-Manero | first1 = G. | title = Myelodysplastic syndromes: 2011 update on diagnosis, risk-stratification, and management. | journal = Am J Hematol | volume = 86 | issue = 6 | pages = 490-8 | month = Jun | year = 2011 | doi = 10.1002/ajh.22047 | PMID = 21594886 }} | |||
</ref> abbreviated ''IPSS'': | |||
*Higher score = worse outcome. | |||
=WHO classification (2008)= | |||
*Refractory cytopenia with unilineage dysplasia (RCUD). | |||
*#Anemia. | |||
*#Neutropenia | |||
*#Thrombocytopenia. | |||
*Refractory anemia with ringed sideroblasts (RARS). | |||
*Refractory cytopenia with multilineage dysplasia (RCMD).<ref name=pmid8558932>{{Cite journal | last1 = Rosati | first1 = S. | last2 = Mick | first2 = R. | last3 = Xu | first3 = F. | last4 = Stonys | first4 = E. | last5 = Le Beau | first5 = MM. | last6 = Larson | first6 = R. | last7 = Vardiman | first7 = JW. | title = Refractory cytopenia with multilineage dysplasia: further characterization of an 'unclassifiable' myelodysplastic syndrome. | journal = Leukemia | volume = 10 | issue = 1 | pages = 20-6 | month = Jan | year = 1996 | doi = | PMID = 8558932 }}</ref> | |||
*Refractory anemia with excess blasts (RAEB). | |||
**RAEB-1. | |||
**RAEB-2: Auer rods ''or'' >= 10% blasts. | |||
*MDS with isolated del(5q). | |||
*MDS unclassifiable. | |||
=See also= | =See also= | ||
*[[Haematopathology]]. | *[[Haematopathology]]. | ||
*[[Myeloproliferative neoplasms]]. | *[[Myeloproliferative neoplasms]]. | ||
*[[Leukemia]]. | |||
=References= | =References= | ||