Sjogren’s syndrome and Lymphoma

A 55-year-old woman with a medical history significant for Sjögren’s syndrome (diagnosed about 21 years ago) and Raynaud’s phenomenon presented with palpable lymphadenopathy involving the right middle and lower neck and weight loss. A review of systems was negative for cough, hemoptysis, abdominal symptoms, fever, chills, night sweats, joint pain, and malaise. Her personal history revealed no smoking or alcohol abuse. Physical examination revealed significant xerostomia, discoloration of the teeth, and several small, rubbery lymph nodes in the right cervical region, but no other regional lymph node involvement, visceromegaly, or palpable masses on abdominal examination. What’s your diagnosis?

◻ Tuberculosis	◻ Squamous cell carcinoma
◻ Lymphoma	◻ Brucellosis

Answer

Lymphoma—This patient had stage I B-cell non-Hodgkin’s lymphoma secondary to Sjögren’s syndrome (SS). SS is a chronic autoimmune disease associated with the production of autoantibodies and characterized by a progressive lymphocytic and plasma cell infiltration of the salivary and lacrimal glands leading to xerostomia and xerophthalmia. SS may be primary or secondary (associated with another autoimmune disease, e.g., rheumatoid arthritis). It is more common in women than men (9:1), and the average age of presentation is 50 years of age Autoantibodies to Ro/SS-A antigen and La/SS-B antigen may be seen with this syndrome. Ro/SS-A autoantigen consists of 3 polypeptide chains (52, 54, and 60 kDa) in conjunction with RNAs while La/SS-B (48 kDa) is bound to RNA III polymerase transcripts.

Patients with SS are known to have an increased risk of developing B-cell non-Hodgkin’s lymphoma. However, such a complication occurs in less than 10% of patients and is mainly reported in those with primary SS. A risk of greater than 40 times compared with normal has been estimated. Extranodal low-grade B-cell non-Hodgkin’s lymphoma is observed most frequently. Persistent enlargement of parotid glands, adenopathy, monoclonal gammopathy, and cross-reactive idiotypes are all harbingers of a possible lymphoma evolution. Factors involved in the lymphogenesis in SS leading to lymphoma include dysregulation in the mechanisms responsible for apoptosis, hyperstimulation of B-1 cells, and an infectious agent.

Tuberculosis must be considered in all cervical lymphadenopathy. Clinical features include fever, weight loss, night sweats, hemoptysis, and pleural pain. Diagnosis rests in part on clinical suspicion, particularly in high-risk groups, and in the characteristic caseating granuloma.

Eight percent of carcinomas involving lymph nodes are metastatic squamous cell carcinomas, and 90% of these have their primaries in the head and neck. Also, a history of smoking and alcohol is present in such patients.

Brucellosis is a zoonotic disease caused by a gram-negative coccobacillus, Brucella melitensis. The clinical features include pyrexia of unknown origin, hepatosplenomegaly, pancytopenia, weight loss, lymphadenopathy, malaise, and arthritis.