Difference between revisions of "Myelodysplastic syndromes"

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They should not be confused with ''[[myeloproliferative neoplasms]]''.
They should not be confused with ''[[myeloproliferative neoplasms]]''.


==See also==
=Overview=
==Lab fidnings==
*+/-[[Anemia]].
*+/-Neutropenia.
*+/-Thrombocytopenia.
 
Notes:
*MDS is usu. an incidental finding, i.e. it is asymptomatic.
 
==Associations==
Syndromes associated with MDS:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref>
*Fanconi syndrome.
*Diamond-blackfan syndrome.
*Shwachman-diamond syndrome.
 
==Diagnostic criteria==
All of the following:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref>
#At least 6 months + persistent.
#Dysplasia.
#*Cytologic:
#**Need >=10% abnormal.
#*Cytogenetic.
#All other causes excluded.
#Blast percentage <20%.
#*If >=20% = [[leukemia]].
#*Criteria blast percentage:
#**Bone marrow: 500 nucleated cells - includes erythroblasts, plasma cells; excludes megakaryocytes.
#**Peripheral blood: 200 leukocytes.
 
===Required clinical information===
*CBC - usu. macrocytic anemia.
*Rx - may be Rx effect.
*Peripheral blood film.
*Clinical history (symptoms/presentation/PMHx).
 
===Laboratory work-up===
*[[H&E stain]].
*Giemsa/Wright stain.
*Iron stain.
*Gomori silver stain.
 
===DDx of MDS===
#Nutritional deficiency.
#*Vitamin B12.
#*Folate.
#Toxic exposures.
#*Rx.
#**G-CSF.
#*Biologic agents.
#*Heavy metals.
#*Chemotherapy.
#Infections.
#*Parovirus B19.
#Other.
#*Paroxysmal nocturnal hemoglobinuria (PNH).<ref>URL: [http://emedicine.medscape.com/article/207468-overview http://emedicine.medscape.com/article/207468-overview]. Accessed on: 29 May 2011.</ref> (???)
#*Congenital hematopoietic disorder.
#**Congenital dyserythropoietic anemia.
 
=Histologic features=
*Auer rods - '''used to be''' diagnostic of MDS regardless of blast count.
 
==Dyserythropoiesis==
*Abnormal [[RBC]] formation.
 
===Histology===
====Nuclear====
Features:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref>
*Nuclear budding.
*Intranuclear bridging (nuclei fail to separate post-division).
*Multinucleation.
*Megablastoid change.
**May be hard to see.
*Karyorrhexis (nuclear fragmentation).
 
====Cytoplasmic====
Features:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref>
*Ring sideroblasts.
**Rim of RBC has ring of iron.
*Vacuolization.
 
==Dysgranulopoiesis==
*Abnormal granulocyte formation.
 
===Histology===
Features:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref>
*Nuclear hypolobation (pseudo-Pelger-Huet).
*Hypersegmentation.
**May be seen in vitamin B12 deficiency, Rx.
*Cytoplasmic hypogranulation.
*Pseudo-Chediak-Higashi granules.
*Small size.
 
==Dysmegakaryocytopoiesis==
*Abnormal megakaryocyte formation.
 
===Histology===
Features:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref>
*Micromegakaryoctes with ypolobated nuclei.
*Non-lobated nuclei of any size.
*Multiple widely separated nuclear lobes.
 
 
=See also=
*[[Haematopathology]].
*[[Haematopathology]].
*[[Myeloproliferative neoplasms]].
*[[Myeloproliferative neoplasms]].
=References=
{{Reflist|1}}


[[Category:Haematopathology]]
[[Category:Haematopathology]]
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