An Assessment of the Usefulness of Immunohistochemical Stains in the Diagnosis of Hairy Cell Leukemia

Sherman, Michael; Hanson, Curtis A.; Hoyer, James D.

doi:10.1309/ajcp5ge1psbmbztw

Cited by 51 publications

(35 citation statements)

References 12 publications

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“…23,24 It is noteworthy that in extensive immunohistochemical staining for ANXA1 and other diagnostic markers in HCL cases showing weak or focal staining were scored as positive. 18 These criteria affirm ANXA1 expression in the HCLL-7876 cell line, and indicate that its expression in the other three cell lines (Eskol, HC-1 and Hair-M) can also be considered positive. Two cell lines (EH and HCLv-07) were clearly negative for ANXA1 (Figures 1a and b) by western blot analysis, an expected phenotype for HCLv-07.…”

mentioning

confidence: 82%

“…6 Under the WHO (World Health Organization) (2008) guidelines, HCL immunophenotype includes bright CD11c expression with CD103, CD25, CD123 and Annexin A1 (ANXA1) coexpression. 16 ANXA1 is regarded as the most specific tumor marker for HCL, as it is absent on other B-cell lymphomas [16][17][18] and is negative in HCLv to provide a highly relevant marker to segregate these two forms of disease. 16,17 Immunostaining for tartrate-resistant acid phosphatase (TRAP) is also a diagnostic tool for HCL.…”

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confidence: 99%

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Hairy cell leukemia cell lines expressing annexin A1 and displaying B-cell receptor signals characteristic of primary tumor cells lack the signature BRAF mutation to reveal unrepresentative origins

et al. 2012

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confidence: 82%

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confidence: 99%

Hairy cell leukemia cell lines expressing annexin A1 and displaying B-cell receptor signals characteristic of primary tumor cells lack the signature BRAF mutation to reveal unrepresentative origins

et al. 2012

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“…The characteristic immunophenotype of CD19 and CD123, and most of these patients will not be monocytopenic. 32,35 A trephine bone marrow biopsy and aspirate are important for understanding the extent of bone marrow infiltration. At diagnosis, a successful bone marrow aspirate is often not attainable because of a dry tap, in which extensive fibrosis precludes the ability to obtain a cellular aspirate.…”

Section: Establishing the Diagnosismentioning

confidence: 99%

“…Differential diagnosis Consider hairy cell leukemia; hairy cell leukemia variant; splenic marginal zone lymphoma; splenic diffuse red pulp small B-cell lymphoma (outlines for specific immunophenotypic profiles of differential entities [33][34][35][36] ).…”

Section: Determination Of Mrd After Therapymentioning

confidence: 99%

Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia

Grever

Abdel‐Wahab

Andritsos

et al. 2017

Blood

220

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Hairy cell leukemia is an uncommon hematologic malignancy characterized by pancytopenia and marked susceptibility to infection. Tremendous progress in the management of patients with this disease has resulted in high response rates and improved survival, yet relapse and an appropriate approach to re-treatment present continuing areas for research. The disease and its effective treatment are associated with immunosuppression. Because more patients are being treated with alternative programs, comparison of results will require general agreement on definitions of response, relapse, and methods of determining minimal residual disease. The development of internationally accepted, reproducible criteria is of paramount importance in evaluating and comparing clinical trials to provide optimal care. Despite the success achieved in managing these patients, continued participation in available clinical trials in the first-line and particularly in the relapse setting is highly recommended. The Hairy Cell Leukemia Foundation convened an international conference to provide common definitions and structure to guide current management. There is substantial opportunity for continued research in this disease. In addition to the importance of optimizing the prevention and management of the serious risk of infection, organized evaluations of minimal residual disease and treatment at relapse offer ample opportunities for clinical research. Finally, a scholarly evaluation of quality of life in the increasing number of survivors of this now manageable chronic illness merits further study. The development of consensus guidelines for this disease offers a framework for continued enhancement of the outcome for patients

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“…53,54 Moreover, the constitutive activation of the RAF-MEK-ERK pathway likely contributes to some of the immunophenotypic features of HCL, such as expression of cyclin D1 [55][56][57] and negativity for p27.…”

Section: Introductionmentioning

confidence: 99%

BRAF V600E mutation in hairy cell leukemia: from bench to bedside

Falini

Martelli

Tiacci

2016

Blood

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Hairy cell leukemia (HCL) is a distinct clinicopathological entity whose underlying genetic lesion has remained a mystery for over half a century. The BRAF V600E mutation is now recognized as the causal genetic event of HCL because it is somatic, present in the entire tumor clone, detectable in almost all cases at diagnosis (encompassing the whole disease spectrum), and stable at relapse. BRAF V600E leads to the constitutive activation of the RAF-MEK-extracellular signal-regulated kinase (ERK) signaling pathway which represents the key event in the molecular pathogenesis of HCL. KLF2 and CDNK1B (p27) mutations may cooperate with BRAF V600E in promoting leukemic transformation. Sensitive molecular assays for detecting BRAF V600E allow HCL (highly responsive to purine analogs) to be better distinguished from HCL-like disorders, which are treated differently. In vitro preclinical studies on purified HCL cells proved that BRAF and MEK inhibitors can induce marked dephosphorylation of MEK/ERK, silencing of RAF-MEK-ERK pathway transcriptional output, loss of the HCL-specific gene expression profile signature, change of morphology from “hairy” to “smooth,” and eventually apoptosis. The overall response rate of refractory/relapsed HCL patients to the BRAF inhibitor vemurafenib approached 100%, with 35% to 40% complete remissions (CRs). The median relapse free-survival was about 19 months in patients who had achieved CR and 6 months in those who had obtained a partial response. Future therapeutic perspectives include: (1) combining BRAF inhibitors with MEK inhibitors or immunotherapy (anti-CD20 monoclonal antibody) to increase the percentage of CRs and (2) better understanding of the molecular mechanisms underlying resistance of HCL cells to BRAF inhibitors.

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An Assessment of the Usefulness of Immunohistochemical Stains in the Diagnosis of Hairy Cell Leukemia

Cited by 51 publications

References 12 publications

Hairy cell leukemia cell lines expressing annexin A1 and displaying B-cell receptor signals characteristic of primary tumor cells lack the signature BRAF mutation to reveal unrepresentative origins

Hairy cell leukemia cell lines expressing annexin A1 and displaying B-cell receptor signals characteristic of primary tumor cells lack the signature BRAF mutation to reveal unrepresentative origins

Consensus guidelines for the diagnosis and management of patients with classic hairy cell leukemia

BRAF V600E mutation in hairy cell leukemia: from bench to bedside

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