Difference between revisions of "Leukemia"

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m (subclude CML)
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[[Image:Acute leukemia-ALL.jpg | thumb|right|A bone marrow of an individual with precursor B-cell lymphoblastic leukemia. [[Wright stain]]. (WC)]]
[[Image:Acute leukemia-ALL.jpg | thumb|right|A bone marrow of an individual with precursor B-cell lymphoblastic leukemia. [[Wright stain]]. (WC)]]
The article addresses '''leukemia''', which is uncommonly seen by anatomical pathologists.  It is a subset of [[hematopathology]].
The term '''leukemia''' is broadly used to refer to any haematological malignancy where the neoplastic cells in the circulation are the prominent feature. It is generally only seen by anatomical pathologists only in countries where hematopathology reporting is done by anatomical pathologists rather than hematologists.


Lymphoma is discussed in the ''[[lymphoma]]'' article, and overlaps somewhat with leukemia as the clear distinction between the two is historical (see below).
Lymphoma is discussed in the ''[[lymphoma]]'' article, and overlaps somewhat with leukemia as the clear distinction between the two is somewhat arbitrary and historical:<ref>{{Ref PCPBoD8|314}}</ref>
 
Historical classification:<ref>{{Ref PCPBoD8|314}}</ref>
*Leukemia = involves bone marrow +/- peripheral blood.
*Leukemia = involves bone marrow +/- peripheral blood.
**Classic presentation: infection, bleeding, anemia.
**Classic presentation: infection, bleeding, anemia.
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=Definition=
=Definition=
All of the following:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref>
This varies with the type.
 
For acute myeloid leukemia, all of the following:<ref name=dg21mar20011>D. Good. 21 March 2011.</ref>
#Morphologic abnormalities.
#Morphologic abnormalities.
#>20% blasts ''or'' recurrent cytogenetic abnormality.
#>20% blasts ''or'' recurrent cytogenetic abnormality.
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*inv(16).
*inv(16).
*t(15;17).
*t(15;17).
=Leukemia classification=
*[[Acute myeloid leukemia]] (AML):
*#AML.
*#AML with recurrent cytogenetic abnormalities.
*#AML from [[MDS]].
*#AML in the setting of [[Down syndrome]].
*[[Acute lymphoblastic leukemia]] (ALL):
*#B cell.
*#B cell with recurrent cytogenetic abnormalities.
*#T cell (sometimes abbreviated to T-ALL)
*Not to be confused with [[adult T-cell leukemia/lymphoma]] (ATLL)
*[[Chronic myeloid leukemia]] (CML)
*[[Chronic lymphocytic leukemia]] (CLL) - usually classified with lymphomas
Rarer leukemias:
*Plasma cell leukaemia
*Mast cell leukemia


=Histomorphologic overview=
=Histomorphologic overview=
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|}
|}
† should be easy to remember as smALL people, i.e. kids, get this type of acute leukemia.
† should be easy to remember as smALL people, i.e. kids, get this type of acute leukemia.


=Algorithms=
=Algorithms=
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*[[Down syndrome]]?
*[[Down syndrome]]?


=Leukemia classification=
Acute myeloid leukemia (AML):
#AML.
#AML with recurrent cytogenetic abnormalities.
#AML from [[MDS]].
#AML in the setting of [[Down syndrome]].
Acute lymphoid leukemia (ALL):
#B cell.
#B cell with recurrent cytogenetic abnormalities.
#T cell.
Chronic myeloid leukemia (CML).


=Prognosis=
=Prognosis=
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=Specific diagnoses=
=Specific diagnoses=
==Acute myeloid leukemia==
*[[Acute myeloid leukemia]]
*Abbreviated ''[[AML]]''.
*[[Acute lymphoblastic leukemia]]
===General===
*[[Chronic myeloid leukemia]]
*Adults.
*[[Chronic lymphocytic leukemia]]
 
Exclusions for this diagnosis:
*Prior [[MDS]].
*[[Down syndrome]].
 
===Microscopic===
Features:
*[[Auer rods]] present
*Cytoplasmic granularity.
*Large cells.
 
Note:
*May be classified by morphology, using the (old) French-American-British (FAB) classification (M0-M7).
 
====Image====
<gallery>
Image:Auer_rods.PNG | Auer rods in an AML. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case344.html AML - several images (upmc.edu)].
 
===Molecular===
*Must exclude all the recurrent cytogenetic abnormalities - see below.
 
==AML with recurrent cytogenetic abnormalities==
===Acute myeloid leukemia with t(8;21)===
*t(8;21)(q22;q22).<ref>{{Cite journal  | last1 = Berger | first1 = R. | title = Translocation t(8;21)(q22;q22): cytogenetics and molecular biology. | journal = Nouv Rev Fr Hematol | volume = 36 Suppl 1 | issue =  | pages = S67-9 | month =  | year = 1994 | doi =  | PMID = 8177719 }}</ref>
 
IHC:
*CD34+, CD13+, MPO+ (cytoplasm), CD33+ (weak).
*[[CD56]]+, CD117+.
**Usu. assoc. with a bad prognosis.
 
Flow cytometry:
*CD19+, PAX5+, CD79a +/-.
 
Images:
*[http://path.upmc.edu/cases/case712.html AML with t(8;21) (upmc.edu)].
 
===Acute myeloid leukemia with inv(16)===
*inv(16)(p13.1q22).<ref name=pmid16917916>{{Cite journal  | last1 = Lu | first1 = CM. | last2 = Murata-Collins | first2 = JL. | last3 = Wang | first3 = E. | last4 = Siddiqi | first4 = I. | last5 = Lawrence | first5 = HJ. | title = Concurrent acute myeloid leukemia with inv(16)(p13.1q22) and chronic lymphocytic leukemia: molecular evidence of two separate diseases. | journal = Am J Hematol | volume = 81 | issue = 12 | pages = 963-8 | month = Dec | year = 2006 | doi = 10.1002/ajh.20716 | PMID = 16917916 }}
</ref>
 
*Associated with [[myeloid sarcoma]].
 
Microscopic:
*Blast count usu. ~20% (low).
*Eosinophilic granules.
**Used to be classified as "M4" with eosinophilia.
 
IHC:
*CD2+ -- common.
 
===Acute myeloid leukemia with t(15;17)===
*[[AKA]] ''acute promyelocytic leukemia''
**Abbreviated ''APL''.
*t(15;17)(q22;q12).
**Fusion transcripts: PML<ref name=omim102578>{{OMIM|102578}}</ref>-RARA.<ref name=omim180240>{{OMIM|180240}}</ref>
 
====General====
Clinical:
*Associated with [[DIC]].
*Treatment: all-trans retinoic acid (ATRA).
 
Variants:
*t(11;17) -- ATRA doesn't work.<ref>{{Ref APBR|623 Q2}}</ref>
*t(17;17) -- ATRA doesn't work.
*t(5;17). (???)
 
====Microscopic====
Comes in two flavours.
 
Microscopic (Hypergranular ''or'' typical APL):
*Bean-shaped nucleus ''or'' bilobed nucleus.
*Buddles of Auer rods - known as "Faggot cells".
 
Microscopic (Microgranular ''or'' hypogranular APL):
*Bilobed nuclei with nuclear overlap. (???)
*Absence of granules on light microscopy.
 
=====Images=====
<gallery>
Image:Faggot cell in AML-M3.jpg |Faggot cell in AML-M3. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case457/images/fig01.jpg AML - showing Auer rods (upmc.edu)].<ref>URL: [http://path.upmc.edu/cases/case457.html http://path.upmc.edu/cases/case457.html]. Accessed on: 21 January 2012.</ref>
*[http://path.upmc.edu/cases/case705.html APML - several images (upmc.edu)].
 
====IHC====
*CD2 +ve, CD34 +ve/-ve, CD56 +ve/-ve.
 
====[[Flow cytometry]]====
*CD34 -ve, HLA-DR -ve.
*CD33 +ve, CD13 +ve/-ve, CD117 +ve (weak), CD56 +ve/-ve.
 
===Acute myeloid leukemia with t(9;11)===
*t(9;11).
 
Microscopic:
*Monoblastic morphology. (???)
*Myelomonocytic morphology. (???)
 
Clinical:
*+/-[[DIC]].
*Usu. children.
 
IHC:
*CD33+, CD65+, CD4+, HLA-DR+.
*CD34+. (???)
*CD13+. (???)
 
==Chronic myeloid leukemia==
{{:Chronic myeloid leukemia}}


=See also=
=See also=

Revision as of 19:00, 26 May 2018

A bone marrow of an individual with precursor B-cell lymphoblastic leukemia. Wright stain. (WC)

The term leukemia is broadly used to refer to any haematological malignancy where the neoplastic cells in the circulation are the prominent feature. It is generally only seen by anatomical pathologists only in countries where hematopathology reporting is done by anatomical pathologists rather than hematologists.

Lymphoma is discussed in the lymphoma article, and overlaps somewhat with leukemia as the clear distinction between the two is somewhat arbitrary and historical:[1]

  • Leukemia = involves bone marrow +/- peripheral blood.
    • Classic presentation: infection, bleeding, anemia.
  • Lymphoma = discrete mass(es), usu. lymph node.
    • Classic presentation: non-tender lymph nodes

Definition

This varies with the type.

For acute myeloid leukemia, all of the following:[2]

  1. Morphologic abnormalities.
  2. >20% blasts or recurrent cytogenetic abnormality.

Some recurrent cytogenetic abnormalities:

  • t(8;21).
  • inv(16).
  • t(15;17).

Leukemia classification

Rarer leukemias:

  • Plasma cell leukaemia
  • Mast cell leukemia

Histomorphologic overview

Disease/Feature Blast size Auer rods Granulation of cytoplasm
Acute myeloid leukemia (AML) larger present present
Acute lymphoid leukemia (ALL) smaller † none absent or present

† should be easy to remember as smALL people, i.e. kids, get this type of acute leukemia.

Algorithms

There is a nice set of algorithms from D. Arber - that were presented at the 2009 USCAP.

Clinical factors in classification

Clinical are important in the classification of leukemia.


Prognosis

Highly dependent on health care system and treatment available[3]

  • 5-year overall survival in children ranges:
  1. lymphoid leukaemia: 52.4% (Colombia) to 91.6% (Germany)
  2. acute myleoid leukemia: 33.3% (Bulgaria) to 78.2% (Germany)

Specific diagnoses

See also

References

  1. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 314. ISBN 978-1416054542.
  2. ↑ D. Good. 21 March 2011.
  3. ↑ Bonaventure, A.; Harewood, R.; Stiller, CA.; Gatta, G.; Clavel, J.; Stefan, DC.; Carreira, H.; Spika, D. et al. (Apr 2017). "Worldwide comparison of survival from childhood leukaemia for 1995-2009, by subtype, age, and sex (CONCORD-2): a population-based study of individual data for 89 828 children from 198 registries in 53 countries.". Lancet Haematol. doi:10.1016/S2352-3026(17)30052-2. PMID 28411119.
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