Chronic lymphocytic leukemia and autoimmunity: a systematic review

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BlankHaematologica 2011, 10.3324/haematol.2010.036152

Chronic lymphocytic leukemia and autoimmunity: a systematic review

Kate Hodgson, Gerardo Ferrer, Emili Montserrat*, Carol Moreno

Hospital Clinic, University of Barcelona, Spain

* Corresponding author; email: emontse@...

ABSTRACT

Chronic lymphocytic leukemia is frequently associated with immune disturbances.

The relationship between chronic lymphocytic leukemia and autoimmune cytopenias,

particularly autoimmune hemolytic anemia and immune thrombocytopenia, is well

established. The responsible mechanisms, particularly the role of leukemic cells

in orchestrating the production of polyclonal autoantibodies, are increasingly

well understood. Recent studies show that autoimmune cytopenia is not

necessarily associated with poor prognosis. On the contrary, patients with

anemia or thrombocytopenia due to immune mechanisms have a better outcome than

those in whom these features are due to bone marrow infiltration by the disease.

Moreover, fears about the risk of autoimmune hemolysis following single agent

fludarabine may no longer be appropriate in the age of chemo immunotherapy

regimens. However, treatment of patients with active hemolysis may pose

important problems needing an individualized and clinically sound approach. The

concept that autoimmune cytopenia may precede the leukemia should be revisited

in the light of recent data showing that autoimmune cytopenia may be observed in

monoclonal B cell lymphocytosis, a condition that can only be detected by using

sensitive flow cytometry techniques. On the other hand, there is no evidence of

an increased risk of non-hemic autoimmune disorders in chronic lymphocytic

leukemia. Likewise, there is no epidemiological proof of an increased risk of

chronic lymphocytic leukemia in patients with non-hemic autoimmunity. Finally,

since immune disorders are an important part of chronic lymphocytic leukemia,

studies aimed at unrevealing the mechanisms linking the neoplastic and the

immune components of the disease should contribute to the progress in the

understanding of this form of leukemia.