Difference between revisions of "Myelodysplastic syndromes"

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Myelodysplastic syndromes (view source)

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→‎Dysmegakaryocytopoiesis

Michael

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 They should not be confused with ''[[myeloproliferative neoplasms]]''.
-==See also==
+=Overview=
+==Lab findings==
+*+/-[[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.
+=Bone marrow specimens=
+==Normal==
+*Age (years) ~ percentage of fat.
+**Example 80 years old has ~ 80% fat in marrow space.
+Erythroblasts:
+*Typically away from bone.
+Megakaryocytes:
+#Alone - not in clusters.
+#Not close to bone.
+Myeloid cells:
+*Adjacent to bone.
+=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-Huët).
+*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 hypolobated nuclei.
+*Non-lobated nuclei of any size.
+*Multiple widely separated nuclear lobes.
+Image:
+*[http://www.atlasbloodcells.es/ficha.php?codigo=410&codigo2=499&indice=410 Dysmegakaryocytopoiesis (atlasbloodcells.es)].
+=IHC=
+Typical [[IHC]] in work-up of MDS:
+*CD34 - (myeloid) progenitor/precursor cells.
+*CD117 - (myeloid) progenitor/precursor cells, [[mast cell]]s.
+*Tryptase - mast cells, immature basophils.
+**Uncommonly done.
+*CD61 - megakaryocytes.
+*CD42b - megakaryocytes.
+*CD20 - B cells.
+*CD3 - T cells.
+*Glycophorin A - erythroid cells.
+*Glycophorin C - erythroid cells.
+Notes:
+*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=
 *[[Haematopathology]].
 *[[Myeloproliferative neoplasms]].
+*[[Leukemia]].
+=References=
+{{Reflist|1}}
 [[Category:Haematopathology]]

Difference between revisions of "Myelodysplastic syndromes"

Myelodysplastic syndromes (view source)

Revision as of 15:42, 18 February 2016

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