Hairy cell leukemia: Difference between revisions

(4 intermediate revisions by 2 users not shown)

Line 1:

~~#redirect~~ [[~~Small_cell_lymphomas#Hairy_cell_leukemia~~]]

[[File:Bone marrow infiltration by hairy cell leukaemia.jpg|thumb|High power magnification of a bone marrow trephine showing infiltration by hairy cell leukaemia.]]

Indolent B-cell neoplasm, abbreviated ''HCL''. Named for the appearances on peripheral blood smears. The vast majority (>95%) harbour the BRAF V600E mutation.<ref name=pmid25992240>{{Cite journal | last1 = Ahmadzadeh | first1 = A. | last2 = Shahrabi | first2 = S. | last3 = Jaseb | first3 = K. | last4 = Norozi | first4 = F. | last5 = Shahjahani | first5 = M. | last6 = Vosoughi | first6 = T. | last7 = Hajizamani | first7 = S. | last8 = Saki | first8 = N. | title = BRAF Mutation in Hairy Cell Leukemia. | journal = Oncol Rev | volume = 8 | issue = 2 | pages = 253 | month = Sep | year = 2014 | doi = 10.4081/oncol.2014.253 | PMID = 25992240 }}</ref> Mutations in other parts of the BRAF gene are described, but rare.<ref name=pmid24433452>{{Cite journal | last1 = Tschernitz | first1 = S. | last2 = Flossbach | first2 = L. | last3 = Bonengel | first3 = M. | last4 = Roth | first4 = S. | last5 = Rosenwald | first5 = A. | last6 = Geissinger | first6 = E. | title = Alternative BRAF mutations in BRAF V600E-negative hairy cell leukaemias. | journal = Br J Haematol | volume = 165 | issue = 4 | pages = 529-33 | month = May | year = 2014 | doi = 10.1111/bjh.12735 | PMID = 24433452 }}</ref>

[[Category:~~Diagnosis~~]]

In the bone marrow, they are associated with heavy reticulin fibrosis, commonly resulting in ''dry taps''.<ref name=pmid22499303>{{Cite journal | last1 = Galani | first1 = KS. | last2 = Subramanian | first2 = PG. | last3 = Gadage | first3 = VS. | last4 = Rahman | first4 = K. | last5 = Ashok Kumar | first5 = MS. | last6 = Shinde | first6 = S. | last7 = Mahadik | first7 = S. | last8 = Ansari | first8 = R. | last9 = Sengar | first9 = M. | title = Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital. | journal = Indian J Pathol Microbiol | volume = 55 | issue = 1 | pages = 61-5 | month = | year = | doi = 10.4103/0377-4929.94858 | PMID = 22499303 }}</ref>

Clinical:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022]. Accessed on: 20 August 2010.</ref>

*Pancytopenia. Monocytopenia common in earlier disease.

*Splenic enlargement.

*No lymphadenopathy.

*Good prognosis (with treatment), though (likely) not curable.

==Gross==

Features:<ref name=Ref_PCPBoD8_326>{{Ref PCPBoD8|326}}</ref>

*Huge beefy red [[spleen]].

**Red as white pulp obliterated.

==Microscopic==

Features:<ref>URL: [http://emedicine.medscape.com/article/200580-diagnosis http://emedicine.medscape.com/article/200580-diagnosis]. Accessed on: 18 August 2010.</ref>

*Small cells (10-20 micrometers) with "Fried egg"-like appearance:

**Well-demarcated fuzzy cell borders,

**Clear/whispy cytoplasm and,

**Central round nucleus.

***Peri-nuclear clearing ("water-clear rim"<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022]. Accessed on: 20 August 2010.</ref>) -- '''key feature'''.

DDx:

*[[Hepatosplenic T-cell lymphoma]].

===Images===

Image:Hairy_cell_leukemia.jpg | HCL - blood film. (WC)

Image:Hairy cell leukemia - high mag.jpg | HCL - high mag. (WC)

Image:Hairy_cell_leukemia_-_very_high_mag.jpg | HCL - very high mag. (WC)

</gallery>

www:

*[http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=figure&id=A34027 HCL - bone marrow (nlm.nih.gov)] from [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 Holland-Frei Cancer Medicine (nlm.nih.gov)].

*[http://path.upmc.edu/cases/case240.html HCL - several images (upmc.edu)].

*[http://path.upmc.edu/cases/case691.html HCL - another case with several images (upmc.edu)].

*[http://webpathology.com/image.asp?case=380&n=3 HCL in the spleen (webpathology.com)].

==IHC==

Features:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029]. Accessed on: 20 August 2010.</ref>

*CD20 +ve, CD11c+ve, CD25 +ve, CD103 +ve, CD123 +ve, cyclin D1 +ve, DBA44 +ve

*Annexin A1 +ve (only useful in heavy bone marrow infiltration as it is also positive in T-cells and granulopoietic cells)

*TRAP - tartrate-resistant acid phosphatase (historically histochemically, now immunohistochemically)

*[[HBME1]]<ref name=pmid24092261>{{cite journal |authors=Krenács L, Tóth-Lipták J, Demeter J, Piukovics K, Borbényi Z, Gogolák P, Sári E, Bagdi E |title=Monoclonal antibody HBME-1 reacts with a minor subset of B cells with villous surface and can be useful in the diagnosis of hairy cell leukemia and other indolent lymphoproliferations of villous B lymphocytes |journal=Virchows Arch. |volume=463 |issue=6 |pages=787–94 |date=December 2013 |pmid=24092261 |doi=10.1007/s00428-013-1490-5 |url=}}</ref>

*BRAF V600E mutation-specific antibody

*CD5 -ve.

Flow cytometry:

*CD19 +ve, CD11c +ve, FMC7 +ve.

==See also==

*[[Small cell lymphomas]].

==References==

[[Category: Haematopathology]]

@@ Line 1: / Line 1: @@
-#redirect [[Small_cell_lymphomas#Hairy_cell_leukemia]]
+[[File:Bone marrow infiltration by hairy cell leukaemia.jpg|thumb|High power magnification of a bone marrow trephine showing infiltration by hairy cell leukaemia.]]
+Indolent B-cell neoplasm, abbreviated ''HCL''. Named for the appearances on peripheral blood smears. The vast majority (>95%) harbour the BRAF V600E mutation.<ref name=pmid25992240>{{Cite journal  | last1 = Ahmadzadeh | first1 = A. | last2 = Shahrabi | first2 = S. | last3 = Jaseb | first3 = K. | last4 = Norozi | first4 = F. | last5 = Shahjahani | first5 = M. | last6 = Vosoughi | first6 = T. | last7 = Hajizamani | first7 = S. | last8 = Saki | first8 = N. | title = BRAF Mutation in Hairy Cell Leukemia. | journal = Oncol Rev | volume = 8 | issue = 2 | pages = 253 | month = Sep | year = 2014 | doi = 10.4081/oncol.2014.253 | PMID = 25992240 }}</ref> Mutations in other parts of the BRAF gene are described, but rare.<ref name=pmid24433452>{{Cite journal  | last1 = Tschernitz | first1 = S. | last2 = Flossbach | first2 = L. | last3 = Bonengel | first3 = M. | last4 = Roth | first4 = S. | last5 = Rosenwald | first5 = A. | last6 = Geissinger | first6 = E. | title = Alternative BRAF mutations in BRAF V600E-negative hairy cell leukaemias. | journal = Br J Haematol | volume = 165 | issue = 4 | pages = 529-33 | month = May | year = 2014 | doi = 10.1111/bjh.12735 | PMID = 24433452 }}</ref>
-[[Category:Diagnosis]]
+In the bone marrow, they are associated with heavy reticulin fibrosis, commonly resulting in ''dry taps''.<ref name=pmid22499303>{{Cite journal  | last1 = Galani | first1 = KS. | last2 = Subramanian | first2 = PG. | last3 = Gadage | first3 = VS. | last4 = Rahman | first4 = K. | last5 = Ashok Kumar | first5 = MS. | last6 = Shinde | first6 = S. | last7 = Mahadik | first7 = S. | last8 = Ansari | first8 = R. | last9 = Sengar | first9 = M. | title = Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital. | journal = Indian J Pathol Microbiol | volume = 55 | issue = 1 | pages = 61-5 | month =  | year =  | doi = 10.4103/0377-4929.94858 | PMID = 22499303 }}</ref>
+Clinical:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022]. Accessed on: 20 August 2010.</ref>
+*Pancytopenia. Monocytopenia common in earlier disease.
+*Splenic enlargement.
+*No lymphadenopathy.
+*Good prognosis (with treatment), though (likely) not curable.
+==Gross==
+Features:<ref name=Ref_PCPBoD8_326>{{Ref PCPBoD8|326}}</ref>
+*Huge beefy red [[spleen]].
+**Red as white pulp obliterated.
+==Microscopic==
+Features:<ref>URL: [http://emedicine.medscape.com/article/200580-diagnosis http://emedicine.medscape.com/article/200580-diagnosis]. Accessed on: 18 August 2010.</ref>
+*Small cells (10-20 micrometers) with "Fried egg"-like appearance:
+**Well-demarcated fuzzy cell borders,
+**Clear/whispy cytoplasm and,
+**Central round nucleus.
+***Peri-nuclear clearing ("water-clear rim"<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022]. Accessed on: 20 August 2010.</ref>) -- '''key feature'''.
+DDx:
+*[[Hepatosplenic T-cell lymphoma]].
+===Images===
+<gallery>
+Image:Hairy_cell_leukemia.jpg | HCL - blood film. (WC)
+Image:Hairy cell leukemia - high mag.jpg | HCL - high mag. (WC)
+Image:Hairy_cell_leukemia_-_very_high_mag.jpg | HCL - very high mag. (WC)
+</gallery>
+www:
+*[http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=figure&id=A34027 HCL - bone marrow (nlm.nih.gov)] from [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022 Holland-Frei Cancer Medicine (nlm.nih.gov)].
+*[http://path.upmc.edu/cases/case240.html HCL - several images (upmc.edu)].
+*[http://path.upmc.edu/cases/case691.html HCL - another case with several images (upmc.edu)].
+*[http://webpathology.com/image.asp?case=380&n=3 HCL in the spleen (webpathology.com)].
+==IHC==
+Features:<ref>URL: [http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029 http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029]. Accessed on: 20 August 2010.</ref>
+*CD20 +ve, CD11c+ve, CD25 +ve, CD103 +ve, CD123 +ve, cyclin D1 +ve, DBA44 +ve
+*Annexin A1 +ve (only useful in heavy bone marrow infiltration as it is also positive in T-cells and granulopoietic cells)
+*TRAP - tartrate-resistant acid phosphatase (historically histochemically, now immunohistochemically)
+*[[HBME1]]<ref name=pmid24092261>{{cite journal |authors=Krenács L, Tóth-Lipták J, Demeter J, Piukovics K, Borbényi Z, Gogolák P, Sári E, Bagdi E |title=Monoclonal antibody HBME-1 reacts with a minor subset of B cells with villous surface and can be useful in the diagnosis of hairy cell leukemia and other indolent lymphoproliferations of villous B lymphocytes |journal=Virchows Arch. |volume=463 |issue=6 |pages=787–94 |date=December 2013 |pmid=24092261 |doi=10.1007/s00428-013-1490-5 |url=}}</ref>
+*BRAF V600E mutation-specific antibody
+*CD5 -ve.
+Flow cytometry:
+*CD19 +ve, CD11c +ve, FMC7 +ve.
+==See also==
+*[[Small cell lymphomas]].
+==References==
+{{Reflist}}
+[[Category: Haematopathology]]

Hairy cell leukemia: Difference between revisions

Navigation menu

Search