PMC5661071_01
Thoracic-abdominal-pelvic computerized tomography scan showing the hepatosplenomegaly (liver and spleen longitudinal axis of 209 cm and 158 cm, resp.), without other significant abnormalities (a). Cerebral MR images showing swelling of the left lateral rectus muscle on a T2-weighted image, with no other additional findings (b).
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The abdominal computerized tomography (CT) scan confirmed the hepatosplenomegaly (liver and spleen longitudinal axis of 209 cm and 158 cm, resp.) and did not show other abnormalities (Figure 1(a)). Five days later he also developed dysphonia, dysphagia, impaired abduction of the left eye, and left eyelid ptosis and the head MRI showed a thickening of the left lateral rectus muscle (Figure 1(b)).
Bone marrow cytomorphology findings. Composite image of circulating leukemic cells obtained from Wright-Giemsa-stained peripheral blood smears.
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Flow cytometry studies in the bone marrow aspirate, using the EuroFlow lymphoid screening tube (LST) and antibody panel for NK cell chronic lymphoproliferative diseases (NK-CLPD), consisting of 8-color combinations of monoclonal antibodies (7), and a FACSCanto II flow cytometer (Becton Dickinson). Data analysis was performed using the Infinicyt software (Cytognos, Spain). Dot-plots illustrate the phenotypic features of the neoplastic NK cells. Blue dots correspond to the neoplastic NK cells whereas pink dots and yellow dots are the normal residual T cells and B cells present in the bone marrow sample. Other cells are represented as gray dots. The abnormal NK cells were positive for surface CD45 (high), CD2, CD26, CD38, CD94 (high), and HLA-DR and cytoplasmic granzyme B and perforin; and they were negative for surface CD3, CD4, CD5, CD7, CD8, CD11c, CD16, CD19, CD20, CD25, CD56, Ig kappa, and lambda chains.
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BM aspirate cells using the EuroFlow lymphoid screening tube (LST) and antibody panel for NK cell chronic lymphoproliferative diseases (NK-CLPD), complemented with other cell surface markers, showed that the neoplastic cells were positive for CD45 (high), CD2, CD26, CD38, CD94 (high), and HLA-DR (high, heterogeneous) antigens, and negative for surface CD3, TCR, CD4, CD5, CD7, CD8, CD11b, CD11c, CD16, CD56, CD57, and CD161, as well as for cytoplasmic CD3; in addition, they express intracellular granzyme B and perforin (Figure 3). B cell (CD19, CD20, and CD79a), myeloid (CD13, CD14, CD15, CD64, CD65, and myeloperoxidase), immature (CD34, TdT), and dendritic (CD123) cell associated markers were all negative (data not shown).
Bone marrow histological findings. Bone marrow biopsy section Wright-Giemsa stained (40x) (a). The lymphoid infiltrate is positive for CD3 epsilon (b) and negative for CD56 (c). In situ hybridization showing positivity of the neoplastic cells for EBER (d).
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Bone marrow karyotype with chromosome banding analysis with complex aberrancies: 46,add(X)(q27),-Y, i(7)(q10),+8,add(17)(p13),add(19)(q13). Arrows indicate the chromosomal abnormalities.
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