TY - JOUR
T1 - Lymphocytic variant of hypereosinophilic syndrome
T2 - A report of seven cases from a single institution
AU - Hu, Zhihong
AU - Wang, Wei
AU - Thakral, Beenu
AU - Chen, Zhining
AU - Estrov, Zeev
AU - Bueso-Ramos, Carlos E.
AU - Verstovsek, Srdan
AU - Medeiros, L. Jeffrey
AU - Wang, Sa A.
N1 - Publisher Copyright:
© 2020 International Clinical Cytometry Society
PY - 2021/5
Y1 - 2021/5
N2 - Background: Lymphocytic variant of hypereosinophilic syndrome (L-HES) is a subtype of HES driven by cytokines produced by clonal T-cells. Due to the rarity of its occurrence and challenges in diagnosis, this subtype of HES is under recognized. Methods and Results: We report seven patients with L-HES, diagnosed from a group of 136 patients who were referred to our institution for the work-up of hypereosinophilia. The clinical presentation, symptoms and signs were heterogeneous and uncharacteristic; indistinguishable from idiopathic HES. Flow cytometry immunophenotypic analysis revealed aberrant T-cells in all patients, with a Th2 immunophenotype, CD2 + CD3−CD4 + CD5 + CD7dim+/−CD8− in six of seven (86%) cases. CD10 was partially expressed in one of seven (14%) cases, and clonal TCR gene rearrangement was detected by PCR in five of seven (71%) patients. All patients were treated with corticosteroids and two of seven (29%) patients received anti-IL5 antibody therapy. With a median follow-up time of 7.5 years (2.3–14.1 years), one (11%) patient developed peripheral T-cell lymphoma 6.1 years after the initial diagnosis of L-HES and responded well to chemotherapy. All patients were alive at the last follow-up. Conclusion: In conclusion, a combination of flow cytometry immunophenotyping and molecular analysis allows the identification of aberrant T-cells, facilitating a diagnosis of L-HES in patients with eosinophilia. A correct diagnosis is essential for the proper management of these patients.
AB - Background: Lymphocytic variant of hypereosinophilic syndrome (L-HES) is a subtype of HES driven by cytokines produced by clonal T-cells. Due to the rarity of its occurrence and challenges in diagnosis, this subtype of HES is under recognized. Methods and Results: We report seven patients with L-HES, diagnosed from a group of 136 patients who were referred to our institution for the work-up of hypereosinophilia. The clinical presentation, symptoms and signs were heterogeneous and uncharacteristic; indistinguishable from idiopathic HES. Flow cytometry immunophenotypic analysis revealed aberrant T-cells in all patients, with a Th2 immunophenotype, CD2 + CD3−CD4 + CD5 + CD7dim+/−CD8− in six of seven (86%) cases. CD10 was partially expressed in one of seven (14%) cases, and clonal TCR gene rearrangement was detected by PCR in five of seven (71%) patients. All patients were treated with corticosteroids and two of seven (29%) patients received anti-IL5 antibody therapy. With a median follow-up time of 7.5 years (2.3–14.1 years), one (11%) patient developed peripheral T-cell lymphoma 6.1 years after the initial diagnosis of L-HES and responded well to chemotherapy. All patients were alive at the last follow-up. Conclusion: In conclusion, a combination of flow cytometry immunophenotyping and molecular analysis allows the identification of aberrant T-cells, facilitating a diagnosis of L-HES in patients with eosinophilia. A correct diagnosis is essential for the proper management of these patients.
KW - IL5
KW - NGS
KW - T-cell lymphoma
KW - Th2 immunophenotype
KW - flow cytometry
KW - hypereosinophilia
KW - hypereosinophilic syndrome
KW - lymphocytic variant
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U2 - 10.1002/cyto.b.21874
DO - 10.1002/cyto.b.21874
M3 - Article
C2 - 32157815
AN - SCOPUS:85081341373
SN - 1552-4949
VL - 100
SP - 352
EP - 360
JO - Cytometry Part B - Clinical Cytometry
JF - Cytometry Part B - Clinical Cytometry
IS - 3
ER -