Severe deficiency of natural killer activity in the peripheral blood of patients with hairy cell leukemia.

Hairy cell leukaemia (HCL) is a rare neoplasm of peripheral B cells which represents a paradox in oncology. Despite its largely unknown origin and behaviour, HCL is one of the few example of dramatic success in the treatment of a malignancy. The recent steps forward to understanding the biology of HCL from immunogenetic and genomic studies have recently provided new insight into diagnosis and prognosis. Several data from immunoglobulin gene (IG) analysis have provided hints regarding the cell of origin and the ongoing selective interactions of the tumour BCR with environmental stimuli. It has also recently emerged that an unmutated status of the HCL IG can be associated with failure to respond to cladribine, genetic abnormalities indicative of poor outcome and aggressive disease. These observations suggest a central role of the tumour B-cell receptor in defining the outcome of HCL and that that IG gene analysis may have biological and prognostic relevance. Hopefully, IG analysis will help tailor treatment strategies for the most aggressive cases.

Hairy cell leukaemia: biological and clinical overview from immunogenetic insights

Although there is increasing evidence that the majority of cases of hairy cell leukemia represent B-lymphoproliferative disorders, the exact subset of B-cells from which hairy cells are derived, is still unclear. On the basis of results obtained in previous studies, and data collected from the literature, it is suggested that B-lymphocytes normally residing in the marginal zone of the splenic white pulp (so called 'marginal zone lymphocytes') represent the cell type from which B-lineage derived hairy cells originate.

Hairy Cell Leukemia: A B-Lymphocytic Disorder Derived from Splenic Marginal Zone Lymphocytes?

Hairy cell leukemia: Clinical effects of the methanol extraction residue (MER) of BCG, lithium carbonate and mononuclear cell-enriched leukocyte transfusions

Hairy cell leukemia: Absence of natural killer activity and of interleukin 1 release in OKM-1+ spleen hairy cells☆

The majority of hairy cells from three patients with leukaemic reticuloendotheliosis were adherent cells bearing surface immunoglobulin, complement receptors, and Fc receptors. Highly purified populations of malignant hairy cells, which readily bound soluble antigen -antibody complexes in suspension and were able to adhere to immobilized antigen-antibody complexes, were examined for their ability to mediate ADCC.* Two patients with greater than 90% FcR positive cells failed to mediate ADCC. When initially examined, a third paitent, with fewer malignant cells, demonstrated a less marked impairment of cytolysis. When reexamined at a later date, this patient had an increased number of hairy cells and concomitantly demonstrated more impaired ADCC effector cell activity. Absorption of surface immunoglobulin bearing cells onto plastic surfaces coated with goat anti-human immunoglobulin resulted in a complete depletion of FcR, sIg positive, hairy cells. The remaining nonadherem fraction, containing 5.5% FcR positive, sIg negative cells, was able to mediate ADCC as effectively as the normal controls. These results indicate that although FcR bearing hairy cells readily bind soluble antigen-antibody complexes and adhere to immobilized complexes, they were unable to mediate ADCC.

Fc Receptor Bearing ‘Hairy Cells’ of Leukaemic Reticuloendotheliosis Bind Soluble Antigen‐Antibody Complexes and Adhere to Immobilized Complexes, but Fail to Mediate Antibody Dependent Cellular Cytotoxicity

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Fc Receptor Bearing ‘Hairy Cells’ of Leukaemic Reticuloendotheliosis Bind Soluble Antigen‐Antibody Complexes and Adhere to Immobilized Complexes, but Fail to Mediate Antibody Dependent Cellular Cytotoxicity