Hairy cell leukemia classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2], Vamsikrishna Gunnam M.B.B.S [3], James Nasr[4]; Grammar Reviewer: Natalie Harpenau, B.S.[5]

Overview

  • Hairy cell leukemia is a distinct, indolent, small, mature B-cell neoplasm. [1]

Classification

  • Hairy cell leukemia is a distinct, indolent, small, mature B-cell neoplasm. [1]
  • Several splenic B-cell neoplasms may mimic its clinical and morphologic features, including hairy cell leukemia variant, also referred to as splenic B-cell lymphoma or leukemia with prominent nucleoli.[2]
  • The chart below illustrates characteristic findings associated with hairy cell leukemia and splenic B-cell lymphoma:[3][4][5][6][7]
 
 
 
 
 
 
 
Differential diagnosis of hairy cell leukemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Hairy cell leukemia
 
Splenic B-cell lymphoma
 
 
 
 
 
  • The male to female ratio is approximately 4 to 1
  • The median age at diagnosis is between 55 and 60 years
  • Leukocytosis is observed in 10-15% of patients
  • Decreased level of monocyte on peripheral blood smear
  • Decreased hemoglobin level in approximately 85% of the patients
  • Thrombocytopenia is present in approximately 80% of the patients
  • CD103 +ve, CD11c +ve, and CD25 +ve on immunophenotype
  • DBA 44, and Annexin A1 are positive on immunohistochemistry, Cyclin D1 may be variably expressed
  • Presence of BRAF V600E mutation detectable by molecular or immunohistochemical methods.
 
  • The male to female ratio is approximately 2 to 1
  • The median age at diagnosis is greater than 70 years
  • Greater than 90% lymphocytosis is observed
  • Normal level of monocyte on peripheral blood smear
  • Normal hemoglobin level in most of the patients
  • Normal platelets count in most of the patients
  • CD103 +ve, CD11c +ve, and CD25 -ve on immunophenotype
  • DBA 44 +ve, and Annexin A1 -ve on immunohistochemistry
  • Absence of BRAF V600E mutation



References

  1. ↑ 1.0 1.1 Teras LR, DeSantis CE, Cerhan JR, Morton LM, Jemal A, Flowers CR. 2016 US lymphoid malignancy statistics by World Health Organization subtypes. CA Cancer J Clin. 2016 Nov 12;66(6):443-459. doi: 10.3322/caac.21357. Epub 2016 Sep 12. PMID: 27618563.
  2. ↑ Turakhia S, Lanigan C, Hamadeh F, Swerdlow SH, Tubbs RR, Cook JR. Immunohistochemistry for BRAF V600E in the Differential Diagnosis of Hairy Cell Leukemia vs Other Splenic B-Cell Lymphomas. Am J Clin Pathol. 2015 Jul;144(1):87-93. doi: 10.1309/AJCP5WVXJ2KTLODO. PMID: 26071465.
  3. ↑ Epperla N, Zhao Q, Anghelina M, Neal J, Blachly JS, Rogers KA, Lozanski G, Oakes CC, Bhat SA, Zent CS, Banerji V, Grever M, Andritsos LA. Impact of sex on outcomes in patients with hairy cell leukemia. An HCL patient data registry analysis. Am J Hematol. 2023 May;98(5):E116-E118. doi: 10.1002/ajh.26881. Epub 2023 Feb 24. PMID: 36808760.
  4. ↑ Matutes E (2006). "Immunophenotyping and differential diagnosis of hairy cell leukemia". Hematol Oncol Clin North Am. 20 (5): 1051–63. doi:10.1016/j.hoc.2006.06.012. PMID 16990106.
  5. ↑ Kraut E. Infectious complications in hairy cell leukemia. Leuk Lymphoma. 2011 Jun;52 Suppl 2:50-2. doi: 10.3109/10428194.2011.570819. Epub 2011 Apr 19. PMID: 21504285.
  6. ↑ Chung SS, Kim E, Park JH, Chung YR, Lito P, Teruya-Feldstein J, Hu W, Beguelin W, Monette S, Duy C, Rampal R, Telis L, Patel M, Kim MK, Huberman K, Bouvier N, Berger MF, Melnick AM, Rosen N, Tallman MS, Park CY, Abdel-Wahab O. Hematopoietic stem cell origin of BRAFV600E mutations in hairy cell leukemia. Sci Transl Med. 2014 May 28;6(238):238ra71. doi: 10.1126/scitranslmed.3008004. PMID: 24871132; PMCID: PMC4501573.
  7. ↑ Waterfall JJ, Arons E, Walker RL, Pineda M, Roth L, Killian JK, Abaan OD, Davis SR, Kreitman RJ, Meltzer PS. High prevalence of MAP2K1 mutations in variant and IGHV4-34-expressing hairy-cell leukemias. Nat Genet. 2014 Jan;46(1):8-10. doi: 10.1038/ng.2828. Epub 2013 Nov 17. PMID: 24241536; PMCID: PMC3905739.


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