Persistent polyclonal B-cell lymphocytosis

Signs and symptoms

Ten percent of patients present with splenomegaly and lymphadenopathy. Some patients report a varying degree of fatigue, consistent with a chronic fatigue syndrome, or postviral fatigue as seen in EBV infections while others remain asymptomatic.

Genetics

Genetically, PPBL has been associated with a few unusual genetic characteristics. Among them, it is associated with a particular genetic variant of the human leukocyte antigen called HLA-DR7. This variant is normally present in 26% in the Caucasian population. Chromosome analysis has detected an isochromosome +i(3q), with or without premature chromosome condensation. Also, a t(14;18)(q22;21) bcl-2/IgH rearrangement has been described, as usually seen in follicular lymphoma. Immunologically, peripheral B-cells show more functional IgD+ positive CD27 cells than usual.

Prognosis

In the followup of 111 patients, most remained stable and event free. However, two patients developed IgM gammopathy 2 lung cancer; one developed cervical cancer and three developed non-Hodgkin's lymphoma. The possibility of developing a clonal proliferation, malignant lymphoma or secondary solid cancer led the authors to conclude not to classify PPBL as a benign pathology, as has been previously postulated but rather to recommend a careful and continued clinical and biological longterm follow-up.

References

References

1. Mitterer, M. (July 1995). "Chronic active Epstein-Barr virus disease in a case of persistent polyclonal B-cell lymphocytosis". Br J Haematol.
2. (October 2000). "Persistent polyclonal B lymphocytosis: morphological, immunological, cytogenetic and molecular analysis of an Italian case". Leuk. Res..
3. Cornet, E. (February 2009). "Long-term follow-up of 111 patients with persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes". Leukemia.