Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Dec;92(12):1382-1390.
doi: 10.1002/ajh.24936.

Hairy cell leukemia 2018: Update on diagnosis, risk-stratification, and treatment

Xavier Troussard  1 , Edouard Cornet  1
Affiliations

Hairy cell leukemia 2018: Update on diagnosis, risk-stratification, and treatment

Xavier Troussard et al. Am J Hematol. 2017 Dec.

Abstract

Disease overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B-cell disorders, characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment.

Diagnosis: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11C, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation.

Risk stratification: Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood and the immunoglobulin heavy chain variable region gene mutational status. VH4-34 positive HCL cases are associated with poor prognosis RISK ADAPTED THERAPY: Purine analogs (PNA) are indicated in symptomatic first line HCL patients. The use of PNA followed by rituximab represents an alternative option.

Management of progressive or refractory disease: It is based on the use of BRAF inhibitors associated or not with MEK inhibitors, recombinant immunoconjugates targeting CD22 or BCR inhibitors.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Cytological aspects of hairy cell leukemia (HCL) (A) and HCL‐variant (HCL‐V) (B)
Figure 2
Figure 2
Therapeutic algorithm for the treatment of patients with hairy cell leukemia (HCL)
Figure 3
Figure 3
Therapeutic algorithm for treatment of patients with hairy cell leukemia‐variant (HCL‐V) and splenic diffuse red pulp lymphoma (SDRPL)
See this image and copyright information in PMC

References

    1. Foucar K, Falini B, Catovsky D, Stein H. Hairy cell leukemia In: Swerdlow SH, Campo E, Harris NL, eds. WHO Classification of Tumours of Haematopietic and Lymphoid Tissues. Lyon, France: IARC Press; 2008.
    1. Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood. 2016;127(20):2375–90. https://doi.org/10.1182/blood-2016-01-643569. - DOI - PMC - PubMed
    1. Robak T. Hairy‐cell leukemia variant: recent view on diagnosis, biology and treatment. Cancer Treat Rev. 2011;37(1):3–10. https://doi.org/10.1016/j.ctrv.2010.05.003. - DOI - PubMed
    1. Traverse‐Glehen A, Baseggio L, Bauchu EC, et al. Splenic red pulp lymphoma with numerous basophilic villous lymphocytes: a distinct clinicopathologic and molecular entity? Blood. 2008;111(4):2253–2260. https://doi.org/10.1182/blood-2007-07-098848. - DOI - PubMed
    1. Matutes E, Morilla R, Owusu‐Ankomah K, Houliham A, Meeus P, Catovsky D. The immunophenotype of hairy cell leukemia (HCL). Proposal for a scoring system to distinguish HCL from B‐cell disorders with hairy or villous lymphocytes. Leuk Lymphoma. 1994;14(Suppl 1):57–61. - PubMed

MeSH terms

LinkOut - more resources