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

Cortazar, Jacqueline M; DeAngelo, Daniel J.; Pinkus, Geraldine S.; Morgan, Elizabeth A.

doi:10.1111/his.13206

Cited by 10 publications

(9 citation statements)

References 39 publications

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“…Cortazar et al reported that the immunophenotype of nodal/extranodal diseases overlapped with that of other small B-cell lymphomas; proteins expressed by both sets of diseases included CD5 and cyclin D1( 3 ). MCL is a mature B-cell neoplasm that accounts for 3-10% of malignant lymphomas.…”

Section: Discussionmentioning

confidence: 99%

“…The HCL immunophenotypic profile is characterized by the expression of CD19, CD20, CD22, and CD200. Cyclin D1 is expressed in approximately 50-70% of cases, whereas CD5 is weakly expressed in only approximately 0-2% ( 1 , 3 ). The BRAF V600E gene mutation is found in >97% of HCL cases ( 4 ), and is associated with poor prognosis ( 2 ).…”

Section: Introductionmentioning

confidence: 99%

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A rare case of hairy cell leukemia with co‑expression of CD5 and cyclin D1: A diagnostic pitfall

Zhou

et al. 2020

Mol Clin Oncol

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Hairy cell leukemia (HCL) is an uncommon chronic B-cell lymphoproliferative disease with an indolent course. It mainly occurs in elderly men, although abdominal lymphadenopathy is rare. HCL cells are mostly found in the bone marrow, peripheral blood, and spleen and typically express CD11c, CD20, CD25 and CD103. We present a case of HCL with a novel immunophenotype. A 48-year-old woman presented with pancytopenia and splenomegaly. The diagnosis was HCL with lymph node infiltration. Unlike previously described HCL cases, the current case showed strong expression of CD5 and cyclin D1 in the lymph nodes. The patient underwent cladribine chemotherapy, and the leukocyte count increased during and after treatment. The 8-month follow-up revealed that she had recovered well. This case highlights the distinctive immunophenotype of HCL infiltrating the lymph nodes and the potential misdiagnosis of HCL as mantle cell lymphoma. It also adds to our limited understanding of HCL.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

A rare case of hairy cell leukemia with co‑expression of CD5 and cyclin D1: A diagnostic pitfall

Zhou

et al. 2020

Mol Clin Oncol

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“…Unusual markers in classic HCL like CD38 have been associated with shorter time to progression and next treatment [ 75 ]. Other aberrant markers like CD5 or CD10 have not shown prognostic implications [ [76] , [77] , [78] , [79] ].…”

Section: How To Tell Hcl From Variantsmentioning

confidence: 99%

Diagnosis and treatment of hairy cell leukemia as the COVID-19 pandemic continues

Kreitman

Arons

2022

Blood Reviews

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Hairy cell leukemia (HCL) is an indolent B-cell malignancy, usually driven by the BRAF V600E mutation. For 30 years, untreated and relapsed HCL was successfully treated with purine analogs, but minimal residual disease (MRD) remained in most patients, eventually causing relapse. Repeated purine analogs achieve decreasing efficacy and increasing toxicity, particularly to normal T-cells. MRD-free complete remissions (CRs) are more common using rituximab with purine analogs in both 1 st -line and relapsed settings. BRAF inhibitors and Ibrutinib can achieve remission, but due to persistence of MRD, must be used chronically to prevent relapse. BRAF inhibition combined with Rituximab can achieve high MRD-free CR rates. Anti-CD22 recombinant immunotoxin moxetumomab pasudotox is FDA-approved in the relapsed setting and is unique in achieving high MRD-free CR rates as a single-agent. Avoiding chemotherapy and rituximab may be important in ensuring both recovery from COVID-19 and successful COVID-19 vaccination, an area of continued investigation.

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“…The detection of Annexin A1, TRAP, BRAF and CD72/DBA.44 by IHC may contribute to the diagnosis. The expression of CD5 is exceedingly rare but was described in up to 2% of cases [ 125 , 126 , 127 ]. Together with the positivity for Cyclin D1 in half of the patients and the possible presence of lymphadenopathy, CD5-positive cases can be misdiagnosed as MCL.…”

Section: Lymphomas Rarely Expressing Cd5mentioning

confidence: 99%

How to Diagnose and Treat CD5-Positive Lymphomas Involving the Spleen

et al. 2021

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Patients with CD5-expressing lymphomas presenting with splenomegaly are frequently diagnosed with chronic lymphocytic leukemia. The most important differential diagnosis is mantle cell lymphoma, both in its classical and leukemic, non-nodal forms, given its prognostic and therapeutic implications. Other small B-cell neoplasms that frequently involve the spleen and occasionally express CD5 include the splenic marginal zone lymphoma, hairy cell leukemia and, rarely, lymphoplasmacytic lymphoma. The frequency of CD5 positivity depends in part on the sensitivity of the detection methods employed. Usually, a combination of morphological, immunophenotypic and molecular findings allows for a precise sub-classification of CD5-positive, low-grade B-cell lymphomas of the spleen. Some of these tumors may display a mixture of small and larger B cells, raising the possibility of more aggressive lymphomas, such as diffuse large B-cell lymphomas (DLBCL). Approximately 5–10% of DLBCL are CD5-positive and some may manifest as primary splenic lesions. When available, the morphology of DLBCL in the splenic tissue is distinctive and a leukemic picture is very rare. In conclusion, the appropriate morphological and clinical context assisted by flow cytometry panels and/or immunohistochemistry allows the differential diagnosis of CD5-positive, non-Hodgkin, B-cell lymphomas involving the spleen.

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

Cited by 10 publications

References 39 publications

A rare case of hairy cell leukemia with co‑expression of CD5 and cyclin D1: A diagnostic pitfall

A rare case of hairy cell leukemia with co‑expression of CD5 and cyclin D1: A diagnostic pitfall

Diagnosis and treatment of hairy cell leukemia as the COVID-19 pandemic continues

How to Diagnose and Treat CD5-Positive Lymphomas Involving the Spleen

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