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The diagnostic value of CD123 in B-cell disorders with hairy or villous lymphocytes
Ilaria Del Giudice1, Estella Matutes, Ricardo Morilla
1Academic Department of Hematology and Cytogenetics, The Royal Marsden Hospital and Institute of Cancer Research, London, UK.
Insights
CD123 is a valuable marker for diagnosing hairy cell leukemia (HCL). Most typical HCL cases express CD123, while HCL-variant and splenic lymphoma with villous lymphocytes (SLVL) do not.
Area of Science:
- Hematology
- Immunophenotyping
- Oncology
Background:
- CD123, the alpha chain of the human interleukin-3 receptor, is expressed on various hematopoietic cells, including those in acute leukemia and hairy cell leukemia (HCL).
- Distinguishing between different B-cell disorders with circulating villous lymphocytes can be challenging.
- Accurate diagnostic markers are crucial for effective patient management.
Purpose of the Study:
- To evaluate the diagnostic utility of CD123 expression in differentiating B-cell disorders characterized by circulating hairy and villous lymphocytes.
- To assess the sensitivity and specificity of CD123 as a marker for typical HCL, HCL-variant, and splenic lymphoma with villous lymphocytes (SLVL).
Main Methods:
- Immunophenotypic analysis using flow cytometry was performed on neoplastic cells from 59 patients diagnosed with HCL, HCL-variant, or SLVL.
- Peripheral blood, bone marrow, and spleen cell suspensions were analyzed.
- Cells from 12 patients with chronic lymphocytic leukemia served as controls.
Main Results:
- CD123 expression was detected in 95% of typical HCL cases with strong to moderate intensity.
- CD123 was notably absent in the circulating cells of most HCL-variant (91%) and SLVL (97%) cases.
- The marker demonstrated high sensitivity and specificity for typical HCL.
Conclusions:
- CD123 is a highly effective diagnostic marker for distinguishing typical HCL from other B-cell disorders with circulating villous lymphocytes.
- CD123 expression is characteristic of typical HCL.
- CD123 cannot differentiate between HCL-variant and SLVL, as both are CD123-negative.
Background And Objectives:
CD123 is an antibody that identifies the a chain of the human interleukin-3 receptor and is expressed in a variety of normal hematopoietic cells, acute leukemia and hairy cell leukemia (HCL). The aim of the study was to investigate the diagnostic value of CD123 expression in B-cell disorders with circulating hairy and villous lymphocytes.
Design And Methods:
We investigated the diagnostic value of CD123 expression in neoplastic cells from 59 patients with B-cell disorders with circulating hairy or villous lymphocytes: HCL (n=24), the variant form of HCL (n=11) and splenic lymphoma with villous lymphocytes (SLVL) (n=24). Cells from 12 patients with chronic lymphocytic leukemia were used as controls. Immunophenotypic analysis was performed by flow cytometry on 77 samples from peripheral blood (n=48), bone marrow (n=25) and spleen cell suspensions (n=4).
Results:
Our findings show that cells from 95% of typical HCL express CD123 with strong to moderate intensity while this molecule is absent in circulating cells from most cases of HCL-variant (91%) and SLVL (97%).
Interpretation And Conclusions:
We conclude that CD123 is a useful new marker for distinguishing B-cell disorders with circulating villous lymphocytes as its expression is characteristic of typical HCL with high sensitivity and specificity. However CD123 does not allow the distinction between HCL-variant and SLVL, as both are CD123 negative.
