Hairy Cell Leukemia: Diagnostic Pathology

Sharpe, Robert W.; Bethel, Kelly

doi:10.1016/j.hoc.2006.06.010

Cited by 49 publications

(39 citation statements)

References 61 publications

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Section: Hcl: Establishing the Diagnosis And Considering The Differenmentioning

confidence: 99%

“…21 It is important in planning the successful treatment course to establish the specific identity of the leukemic process and to assess the cellularity of the bone marrow to avoid therapeutic complications.…”

Section: Hcl: Establishing the Diagnosis And Considering The Differenmentioning

confidence: 99%

“…However, on close inspection of the remission bone marrow with immunologic probes, minimal residual disease (MRD) is still demonstrable in a varying percentage of cases (ranging from 15% to 50% or more depending on the method of detection used). 21,22 MRD is defined as identification of persistent HCL after treatment using immunophenotypic analysis, immunohistochemical staining, or DNA polymerase chain reaction in the absence of disease detectable by morphologic criteria.Multicolor 4-channel flow cytometry (eg, CD11c, CD25, CD103, CD20) is highly sensitive and specific for detecting low levels of hairy cells in either the peripheral blood or the bone marrow aspirate (detection limit estimated at 0.003%-0.05%). 23 Residual HCL can be difficult to identify with standard cytochemical staining.…”

mentioning

confidence: 99%

“…Patients with the hairy cell variant may actually have an elevated lymphocyte count and lack the characteristic monocytopenia. 21 Identification of specific therapy for those patients with the variant forms of the disease requires more study.In Table 2, the presentation of other chronic lymphoid malignancies that must be differentiated by flow cytometry from HCL is summarized. 21 It is important in planning the successful treatment course to establish the specific identity of the leukemic process and to assess the cellularity of the bone marrow to avoid therapeutic complications.…”

mentioning

confidence: 99%

“…21 Identification of specific therapy for those patients with the variant forms of the disease requires more study.…”

mentioning

confidence: 99%

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How I treat hairy cell leukemia

Grever

2010

Blood

139

124

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The description of hairy cell leukemia as a specific clinical entity was published 50 years ago. The clinical outcome for patients was hampered by ineffective chemotherapy, and splenectomy was the major therapeutic approach to improve peripheral blood counts. The median survival after diagnosis was 4 years. With the introduction of ␣-interferon in 1984, marked improvements in patient responses were observed. Shortly thereafter, the introduction of the purine nucleoside analogs transformed this disease into a highly treatable form of leukemia, and patients with the classic form of this rare leukemia now have a near-normal life expectancy. However, other clinical entities mimicking this disease do not respond; thus, accurate diagnosis is important. Immunophenotypic features in classic hairy cell leukemia show that the leukemic cells express CD11c, CD25, CD103, and CD123 and display bright CD20. Despite the high percentage of durable complete remissions with modern therapy, the long-term disease-free survival curves have not reached a plateau. Many patients who achieve a complete remission by morphologic criteria have minimal residual disease demonstrable by either flow cytometry or immunohistochemical staining, and this population may be at higher risk for earlier relapse. Continued clinical research is essential to optimize therapy for this disease. (Blood. 2010;115:21-28) IntroductionIn 1958, Bouroncle et al described a series of patients with leukemic reticuloendotheliosis. 1 Although this collection of cases established that the previously described isolated reports actually represented a distinct hematologic malignancy, the classic manuscript contained a very thorough presentation of the many clinical facets of this disease now known as hairy cell leukemia (HCL). Furthermore, it established that therapeutic intervention was limited either to careful titration of alkylating agents or to splenectomy. The ability to alter the clinical course of the patients with this rare form of leukemia did not substantially change until the introduction of ␣-interferon in 1984. 2 Shortly thereafter, observations that a purine nucleoside analog (pentostatin) could induce a high degree of complete remissions (eg, 75%-89%) in this previously "untreatable" chronic leukemia changed the natural history of HCL in record time. [3][4][5][6][7][8] Another purine nucleoside analog (cladribine) produced remarkably high remission rates (eg, 91%) with a single course of therapy. 9,10 The outstanding results with this agent delivered as a single course of therapy led to cladribine being the initial therapy selected by most hematologists. Many of the initial studies with this agent excluded patients with active infection from enrollment, but studies from multiple institutions confirmed the high complete remission rate with this drug. [11][12][13][14] Thus, patients who are treated with either purine nucleoside analog as front-line therapy will achieve a high rate of complete remission (75%-90%). 7,10 The long-term studies reported at 5 to 10...

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Section: Hcl: Establishing the Diagnosis And Considering The Differenmentioning

confidence: 99%

Section: Hcl: Establishing the Diagnosis And Considering The Differenmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

“…21 Identification of specific therapy for those patients with the variant forms of the disease requires more study.…”

mentioning

confidence: 99%

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How I treat hairy cell leukemia

Grever

2010

Blood

139

124

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Current Therapy and New Directions in the Treatment of Hairy Cell Leukemia

2016

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Hairy cell leukemia (HCL) is a chronic B-cell leukemia noted for an indolent course that ultimately results in cytopenias and massive splenomegaly. Whereas treatment with the nucleoside purine analogues cladribine and pentostatin results in lengthy remissions in nearly all patients with HCL, most patients will experience relapse while a small percentage of patients' disease fails to respond to therapy in the first place. Retreatment with a purine nucleoside analogue often leads to an effective but limited response. For decades, few other viable therapeutic options were available to these patients who required retreatment. Recently, new insights into the mechanism of disease of HCL have led to research in new potential treatment agents, either alone or with a purine nucleoside analogue. Clinical trials with rituximab, bendamustine, and conjugate immunotoxins will reveal what role these therapies will have in HCL treatment. A better understanding of the BRAF/MEK/ERK pathway and the B-cell signaling pathway has allowed further exploration into the novel drugs vemurafenib, dabrafenib, trametinib, and ibrutinib.

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Rare B Cell Lymphoproliferative Disorders

Barr

Friedberg

2012

Textbook of Uncommon Cancer

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Hairy Cell Leukemia: Diagnostic Pathology

Cited by 49 publications

References 61 publications

How I treat hairy cell leukemia

How I treat hairy cell leukemia

Current Therapy and New Directions in the Treatment of Hairy Cell Leukemia

Rare B Cell Lymphoproliferative Disorders

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