Solans-Laque et al evaluated patients with primary Sjogren's syndrome for predictors associated with malignant lymphoma. These can help to identify patients who may benefit from closer monitoring over time. The authors are from Vall d'Hebron University and Autonomous University in Barcelona.

Patient selection: primary Sjogren's syndrome


Outcome: malignant non-Hodgkin's lymphoma, primarily MALT lymphoma and diffuse large B-cell lymphoma


A patient with primary Sjogren's syndrome has a 16-fold increased risk for developing lymphoma compared to the general population.


Predictors of malignant lymphoma at presentation:

(1) hypocomplementemia (low C3 and/or low C4)

(2) lymphocytopenia (absolute lymphocyte count <= 1,000 per µL)


Hypocomplementemia was associated with early development while the onset with isolated lymphocytopenia tended to be later.


Other factors at presentation identified on the univariate analysis:

(1) palpable purpura

(2) parotidomegaly

(3) leukopenia (white blood cell count <= 4,000 per µL)

(4) hypergammaglobulinemia (gamma globulin fraction > 22% of total protein)

(5) anemia


Features of malignant lymphoma:

(1) The longer the duration since the diagnosis of Sjogren' syndrome the greater the frequency of malignant lymphoma.

(2) An indolent lymphoma may transform to a higher grade over time.

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