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Hairy Cell Leukemia: Hematological and Immunophenotypic Profile of 13 Patients
Vandana Bhatti1, Gurpreet Kaur1, Aarzoo Ahuja1
1Department of Pathology, Christian Medical College & Hospital, Ludhiana, IND.
Insights
Hairy cell leukemia (HCL) diagnosis relies on a combination of clinical, morphological, and immunophenotypic (IPT) data. This study highlights the IPT profiles of classical HCL and HCL variants, crucial for accurate diagnosis and management.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Hairy cell leukemia (HCL) is a rare B-cell lymphoproliferative disorder.
- It is characterized by cytopenia, splenomegaly, and specific lymphoid cell morphology.
- Immunophenotyping (IPT) is key for diagnosis, with classical HCL typically showing bright CD19, CD20, and CD22 expression.
Purpose of the Study:
- To investigate the hematological and IPT characteristics of classical HCL and HCL variants.
- To differentiate between classical HCL and its variant using diagnostic markers.
Main Methods:
- A cross-sectional study analyzed HCL cases diagnosed between 2015 and 2022.
- Included patients underwent flow cytometry and/or immunohistochemistry (IHC) for diagnosis.
- Bone marrow slides, IHC slides, and flow cytometric IPTs were reviewed.
Main Results:
- The study included 13 HCL patients: 12 classical HCL and 1 HCL-variant (HCL-v).
- Classical HCL showed positive CD19, CD20, CD22, CD123, CD103, and CD11c.
- The HCL-v case had CD103 and CD123 positive, with negative CD25.
Conclusions:
- Accurate HCL diagnosis requires a multimodal approach combining clinical, morphological, and IPT data.
- Distinguishing HCL from other B-cell lymphoproliferative disorders (BCLPDs) is vital for effective patient management.
- Immunophenotyping plays a critical role in achieving higher diagnostic accuracy.
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.
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