Robert Fox, MD

Disclosures

June 17, 2005

Question

A patient presents with dry eyes, waxing and waning lymphocytosis, and no splenomegaly. All other lab work is normal, except for decreased activity of natural killer cells. Can there be an associated lymphocytosis with Sjögren's syndrome?

Diane Batshaw Eisman, MD

Response from Robert Fox, MD

The key question is whether the patient has "relative" or "absolute" lymphocytosis. This is determined by multiplying the total number of white blood cells by the percentage of lymphocytes. Relative lymphocytosis (ie, higher proportion of lymphocytes but a normal absolute number of lymphocytes) is common in Sjögren's syndrome and is usually not clinically significant.[1] This relative lymphocytosis occurs because many patients with this syndrome have mild neutropenia (due to lower absolute neutrophil count) but maintain a normal absolute lymphocyte count. This manifests as relative lymphocytosis. In patients with absolute lymphocytosis, diffuse infiltrative lymphocytic syndrome (DILS) must be considered.[2] This disorder is associated with retroviral infection, an infectious mononucleosis-like syndrome, or development of a lymphoproliferative disorder with chronic lymphocytic leukemic features.[3]

Comment

The mechanism of neutropenia in rheumatoid arthritis, Sjögren's syndrome, or systemic lupus erythematosus may be multifactorial.[4] In most patients, the neutropenia does not reflect decreased production of neutrophils in the bone marrow but is caused by increased "pooling" of the neutrophils in the peripheral splenic and mesenteric vasculature; as a result, neutrophils are not found in the blood sample obtained at venipuncture.[5,6] However, neutrophils may undergo destruction by cell-mediated and humoral mechanisms in such conditions as Felty's syndrome.[5,6]

Absolute lymphocytosis may be due to infection by retrovirus (especially HIV or human T-cell leukemia virus type 1) that clinically mimics Sjögren's syndrome but is characterized by excess CD8+ cells (ie, inverted CD4/CD8 ratio), as evidenced by flow cytometry and positive results on serologic testing for the retrovirus. This manifestation of HIV is frequently characterized by parotid gland swelling and is termed DILS.[7,8] Other infections associated with CD8+ lymphocytosis are mononucleosis (Epstein-Barr virus) or cytomegalovirus. In both of these conditions, the T cells are polyclonal based on the distribution of T-cell receptors as determined at the DNA level.[9] There is also a condition known as T-gamma cell (or natural killer cell) lymphoma that manifests as CD8+/natural killer cell phenotype lymphoma on flow cytometry and monoclonal T-cell receptors on serologic testing.[9] Finally, patients with Sjögren's syndrome may have a lymphoproliferative disorder that has chronic lymphocytic leukemia-like properties (CD5+ CD20+ cells) or B-cell lymphoma with circulating B-cell (usually CD20+ CD5-) monoclonal cells.[3]

Comments

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