Ann Clin Neurophysiol.  2023 Oct;25(2):106-109. 10.14253/acn.2023.25.2.106.

Inclusion body myositis accompanied with T-cell large granular lymphocyte leukemia

• Jeong DY
• Lee SH
• So J
• Kim JY
• Kim YC
• Kim M
• Choi EJ
• Lee EJ
• Park HJ
• Lim YM
• Kim H

• KMID: 2547403
• DOI: http://doi.org/10.14253/acn.2023.25.2.106

Abstract

Inclusion body myositis (IBM) is a late-onset myopathy that manifests as distinct muscle weakness in the quadriceps, finger flexors, and ankle dorsiflexors. T-cell large granular lymphocyte ( T-LGL) leukemia is a late-onset clonal disorder of CD8+ cy totoxic T-cells that is of ten accompanied by autoimmune diseases. To date, the association between IBM and T-LGL leukemia has been infrequently reported. Here, we report a case of a patient with T-LGL leukemia who developed IBM, along with in-depth laboratory, electrophysiological, and pathologic findings.

Figure

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  Fig. 1. Peripheral blood smear, flow cytometry, and thigh magnetic resonance imaging findings. (A) Blood smears show large granular lymphocytes (LGL) with eccentric nuclei and large cytotoxic granules (arrow, ×200). (B) Flow cytometry with large CD8+ expansions of T-LGLs with CD5- expression (arrow). (C, D) Thigh magnetic resonance image revealed T2 high signal intensity (C, T2-weighed image) and mild fatty infiltration (D, T1-weighed image) of the vastus lateralis (arrow) with relatively preserved rectus femoris (arrowhead).

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  Fig. 2. Muscle pathology findings. (A) Hematoxylin and eosin-stained muscle biopsy shows endomysial inflammation and focally invaded myofiber (arrow, ×40). (B) Immunohistochemistry for CD8 reveals CD8+ T-cell invasion of non-necrotic myofiber (arrow, ×400). (C) Immunohistochemistry for CD57 demonstrates endomysial infiltration of the T-cells (×200). (D) Immunohistochemistry for major histocompatibility complex type I demonstrates widespread upregulation in the myofibers (×200).

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