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. 2021 Jun 21;13(12):3102.
doi: 10.3390/cancers13123102.

T-Cell Lymphoblastic Lymphoma Arising in the Setting of Myeloid/Lymphoid Neoplasms with Eosinophilia: LMO2 Immunohistochemistry as a Potentially Useful Diagnostic Marker

Magda Zanelli  1 , Giuseppe G Loscocco  2   3 , Elena Sabattini  4 , Maurizio Zizzo  5   6 , Francesca Sanguedolce  7 , Luigi Panico  8 , Daniela Fanni  9 , Raffaella Santi  10 , Cecilia Caprera  11 , Cristiana Rossi  12 , Alessandra Soriano  13   14 , Alberto Cavazza  1 , Alessandro Giunta  5 , Cristina Mecucci  15 , Alessandro M Vannucchi  2   3 , Stefano A Pileri  16 , Stefano Ascani  11   15
Affiliations

T-Cell Lymphoblastic Lymphoma Arising in the Setting of Myeloid/Lymphoid Neoplasms with Eosinophilia: LMO2 Immunohistochemistry as a Potentially Useful Diagnostic Marker

Magda Zanelli et al. Cancers (Basel). .

Abstract

Background: Rarely, T-lymphoblastic lymphoma (T-LBL) may develop in the setting of myeloid/lymphoid neoplasms with eosinophilia (M/LNs-Eo), a group of diseases with gene fusion resulting in overexpression of an aberrant tyrosine kinase or cytokine receptor. The correct identification of this category has relevant therapeutic implications. LIM domain only 2 (LMO2) is overexpressed in most T-LBL, but not in immature TdT-positive T-cells in the thymus and in indolent T-lymphoblastic proliferations (iT-LBP).

Methods and results: We retrospectively evaluated 11 cases of T-LBL occurring in the context of M/LNs-Eo. Clinical, histological, immunohistochemical and molecular features were collected and LMO2 immunohistochemical staining was performed. The critical re-evaluation of these cases confirmed the diagnosis of T-LBL with morphological, immunohistochemical and molecular features consistent with T-LBL occurring in M/LNs-Eo. Interestingly, LMO2 immunohistochemical analysis was negative in 9/11 cases, whereas only 2 cases revealed a partial LMO2 expression with a moderate and low degree of intensity, respectively.

Conclusions: LMO2 may represent a potentially useful marker to identify T-LBL developing in the context of M/LNs-Eo. In this setting, T-LBL shows LMO2 immunohistochemical profile overlapping with cortical thymocytes and iT-LBP, possibly reflecting different molecular patterns involved in the pathogenesis of T-LBL arising in the setting of M/LNs-Eo.

Keywords: FGFR1; PCM1-JAK2; PDGFRA; PDGFRB; T-cell; eosinophilia; lymphoblastic; lymphoma.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
T-LBL medium-sized cells with high nuclear-cytoplasmic ratio and dispersed chromatin (case 5, Giemsa staining, magnification 400×).
Figure 2
Figure 2
Lymphoblastic proliferation diffusely expressing TdT (case 5, magnification 400×).
Figure 3
Figure 3
CD3 positivity of T-LBL (case 5, magnification 400×).
Figure 4
Figure 4
Immature myeloid cells, admixed to T-LBL, highlighted by myeloperoxidase (MPO) immunostaining (case 7, magnification 200×).
Figure 5
Figure 5
T-LBL containing a discrete component of eosinophils (within red circles) (case 7, Hematoxylin-Eosin staining, magnification 400×).
Figure 6
Figure 6
Aggregates of proerythroblasts in the context of T-LBL, in the PCM1-JAK2 rearranged case (case 5, Giemsa staining, magnification 400×).
Figure 7
Figure 7
Glycophorin C highlighting proerythroblasts within T-LBL, in the PCM1-JAK2 rearranged case (case 5, immunostaining, magnification 400×).
Figure 8
Figure 8
LMO2 negativity of T-LBL; endothelial cells as positive LMO2 controls (case 1, immunostaining, magnification 400×).
Figure 9
Figure 9
Partial and weak LMO2 expression of T-LBL (case 2, immunostaining, magnification 400×).
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