Acta Dermato-Venereologica 2018, No. 6 98-6CompleteContent | Page 17

SHORT COMMUNICATION A Case of Refractory Subcutaneous Panniculitis-like T-cell Lymphoma with Bone Marrow and Lung Involvement Treated Successfully with Oral Etoposide Akimasa SAITO, Naoko OKIYAMA*, Ryota TANAKA, Hiroshi MARUYAMA and Manabu FUJIMOTO Department of Dermatology, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan. *E-mail: [email protected] Accepted Mar 27, 2018; Epub ahead of print Mar 27, 2018 Subcutaneous panniculitis-like T-cell lymphoma (SPT- CL) is a rare type of cutaneous lymphoma accounting for less than 1% of all cutaneous lymphomas. SPTCL is characterized by the infiltration of neoplastic cytotoxic T cells into subcutaneous tissues, which makes it resemble lobular panniculitis. Haemophagocytic syndrome (HPS)/ haemophagocytic lymphohistiocytosis (HLH) is someti- mes associated with SPTCLs, occurring in approximately 17% of cases (1). On the other hand, visceral involvement is rarely observed in patients with SPTCL (1). We report here a case of a patient with SPTCL who developed bone marrow and lung involvement. CASE REPORT An 82-year-old Japanese man presented with a 6-month history of intermittent fever, non-productive cough, weight loss of 8 kg, and recurrent, multiple and painless subcutaneous nodules and plaques. Because plaques and high-grade fever were intractable despite oral prednisolone, 20–30 mg/day, the patient was referred to our hospital. Physical examination revealed plaques, which ranged from 5 to 20 mm in diameter, on the face, neck, upper chest and upper limbs (Fig. 1a, b). Laboratory investigations revealed pancytopaenia (white blood cell count 2.5×10 9 /l (normal range 4.0–9.0 × 10 9 /l); red blood cell count 2,910 × 10 9 /l (4,270–5,700 × 10 9 /l); platelet count 67 × 10 9 /l (150–350 × 10 9 /l)) with prolonged erythrocyte sedimentation rate (81 mm/h), and elevated serum levels of C- reactive protein (6.03 mg/dl), lactate dehydrogenase (441 U/l), 610 ferritin (641.9 ng/ml) and soluble interleukin-2 receptors (sIL-2R) (630 U/ml). Biopsy of the subcutaneous nodule in the left upper limb at the onset of the disease demonstrated lobular panniculitis (Fig. 1c) with infiltrating atypical lymphoid cells with pleomor­ phic nuclei (Fig. 1d). Focal rimming of adipocytes by the atypical cells, karyorrhexis and foamy histiocytes were also observed (Fig. 1d). Immunohistochemistry showed that the cells rimming the adipocytes were positive for CD3, CD8 (Fig. 1e), T-cell intracel- lular antigen-1 (Fig. 1h) and T-cell receptor (TCR)-βF1 (Fig. 1g), and negative for CD4 (Fig. 1f), CD20 (Fig. 1i), CD30, CD56 and TCRγδ (Fig. 1j). TCR-β gene clonal rearrangement was detected by PCR analysis from 2 identical samples of plaques. Bone marrow aspiration smears showed that 8.4% of total cells were atypical large lymphoid cells with enlarged and irregular nuclei. Bone marrow biopsy revealed moderate hypoplastic bone marrow tissue with infiltration of lymphoid cells expressing CD8 in the stromal layer of bone marrow tissue, but without haemophagocytosis (Fig. S1a, b 1 ). A chest X-ray revealed bilateral patch areas of conso- lidation in the middle-lower lung lobes (Fig. S1c 1 ), which were confirmed as peribronchial infiltration on computed tomography. Culture of bronchoalveolar lavage fluid, plasma cytomegalovirus pp56, plasma Epstein-Barr virus DNA and interferon-γ release assays for tuberculosis were all negative, and the serum level of β-D-glucan was normal. Transbronchial lung biopsy showed perialveolar infiltration of predominant CD8 + T cells (Fig. S1d, e 1 ). Accordingly, we diagnosed the patient as SPTCL with bone marrow and lung involvement. After the first course of CHOP chemotherapy (cyclophosphami- de 375 mg/m 2 , doxorubicin 50 mg/m 2 and vincristine 1.4 mg/m 2 at https://www.medicaljournals.se/acta/content/abstract/10.2340/00015555-2932 1 Fig. 1. Clinical and pathological findings. (a) Plaques around the eyes, and (b) on the neck, upper chest, and upper limbs. Haematoxylin and eosin-stained biopsy specimens of the subcutaneous nodule in the left upper limb showed (c) lobular panniculitis (original magnification ×2) and (d) focal rimming of adipocytes by infiltrating atypical lymphoid cells with pleomorphic nuclei and foamy histiocytes (original magnification ×400). Immunohistochemistry were performed for (e) CD8, (f) CD4, (g) TCRβF1, (h) T-cell intracellular antigen-1, (i) CD20 and (j) TCRγδ. (Diaminobenzidine and haematoxylin, original magnification×400). doi: 10.2340/00015555-2932 Acta Derm Venereol 2018; 98: 610–611 This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2018 Acta Dermato-Venereologica.