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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]] | ||