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]]

Revision as of 16:45, 29 May 2011

Myelodysplastic syndromes, abbreviated MDS, can be thought of a pre-leukemia/pre-lymphoma.

They should not be confused with myeloproliferative neoplasms.

Overview

Lab fidnings

  • +/-Anemia.
  • +/-Neutropenia.
  • +/-Thrombocytopenia.

Notes:

  • MDS is usu. an incidental finding, i.e. it is asymptomatic.

Associations

Syndromes associated with MDS:[1]

  • Fanconi syndrome.
  • Diamond-blackfan syndrome.
  • Shwachman-diamond syndrome.

Diagnostic criteria

All of the following:[1]

  1. At least 6 months + persistent.
  2. Dysplasia.
    • Cytologic:
      • Need >=10% abnormal.
    • Cytogenetic.
  3. All other causes excluded.
  4. 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

  1. Nutritional deficiency.
    • Vitamin B12.
    • Folate.
  2. Toxic exposures.
    • Rx.
      • G-CSF.
    • Biologic agents.
    • Heavy metals.
    • Chemotherapy.
  3. Infections.
    • Parovirus B19.
  4. Other.
    • Paroxysmal nocturnal hemoglobinuria (PNH).[2] (???)
    • 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:[1]

  • Nuclear budding.
  • Intranuclear bridging (nuclei fail to separate post-division).
  • Multinucleation.
  • Megablastoid change.
    • May be hard to see.
  • Karyorrhexis (nuclear fragmentation).

Cytoplasmic

Features:[1]

  • Ring sideroblasts.
    • Rim of RBC has ring of iron.
  • Vacuolization.

Dysgranulopoiesis

  • Abnormal granulocyte formation.

Histology

Features:[1]

  • 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:[1]

  • Micromegakaryoctes with ypolobated nuclei.
  • Non-lobated nuclei of any size.
  • Multiple widely separated nuclear lobes.


See also

References

  1. ↑ 1.0 1.1 1.2 1.3 1.4 1.5 D. Good. 21 March 2011.
  2. ↑ URL: http://emedicine.medscape.com/article/207468-overview. Accessed on: 29 May 2011.
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