[Chronic lymphocytic leukaemia: An autoimmune disorder? Prognostic factors and the current view of pathogenesis.]

[Guest guest]

[Chronic lymphocytic leukaemia: An autoimmune disorder? Prognostic factors and

the current view of pathogenesis.]

L Pajor

Orv Hetil, May 13, 2007; 148(19): 867-78.

Pécsi Tudományegyetem, Altalános Orvostudományi Kar Patológiai Intézet Pécs

Szigeti út 12. 7624.

The heterogeneity of clinical course in chronic lymphocytic leukaemia has been

known for a long time. The easily applicable staging systems described by Rai

and Binet decades ago have represented one of the decisive obstacles against the

proper development of clinical desicion making as they correlated well with

survival data in more advanced diseases, but could not differentiate progressive

versus stabil diseases of low stages. This might have been responsible for the

development of the " watch and wait " clinical strategy potentially resulting in

the lack of therapeutical intervention in early due time. Application of recent

research data on gene expression, molecular biological, citogenetic and

phenotypic analysis of the malignancy can provide the opportunity of stratifying

the disease - although with different predictive value - at the onset or early

stages. All these represent a new challange for the diagnostics and expose

important aspects in the management of this work. The author reviews the theory

and practice of these diagnostic approaches with special emphasis on the

immunoglobulin heavy chain mutational status, expression of CD38 and ZAP-70

markers as well as the significance of karyotyping and interphase cytogenetics.

In the light of current data a putative pathogenetic scheme is outlined implying

the fundamental recognition that all individual forms of this heterogeneous

disease arise from similar and unique polyreactive - autoreactive B-cell clones

which underlies the key role of autoimmunization in the pathogenesis of the

disease.

PMID: 17478402