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Skin Cancer in Patients with Chronic Lymphocytic Leukemia and Non-Hodgkin Lymphoma

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Skin Cancer in Patients with Chronic Lymphocytic Leukemia and Non-Hodgkin Lymphoma Skin Cancer in Patients With Chronic Lymphocytic Leukemia and Non-Hodgkin Lymphoma Oluwakemi Onajin and Jerry D. Brewer, MD Ms. Onajin is a student at the Mayo Abstract: The association between non-Hodgkin lymphoma, includ- Medical School College of Medicine at ing chronic lymphocytic leukemia, and aggressive skin cancers is Mayo Clinic, in Rochester, Minnesota. well established. This review highlights existing data that address Dr. Brewer is an Assistant Professor in the increased incidence and clinical characteristics of skin cancers Division of Dermatologic Surgery at Mayo Clinic, in Rochester, Minnesota. in patients with non-Hodgkin lymphoma—specifically, chronic lymphocytic leukemia. Patients with non-Hodgkin lymphoma have Address correspondence to: worse outcomes when melanoma and nonmelanoma skin cancers Jerry D. Brewer, MD develop. The poorer outcomes in these patients are evidenced by Division of Dermatologic Surgery increased rates of local recurrence, regional metastasis, and death. Mayo Clinic Lymphoproliferative neoplasms and certain skin cancers may share 200 First St. SW Rochester, MN 55905 similar pathogenic factors, which could provide insights regarding E-mail: [email protected] their close relationship and the behavior of lymphoma-related skin cancers. As a consequence of the poorer prognosis in patients with lymphoma-related skin cancer, more aggressive therapeutic measures could reduce the risk of skin cancer recurrence, metas- tasis, and death. Strategies such as sun protection, education, and frequent dermatologic examinations may help prevent and success- fully treat skin cancers in patients with lymphoma. Introduction Non-Hodgkin lymphoma (NHL) encompasses a diverse group of lymphoproliferative cancers. It is more common in developed regions, such as North America; Australia; New Zealand; and Northern, Western, and Southern Europe.1 NHL is the seventh most common cancer in the United States.2 Chronic lymphocytic leukemia (CLL) is a member of the NHL family. It is a low-grade lymphoprolifera- tive malignancy characterized by clonal proliferation of B cells, and it represents 25% of all leukemias in developed countries.3 A number of reports describe the increased risk of many forms of cancers in patients with NHL. Such cancers include brain can- cer, malignant melanoma (MM), lung carcinoma, nonmelanoma skin cancers (NMSCs), Hodgkin disease,4-7 Kaposi sarcoma, Merkel Keywords Chronic lymphocytic leukemia, melanoma, non- cell carcinoma (MCC), eruptive multiple piloleiomyomas, mycosis Hodgkin lymphoma, skin neoplasms fungoides, and metastatic adnexal tumors, such as atypical fibrox- Clinical Advances in Hematology & Oncology Volume 10, Issue 9 September 2012 571 ON A J I N A N D B R E W E R anthoma and microcystic adnexal carcinoma.8-13 Many A 5-year recurrence rate of 19%—a rate 7 times higher studies describe the increased incidence and aggressiveness than in control subjects—was reported in these patients of skin cancer in patients with lymphoma. Interestingly, with SCC who had a prior diagnosis of CLL.19 a number of reports also describe an inverse association Patients with CLL who have skin cancer also have whereby patients with MM or NMSC are at increased risk higher rates of metastasis and death. Investigators in Aus- for NHL.14 The diagnosis of skin cancer is common, and tralia demonstrated that patients with CLL had increased therefore it is imperative to obtain high-quality research risk of death due to MM and NMSC (standardized to validate the association between skin cancer and NHL. mortality ratios [SMRs], 4.79 and 17.0, respectively).21 This article will review the data on the epidemiology, clini- Another study of patients with CLL who developed SCC cal characteristics, pathogenesis, prevention, and treatment showed a 5-year metastatic rate of 18% and an eventual of skin cancer in the clinical setting of NHL. mortality rate of 11%; the metastatic rates were the same for patients with CLL treated with chemotherapy and Epidemiologic Factors patients with CLL treated with observation alone.20 In this study, all metastases initially involved the regional A few studies have demonstrated increased risk of NMSC lymph nodes. Hence, patients who have CLL and SCC and MM in patients with NHL. The highest-quality data with aggressive clinical or histopathologic features should come from large, population-based studies conducted in be considered for sentinel lymph node biopsy. Denmark, Sweden, and Switzerland.3,4,6,14,15 One such Some population-based studies have shown that study, from Switzerland, showed that patients with CLL MM occurs 2.3–3.1 times more often in patients with had increased risk of squamous cell carcinoma (SCC) CLL.5,23 Although data on the outcomes of MM are and basal cell carcinoma (BCC; standardized incidence limited, patients with CLL in whom MM develops have ratio, 5.0 and 2.7, respectively).14 A study conducted in been reported to have poorer outcomes. A recent Surveil- the United States reported that patients with CLL were lance Epidemiology and End Results population-based 8 times more likely to have skin cancer than patients study demonstrated decreased overall survival in patients without CLL.15 In addition to SCC, BCC, and MM, with MM and a history of CLL (SMR, 2.6). MM cause- other rare skin cancers—such as Kaposi sarcoma, eruptive specific survival was also worse in these patients, with a multiple piloleiomyomas, mycosis fungoides, metastatic reported SMR of 2.8.22 This same study also reported atypical fibroxanthoma, metastatic microcystic adnexal poor outcomes for patients with MCC and a prior diag- carcinoma, and MCC—have been reported in patients nosis of CLL; overall survival and MCC cause-specific who have NHL.5,8-13 survival were worse in the clinical setting of CLL, with In the studies describing an inverse association reported SMRs of 3.1 and 3.8, respectively.22 As a result whereby patients with MM or NMSC are at increased of the poorer outcomes in patients who have MM, MCC, risk for NHL, this risk varied from 1.1–2.6 times higher and NMSC in the clinical setting of NHL, a more aggres- than average.14 Moreover, NHL patients who had a prior sive approach should be considered in the treatment of diagnosis of skin cancer have a higher mortality rate from these skin cancers in patients with NHL. NHL.16,17 This increased mortality rate is not thought to be due to death from skin cancer metastasis. These find- Mechanism and Pathogenesis of the Increased ings suggest that patients who present with NHL and a Incidence of Skin Cancers in NHL prior diagnosis of skin cancer should be considered for Several pathogenic factors are thought to be involved thorough surveillance and, potentially, for more aggressive in the increased association of skin cancer and NHL. therapy. In addition to an increased incidence of skin can- Some investigators have proposed that the immunosup- cer, patients with NHL are also at increased risk for more pressive effects of chemotherapeutic agents have a role aggressive forms of skin cancer, as evidenced by higher in the association.24 In addition, ultraviolet (UV) radia- rates of recurrence, regional metastasis, and death.18-22 tion may also be involved in the development of NHL.25 A study of patients with BCC treated with Mohs Well-established data support the association between micrographic surgery found a 5-year recurrence rate of UV radiation and skin cancer26; however, the evidence 22% in patients with a prior diagnosis of CLL.18 This regarding the role of UV radiation in NHL is inconclu- reported recurrence rate of BCC in the clinical setting of sive.25 Impaired functioning of the immune system is also CLL was 14 times higher than for control subjects with- thought to play a considerable role in the development out CLL, even when the patients had similar preoperative and clinical characteristics of MM, NMSC, and NHL.27 tumor sizes and histologic subtypes. As with BCC, SCC Sunlight can cause immunosuppression,28 and one study treated with Mohs micrographic surgery was also reported found a correlation between sunlight and the incidence of to have an increased recurrence rate in patients with CLL. NHL and MM (but not CLL).25 572 Clinical Advances in Hematology & Oncology Volume 10, Issue 9 September 2012 SK I N C A N C E R I N No N-H O DGK I N LY M P ho M A Immunosuppression has served as the basis for Other postulates for the association of skin cancer and many hypotheses on the association between NHL and NHL are inconclusive. These postulates include genetic skin cancer. In NHL, immunosuppression occurs amid abnormalities, including aberrations due to viral pathogens, impaired immune surveillance and the cytotoxic function HLA-associated variation, and environmental insults.34 of lymphocytes. In a healthy person, the immune system prevents the development of such immune-responsive Genetics tumors as MM and NMSC by suppressing and eliminat- A shared genetic abnormality could be the cause of the ing mutagenic events that would otherwise progress to association between NHL and skin cancer. Some com- full cancers. This concept of immunosuppression-related mon genetic aberrations are seen in patients with lym- skin cancer is similar to the increased incidence of skin phoma and those with skin cancer. The tumor suppressor cancer in recipients of an organ transplant.29 In NHL, gene p53 is located on the short arm of chromosome 17 the dysfunctional B cells may also have an important part and is commonly mutated in cancers affecting humans. It in immunosuppression.30 Proliferation of dysfunctional, has been linked to lymphomas and skin cancers. In fact, malignant cells leads to a decreased amount of normal 50% of sporadic BCCs have a p53 mutation.35,36 This cells. Investigators have demonstrated that leukemic B cells mutation is also found in 41–69% of SCCs26,35 and has secrete immunosuppressive factors and also downregulate been associated with MM as well.
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