Difference between revisions of "Hairy cell leukemia"

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#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.


[[Category:Diagnosis]]
*Due to the biology, ''dry taps'' are common.<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
*CD5 -ve.
 
Flow cytometry:
*CD19 +ve, CD11c +ve, FMC7 +ve.

Revision as of 20:56, 16 June 2018

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.

  • Due to the biology, dry taps are common.[1]

Clinical:[2]

  • Pancytopenia. Monocytopenia common in earlier disease.
  • Splenic enlargement.
  • No lymphadenopathy.
  • Good prognosis (with treatment), though (likely) not curable.

Gross

Features:[3]

  • Huge beefy red spleen.
    • Red as white pulp obliterated.

Microscopic

Features:[4]

  • 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"[5]) -- key feature.

DDx:

Images

  • HCL - blood film. (WC)

  • HCL - high mag. (WC)

  • HCL - very high mag. (WC)

www:

IHC

Features:[6]

  • CD20 +ve, CD11c+ve, CD25 +ve, CD103 +ve, CD123 +ve, cyclin D1 +ve, DBA44 +ve
  • CD5 -ve.

Flow cytometry:

  • CD19 +ve, CD11c +ve, FMC7 +ve.
  1. ↑ Galani, KS.; Subramanian, PG.; Gadage, VS.; Rahman, K.; Ashok Kumar, MS.; Shinde, S.; Mahadik, S.; Ansari, R. et al. "Clinico-pathological profile of Hairy cell leukemia: critical insights gained at a tertiary care cancer hospital.". Indian J Pathol Microbiol 55 (1): 61-5. doi:10.4103/0377-4929.94858. PMID 22499303.
  2. ↑ URL: http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022. Accessed on: 20 August 2010.
  3. ↑ 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. 326. ISBN 978-1416054542.
  4. ↑ URL: http://emedicine.medscape.com/article/200580-diagnosis. Accessed on: 18 August 2010.
  5. ↑ URL: http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022. Accessed on: 20 August 2010.
  6. ↑ URL: http://www.ncbi.nlm.nih.gov/bookshelf/br.fcgi?book=cmed&part=A34022&rendertype=table&id=A34029. Accessed on: 20 August 2010.
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