Merkel cell carcinoma, chronic lymphocytic leukemia and other lymphoproliferative disorders: an old bond with possible new viral ties

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BlankMerkel cell carcinoma, chronic lymphocytic leukemia and other

lymphoproliferative disorders: an old bond with possible new viral ties

1.. T. Tadmor1,*†,

2.. A. Aviv2,† and

3.. A. Polliack3

+ Author Affiliations

1.. 1Hematology-Oncology Unit, Bnai-Zion Medical Center, Haifa

2.. 2Hematology-Oncology Unit, Emek Medical Center, Afula

3.. 3Department of Hematology, Hadassah University Hospital and Hebrew

University Medical School, Jerusalem, Israel

1.. *Correspondence to: Dr T. Tadmor, Hematology-Oncology Unit, Bnai-Zion

Medical Center, 47 Golomb Street, Haifa 31048, Israel. Tel: +972-4859407; Fax:

+972-48359962; E-mail: tamar.tadmor@...

a.. Received January 29, 2010.

b.. Revision received April 1, 2010.

c.. Accepted April 2, 2010.

Abstract

Merkel cell carcinoma (MCC) is a rare and aggressive skin tumor. The link

between tumorigenesis and immunosuppression is well known and the increased

prevalence of MCC in human immunodeficiency virus carriers and organ transplant

recipients and in patients with hemato-oncological neoplasias is now well

recognized over the past decade. In this respect, chronic lymphocytic leukemia

(CLL) seems to be the most frequent neoplasia associated with the development of

MCC. Very recently, a newly described virus, the Merkel cell polyomavirus, was

found in ~80% of MCC tumor samples and is in fact the first member of the

polyomavirus family to be associated with human tumors. The virus appears to

play a role in the pathogenesis of MCC and may constitute the missing link

between immunosuppression and the development of MCC. This review summarizes the

current knowledge relating to MCC and its pathogenesis, stressing the link with

hematologic neoplasias in general and to CLL in particular. We describe the

permissive immunologic environment, which enables the virus-containing tumor

cells to survive and proliferate in disorders like CLL. More studies are still

needed to confirm this appealing theory in a more convincing manner.