Hairy cell leukemia: Uncommon clinical features, unusual sites of involvement and some rare associations

Tadmor, Tamar; Polliack, Aaron

doi:10.1016/j.beha.2015.10.020

Cited by 18 publications

(19 citation statements)

References 40 publications

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“…To our knowledge, this is the first documented case of leukemic involvement of the brain by hairy cell leukemiavariant. Brain involvement is also rare in patients with classical hairy cell leukemia [20,21].…”

Section: Discussionmentioning

confidence: 99%

Clinicopathologic and molecular features in hairy cell leukemia-variant: single institutional experience

et al. 2018

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Hairy cell leukemia-variant is rare. Only a small number of cases have been reported in the literature with little cytogenetic or molecular data available. In this study, we describe the clinicopathologic and genetic features of 23 patients with hairy cell leukemia-variant (16 men and 7 women) with a median age of 70 years. Most patients had splenomegaly (90%), leukocytosis (77%), and lymphocytosis (82%); no patients had monocytopenia. Histologically, the bone marrow biopsy specimens showed a mixed pattern of predominantly interstitial and lesser intrasinusoidal infiltration by leukemic cells. In bone marrow aspirate smears most cells had villous cytoplasmic features and a small nucleolus. We describe unusual sites of hairy cell leukemia-variant involvement in 4 patients, including brain, omentum, terminal ileum, and skin at the time of initial presentation. Immunophenotyping showed monotypic B-cells positive for pan B-cell antigens, CD11c, and CD103, and negative for CD25 and annexin A1. Conventional cytogenetic or fluorescence in situ hybridization analysis showed deletions of 17p13/TP53 and 11q22/ATM gene in 5/12 (42%) and 2/9 (22%) cases, respectively. Sequencing of the variable region of IGVH showed mutations (>2% deviation from germline) in 40% of the cases assessed. MAP2K1 mutation (p.C121S) was seen in 1 of 14 (7%) patients tested. No BRAF V600E mutations were detected. The patients were treated in a heterogeneous manner, but most often with therapies designed for classical hairy cell leukemia and the 5-year overall survival was 84%. In summary, hairy cell leukemia-variant exhibits a heterogeneous spectrum of clinical, morphologic, immunophenotypic, and genetic features that may overlap with classic hairy cell leukemia and other hairy cell-like B-cell neoplasms. A subset of patients can have an aggressive clinical course. In our experience MAP2K1 mutations are uncommon in this disease.

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Section: Discussionmentioning

confidence: 99%

Clinicopathologic and molecular features in hairy cell leukemia-variant: single institutional experience

et al. 2018

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“…[18][19][20][21][22] Clinically significant extranodal involvement by HCL is rare, and may occur at presentation or in patients with longstanding disease. 8 Affected sites include lytic bone lesions, 23-25 central nervous system, [26][27][28] skin, 29 breast 9,30,31 and salivary gland. 10 Autopsy studies have revealed leukaemic infiltration in multiple organs, although typically without contribution to morbidity or mortality.…”

Section: Discussionmentioning

confidence: 99%

“…As such, detailed descriptions of the morphological features of nodal involvement by HCL are lacking . Clinically detectable extranodal lesions caused by HCL are even more rare, limited to isolated case reports …”

Section: Introductionmentioning

confidence: 99%

“…7 Clinically detectable extranodal lesions caused by HCL are even more rare, limited to isolated case reports. [8][9][10] Given that involvement by HCL is unexpected at nodal and extranodal sites, such specimens can prove to be a diagnostic dilemma for pathologists, particularly in patients without a preceding diagnosis of HCL. 10 Confounding interpretation further is the fact that HCL expresses markers associated typically with other small B cell lymphomas, such as CD10 in 15-25% of cases 11 and cyclin D1 in most cases.…”

Section: Introductionmentioning

confidence: 99%

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Morphological and immunophenotypical features of hairy cell leukaemia involving lymph nodes and extranodal tissues

et al. 2017

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Although uncommon, HCL should be considered in the differential diagnosis of small B cell neoplasms involving nodal/extranodal sites, given the therapeutic implications. In particular, recent discoveries including detection of the BRAF mutation in nearly all cases of HCL and the availability of an antibody to CD103 for use in paraffin-embedded tissues will facilitate the distinction of HCL from other small B cell lymphomas in the nodal/extranodal setting.

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“…Hairy cell leukemia (HCL) has been recognized as an indolent B cell leukemia characterized by splenomegaly, pancytopenia, and neoplastic cells morphologically with irregular cytoplasmic hair-like projections on smears [ 1 ]. However, body fluid retention, such as ascites and pleural effusion, are extremely rare in HCL [ 2 – 4 ].…”

mentioning

confidence: 99%

Hairy cell leukemia presenting with progressive pericarditis and pleuritis

2018

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Hairy cell leukemia: Uncommon clinical features, unusual sites of involvement and some rare associations

Cited by 18 publications

References 40 publications

Clinicopathologic and molecular features in hairy cell leukemia-variant: single institutional experience

Clinicopathologic and molecular features in hairy cell leukemia-variant: single institutional experience

Morphological and immunophenotypical features of hairy cell leukaemia involving lymph nodes and extranodal tissues

Hairy cell leukemia presenting with progressive pericarditis and pleuritis

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