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A case of adult T ‐cell leukemia presenting primary S jögren's syndrome‐like symptoms
Author(s) -
Nakamura Hideki,
Iwamoto Naoki,
Horai Yoshiro,
Takagi Yukinori,
Ichinose Kunihiro,
Kawashiri Shinya,
Taguchi Jun,
Hayashi Tomayoshi,
Nakamura Takashi,
Kawakami Atsushi
Publication year - 2013
Publication title -
international journal of rheumatic diseases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.795
H-Index - 41
eISSN - 1756-185X
pISSN - 1756-1841
DOI - 10.1111/1756-185x.12127
Subject(s) - medicine , xerophthalmia , sicca syndrome , gastroenterology , t cell leukemia , leukemia , immunology , pathology , disease , vitamin , vitamin a deficiency , retinol
Dear Editor, Sj€ ogren’s syndrome (SS) is an autoimmune disorder characterized by xerostomia, xerophthalmia, the presence of autoantibodies and extraglandular manifestations. Exclusion criteria for SS involve various conditions, including hepatitis C infection or graft versus host disease (GVHD), because patients with these conditions show sicca symptoms resembling those of SS. Although human T-cell leukemia virus type I (HTLV-I) is not included in the exclusion criteria, HTLV-I is thought of as a causative agent for SS. Although patients with HTLV-I-associated myelopathy (HAM) often have SS, there is no report of adult T-cell leukemia (ATL) cells infiltrating salivary glands, causing patients to show sicca symptoms. Here, we report a case of ATL presenting with sicca symptoms. A 53-year-old man who had xerostomia and xerophthalmia visited our hospital with complaints of swelling of bilateral parotid glands on November 7, 2008. Physical examination showed mild submandibular lymphadenopathy without anemia. Blood tests showed a total leukocyte count of 6300/mm, with a normal hemoglobin level of 14.4 g/dL and a platelet count of 14.7 9 104/mm; however, 24% of leukocytes were abnormal lymphocytes. A peripheral blood smear revealed numerous lymphocytes, some of which showed irregular, lobulated nuclei, presenting a flower shape. Other laboratory data showed slightly elevated lactate dehydrogenase (507 IU/L; normal: 119–229), normal C-reactive protein (0.02 mg/dL; normal: < 0.17), normal immunoglobulin G (IgG: 1220 mg/dL; normal: 870–1700), normal calcium (9.3 mg/dL; normal: 9.0–10.6), elevated soluble interleukin 2 receptor (sIL-2R; 6080 U/mL, normal: 124–466) and elevated parathyroid hormone-related protein (4.9 pmol/L, normal: < 1.1). Anti-hepatitis C virus antibody, rheumatoid factor, anti-nuclear antibody (ANA), anti-SS-A antibody and anti-SS-B antibody were negative. Antibody for HTLV-I was positive, and monoclonal integration of HTLV-I proviral DNA in peripheral blood mononuclear cells was verified by Southern blot analysis. Schirmer’s test was 6 mm/5 min (< 5 mm = positive), and Saxon’s test was positive (0.72 g/2 min; < 2 g = positive). Chest X-rays and abdominal computed tomography (CT) scans were all normal. The patient was diagnosed with ATL and classified as having the chronic type of ATL. To confirm SS as an auxiliary diagnosis, magnetic resonance imaging (MRI) of the parotid gland and a labial salivary gland (LSG) biopsy were carried out. The MRI showed diffuse enlargement of the parotid gland, and ultrasound images (Fig. 1) showed aggregation of hypoechoic nodules in enlarged parotid glands with irregular septal structure. The LSG biopsy showed massive infiltrating mononuclear cells (MNCs) with cellular atypia. Immunohistochemistry showed CD4-positive T-lymphocyte-dominant infiltration (Fig. 2a–d). Furthermore, HTLV-I-related proteins were shown in LSG of this patient, although not all of these proteins were observed in LSG from a patient with HTLV-I-seronegative SS