Individuals with primary Sjögren's syndrome (pSS) have over 40-fold increased risk of the development B-cell lymphoma.1 The relationship of SS with T-cell lymphoma is, nevertheless, enigmatic. We herein describe a case of a patient with features compatible with SS who evolved to a hepatosplenic gammadelta T-cell lymphoma (GDTL).

The patient, a 25-year-old white female, had complained of fatigue, “dry eyes” (confirmed by an Ophthalmologist), parotid enlargement and xerostomia for the last four years. Physical examination in December 2008 revealed increased parotid glands and hepatosplenomegaly, but no peripheral lymphadenopathy. Pancytopenia was present (hemoglobin 7.9g/dL, white blood cell 1000cells/mm3, platelet count 107,000cells/mm3). The erythrocyte sedimentation rate was of 37mm in the first hour. Polyclonal hypergammaglobulinemia was present. The antinuclear antibody test was strongly positive (1/5120, speckled pattern), and anti-SSA antibodies were detected in high levels (124 units in an ELISA). The rheumatoid factor test was weakly positive (45 units). Abdominal ecography confirmed hepatosplenomegaly. A bone marrow biopsy (BMB) showed hypercellularity, without evidence of malignancy. Considering the clinical and laboratory findings suggestive of pSS, the patient was treated with prednisone and azathioprine.

After eight months, a notable improvement of clinical and hematological features was seen. Hepatosplenomegaly remained, however, and a new BMB plus splenectomy were carried out. At that time, hemoglobin was 10g/dL, the leukocyte count was 21,900cells/cm3 (with 65 erythroblasts per 100 leukocytes), and the platelet count was 106,000/cm3. The spleen histology was inconclusive, and the BMB showed interstitial infiltration by atypical lymphoid cells. Immunohistochemistry of spleen and bone marrow revealed the following lymphocyte profile: CD3+, CD4−, CD5−, CD8−, Ki 67+ with a rate of 50% CD56+ focal. The karyotype showed, of importance, eight trisomy and absence of chromosome seven. Altogether, morphologic, phenotypic and genetic findings were compatible with a hepatosplenic GDTL. After eighteen months of standard chemotherapy, the patient died in September 2011.

Hepatosplenic GDTL is an aggressive and uncommon malignancy (<1% of lymphoid neoplasms). Intense gammadelta T-cell proliferation is characteristically seen in liver, spleen and bone marrow sinusoids.2 The disorder usually affects young adults, and the outcome is poor.3 Hepatosplenomegaly and severe cytopenias, both seen in our patient, are usual aspects; lymphadenopathy is rare.4 Of interest, hepatosplenic GDTL can mimic the hemophagocytic syndrome.5 In 2009, a cutaneous GDTL was diagnosed in a patient with rheumatoid arthritis using etanercept.6

Current ACR classification criteria for SS include autoantibodies, ocular staining and salivary gland histology, suggesting that case definition requires two of the three.7 pSS was initially a suitable diagnosis for our patient due to the presence of ophthalmic sicca, parotiditis, typical autoantibodies, polyclonal hypergammaglobulinemia, and peripheral pancytopenia. The first BMB showed no malignancy, and clinical features responded well to traditional immunosuppression.

The unexpected persistence of hepatosplenomegaly led to an immunohistochemistry diagnosis of hepatosplenic GDTL eight months ahead. Thus, it is conceivable that the patient firstly had pSS and later developed a GDTL. Although one cannot rule out the possibility that she presented GDTL with SS features since the beginning, the reported median survival time of six months for GDTL8 turns it less plausible. Also, an atypical form of SS “secondary” to GDTL could be brought about. Necessary to say, the occurrence of SS and GDTL could have been only coincidental in our patient. For any of these circumstances, no similar clinical scenario combining SS features and GDTL has been described so far.

In summary, we describe a case of a young patient with features of SS who evolved, unusually, to a hepatosplenic GDTL. The interplay of SS with non-B lymphomas has yet to be clarified.