“Persistent polyclonal B-cell lymphocytosis”的意思、由来-开放百科全书

Persistent polyclonal B-cell lymphocytosis (PPBL) is an anomaly of the human immune system characterized by mildly elevated levels of white blood cells (called leukocytosis), chronic, stable absolute polyclonal B-cell lymphocytosis, elevated polyclonal IgM and binucleated cells.

Although cases of non-smoking women or men have been reported, patients are predominantly young smoking women.

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,^[1] or postviral fatigue as seen in EBV infections while others remain asymptomic.^[2]

Molecular and immunological analysis

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-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.^[3]

References

1. ^{{Cite journal|doi=10.1111/j.1365-2141.1995.tb05579.x|last=Mitterer|first=M|author2=Pescosta|title=Chronic active Epstein-Barr virus disease in a case of persistent polyclonal B-cell lymphocytosis|journal=Br J Haematol|date=July 1995|volume=90|issue=3|pages=526–31|pmid=7646989|display-authors=etal}}
2. ^{{Cite journal|doi=10.1016/S0145-2126(00)00069-2|last=Tonelli|first1=S|author2=Petronilla|title=Persistent polyclonal B lymphocytosis: morphological, immunological, cytogenetic and molecular analysis of an Italian case|journal=Leuk. Res.|date=October 2000|volume=24|issue=10|pages=877–9|pmid=10996207|display-authors=etal}}
3. ^{{Cite journal|doi=10.1038/leu.2008.208|last=Cornet|first=E|author2=Lesesve JF|title=Long-term follow-up of 111 patients with persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes|journal=Leukemia|date=February 2009|volume=23|issue=2|pages=419–22|pmid=18668130|pmc=2685812|display-authors=etal|url=http://www.hal.inserm.fr/inserm-00338136/document}}