Cargando…

Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients

Introduction: Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder of the mature B-cells, mostly seen in men, and is characterized by cytopenia, splenomegaly, myelofibrosis, and the presence of atypical lymphoid cells showing the cytoplasmic hairy projection in the peripheral blood, bone...

Descripción completa

Detalles Bibliográficos
Autores principales: Bhatti, Vandana, Kaur, Gurpreet, Ahuja, Aarzoo, Isaacs, Roma
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10560564/
https://www.ncbi.nlm.nih.gov/pubmed/37814728
http://dx.doi.org/10.7759/cureus.44876
_version_ 1785117755973828608
author Bhatti, Vandana
Kaur, Gurpreet
Ahuja, Aarzoo
Isaacs, Roma
author_facet Bhatti, Vandana
Kaur, Gurpreet
Ahuja, Aarzoo
Isaacs, Roma
author_sort Bhatti, Vandana
collection PubMed
description Introduction: Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder of the mature B-cells, mostly seen in men, and is characterized by cytopenia, splenomegaly, myelofibrosis, and the presence of atypical lymphoid cells showing the cytoplasmic hairy projection in the peripheral blood, bone marrow, and spleen. The immunophenotypic (IPT) profile shows the clonal expansion of B-cells with CD19, CD20, and CD22 showing bright expression. The diagnosis requires two hairy cell markers out of CD103, CD123, CD25, and CD11c to be positive. The HCL variant (HCL-v) has a different IPT profile with negative CD25 in most cases. Aim: The aim was to study the hematological and IPT of classical HCL and HCL variants. Methods: This cross-sectional study included all the cases of HCL diagnosed over a retrospective period of eight years from 1st January 2015 to 31st December 2022 in a tertiary care hospital in north India. The patients included in the study were those for whom immunophenotyping; that is, flow cytometry and/or immunohistochemistry (IHC) were done for diagnosis. Bone marrow slides, IHC slides, and flow cytometric IPTs were reviewed. Results: The study included 13 patients who were diagnosed to have HCL, of which 12 were classical HCL and one was HCL-variant (HCL-v). Among classical HCL, IPT was done by flow cytometry in 10 patients, while in two patients, it was done by IHC. CD19, CD20, and CD22 were positive in all patients of classical HCL (10/10, 10/10, and 5/5, respectively), while CD123, CD103, CD25, and CD11C were positive in 100%, 89%, 80%, and 100% cases, respectively. One patient of HCL-v had CD103 and CD123 positive, while CD25 and CD123 were negative. Conclusion: The diagnosis of HCL requires a multipronged approach. The use of clinical features, morphology, and immunophenotyping combined with ancillary techniques provides higher diagnostic accuracy and enables its distinction from other B-cell lymphoproliferative disorders (BCLPDs), leading to better patient management and treatment.
format Online
Article
Text
id pubmed-10560564
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Cureus
record_format MEDLINE/PubMed
spelling pubmed-105605642023-10-09 Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients Bhatti, Vandana Kaur, Gurpreet Ahuja, Aarzoo Isaacs, Roma Cureus Pathology Introduction: Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder of the mature B-cells, mostly seen in men, and is characterized by cytopenia, splenomegaly, myelofibrosis, and the presence of atypical lymphoid cells showing the cytoplasmic hairy projection in the peripheral blood, bone marrow, and spleen. The immunophenotypic (IPT) profile shows the clonal expansion of B-cells with CD19, CD20, and CD22 showing bright expression. The diagnosis requires two hairy cell markers out of CD103, CD123, CD25, and CD11c to be positive. The HCL variant (HCL-v) has a different IPT profile with negative CD25 in most cases. Aim: The aim was to study the hematological and IPT of classical HCL and HCL variants. Methods: This cross-sectional study included all the cases of HCL diagnosed over a retrospective period of eight years from 1st January 2015 to 31st December 2022 in a tertiary care hospital in north India. The patients included in the study were those for whom immunophenotyping; that is, flow cytometry and/or immunohistochemistry (IHC) were done for diagnosis. Bone marrow slides, IHC slides, and flow cytometric IPTs were reviewed. Results: The study included 13 patients who were diagnosed to have HCL, of which 12 were classical HCL and one was HCL-variant (HCL-v). Among classical HCL, IPT was done by flow cytometry in 10 patients, while in two patients, it was done by IHC. CD19, CD20, and CD22 were positive in all patients of classical HCL (10/10, 10/10, and 5/5, respectively), while CD123, CD103, CD25, and CD11C were positive in 100%, 89%, 80%, and 100% cases, respectively. One patient of HCL-v had CD103 and CD123 positive, while CD25 and CD123 were negative. Conclusion: The diagnosis of HCL requires a multipronged approach. The use of clinical features, morphology, and immunophenotyping combined with ancillary techniques provides higher diagnostic accuracy and enables its distinction from other B-cell lymphoproliferative disorders (BCLPDs), leading to better patient management and treatment. Cureus 2023-09-07 /pmc/articles/PMC10560564/ /pubmed/37814728 http://dx.doi.org/10.7759/cureus.44876 Text en Copyright © 2023, Bhatti et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Pathology
Bhatti, Vandana
Kaur, Gurpreet
Ahuja, Aarzoo
Isaacs, Roma
Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients
title Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients
title_full Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients
title_fullStr Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients
title_full_unstemmed Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients
title_short Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients
title_sort hairy cell leukemia: hematological and immunophenotypic profile of 13 patients
topic Pathology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10560564/
https://www.ncbi.nlm.nih.gov/pubmed/37814728
http://dx.doi.org/10.7759/cureus.44876
work_keys_str_mv AT bhattivandana hairycellleukemiahematologicalandimmunophenotypicprofileof13patients
AT kaurgurpreet hairycellleukemiahematologicalandimmunophenotypicprofileof13patients
AT ahujaaarzoo hairycellleukemiahematologicalandimmunophenotypicprofileof13patients
AT isaacsroma hairycellleukemiahematologicalandimmunophenotypicprofileof13patients