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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 954-956

A rare coexistence - Chronic lymphocytic leukemia and Kaposi sarcoma: Case report and review of the literature

Department of Medical Oncology, Diskapi Yildirim Beyazit Education and Research Hospital, Ankara, Turkey

Date of Web Publication15-Feb-2016

Correspondence Address:
Muhammet Bekir Hacioglu
Irfan Bastug Street, Ulus/Diskapi, Ankara
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.165863

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 > Abstract 

Chronic lymphocytic leukemia (CLL) is the most common leukemia worldwide. Skin lesions associated with CLL mostly develop on the bases of infectious or a hemorrhagic origin with an estimated incidence of 25% of all the cases. Kaposi sarcoma (KS)-associated with human herpes virus-8 infection is a spindle-cell, malignant, low-grade tumor originating from vascular and lymphatic endothelium. KS mostly presents with skin lesions as the initial presentation. The relation between these two pathologies has not yet been clarified up to date. Herein, we report a case of KS along with CLL to illustrate the possible relation between these two pathologies.

Keywords: Chronic lymphocytic leukemia, coexistence, human herpes virus-8, immune deficiency, Kaposi sarcoma

How to cite this article:
Hacioglu MB, Sahin S, Karatas F, Aytekin A. A rare coexistence - Chronic lymphocytic leukemia and Kaposi sarcoma: Case report and review of the literature. J Can Res Ther 2015;11:954-6

How to cite this URL:
Hacioglu MB, Sahin S, Karatas F, Aytekin A. A rare coexistence - Chronic lymphocytic leukemia and Kaposi sarcoma: Case report and review of the literature. J Can Res Ther [serial online] 2015 [cited 2021 Jan 19];11:954-6. Available from: https://www.cancerjournal.net/text.asp?2015/11/4/954/165863

 > Introduction Top

Kaposi sarcoma (KS), which is caused by human herpes virus-8 (HHV-8) infection, is a multifocal cutaneous neoplasm arising from the vascular endothelium or pericytes. [1],[2] Serological evidences have shown that HHV-8 infection is required for the development of KS. [3],[4] Classical form of KS has been commonly reported to be as a secondary malignancy rather than primary disease. In view of these findings, lymphoproliferative disorders and the presence of immunosuppression suggest the possible relation between these two pathologies in the best way. [5],[6]

Chronic lymphocytic leukemia (CLL) is characterized by the increased number of mature dysfunctional lymphocytes in peripheral blood, bone marrow, and reticuloendothelial system. Cutaneous lesions in CLL patients are present in 25% of all the cases. The risk of skin cancer in patients with CLL has been reported to be increased 7-8-fold when compared to the general population. Therefore, skin lesions in patients with CLL may be the first clinical signs of secondary malignancies. [7]

 > Case report Top

A 70-year-old male patient was admitted to the hospital complaining with fatigue, weakness, night sweats, and pelvic pain. Physical examination revealed a splenomegaly and multiple lymph nodes in a size of 2 cm in the bilateral cervical and submandibular region. Laboratory findings at the arrival were as follows: Hemoglobin 10,5 g/dL, platelet: 96000/mL, leukocyte: 70,000/mL, and lymphocyte: 60,100/μL. Abdominal ultrasonography showed a large spleen and paraaortic multiple lymph nodes in the size of 45 mm × 31 mm. Chest X-ray, gastroscopy, and colonoscopy findings have no significant findings. Serological markers for hepatitis B, C, and anti-HIV were negative. Immunoglobulin G, A, and M were below the normal ranges. Bone marrow biopsy revealed small lymphoid cells infiltration with nodular pattern. Neoplastic cells in the bone marrow were found strong positive for CD5, CD20, CD23, bcl-2 antibodies, and negative for CD10 and cyclin-D1 antibody in the immunohistochemical study. Based upon these findings, patient was diagnosed as CLL showing small lymphoid infiltration with a diffuse pattern in the bone marrow biopsy [Figure 1]a and b]. Flow cytometric analysis in peripheral blood sample identified a composition of 95% lymphocytes, 4% granulocytes, and 1% monocytes. Lymphocytes analysis showed kappa-positive CLL staining positive with CD5, CD19, CD23, CD20, CD22, CD25, and CD43. Fluorescent in situ hybridization method was negative for 17p13.1 deletion in the peripheral blood cytogenetic examination. The patient was given chemotherapy scheme of rituximab + fludarabine + cyclophosphamide combination for 6 cycles. Complete response was achieved at the end of the treatment. A short time after the chemotherapy completion, patient was represented with a new complaint of a painless, purple-black lesion on the left foot [Figure 2]. Punch biopsy of the skin lesion revealed KS with positive staining for CD34 in vessel endotel and nuclear staining for HHV-8 in spindle cell by immunochemical study [Figure 3]a and b]. Patient received radiotherapy with a total dose of 30 Gy for skin lesion. Three months later on follow-up, skin lesion was completely regressed with leaving hyperpigmented patch.
Figure 1: (a) Infiltration of atypical lymphocytes in the nodular pattern in the bone marrow. (H and E, 40). (b) CD23 positivity in neoplastic cells in bone marrow (anti-CD23, 100)

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Figure 2: Purple-black lesion on the left foot compatible with Kaposi sarcoma

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Figure 3: (a) Hemosiderin deposits in the dermis (×10). (b) Vascular structures with irregular lumen surrounded by spindle-looking or adult endothelial, hemosiderin deposits in the interim (×40)

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 > Discussion and conclusion Top

HHV-8 is the main cause of KS in 90% of the cases. Serological evidences have shown that development of KS requires a latent HHV-8 infection which promotes the oncogenic transformation in spindle cells. Infected endothelial cells by latent HHV-8 virus infection subsequently lead a spindle cells transformation in endothelial cells. The proliferative phase or tumor growth is promoted by the endothelial growth factor which was secreted from infected endothelial cells. Control of HHV-8 infection is mediated by an antiviral T-cell response. However, it has been shown that there is an instability in the circulating T-lymphocytes in patient with CLL. Based upon these findings, it can be hypothesized that latent HHV-8 infection may trigger the development of KS in patients with CLL. Thus, lymphoproliferative disorders along with the presence of immunosuppression may suggest a significant relation between CLL and KS as a possible result of immune disorderliness.

There is no much information in the literature regarding the relation between CLL and KS. Previous studies in patients with CLL have reported increased risks for the various forms of skin cancers, including malignant melanoma, as a result of immune disorderliness. [8] In a register-based study which indicated 3-fold increased risk for sarcoma in CLL patients, KS-specific risks were not estimated. [9] In another analysis, 2-7-fold increased risk for KS has been reported in the patients with lymphoid malignancies (Hodgkin's disease, non-Hodgkin's lymphoma, and leukemia), but risks for CLL were not calculated specifically. [6] According to a study designed by Hisada et al., significantly increased risk was observed for KS, malignant melanoma, larynx, and lung cancer in CLL patients at 5-, 3-, 1.7-, and 1.6-fold, respectively, with 95% confidence interval: 1.15-1.26. [10] Risk of secondary cancer in treated CLL patients were found similar with previously treated CLL patients. [10] Although the role of etiological factors remains unclear, increased incidence of secondary malignancies in CLL patients suggest an effect of immunodeficiency related to CLL.

As a result, there is a relation between CLL and KS. Herein, we agree with that KS developed secondary to immune system disorder related to CLL. We presented this case to illustrate the possible relation between CLL and KS and also to emphasize that secondary malignancies may arise on the basis of CLL. However, studies including large number of patients should be designed to evaluate and illustrate this hypothesis.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Boshoff C, Schulz TF, Kennedy MM, Graham AK, Fisher C, Thomas A, et al. Kaposi's sarcoma-associated herpesvirus infects endothelial and spindle cells. Nat Med 1995;1:1274-8.  Back to cited text no. 1
Schalling M, Ekman M, Kaaya EE, Linde A, Biberfeld P. A role for a new herpes virus (KSHV) in different forms of Kaposi's sarcoma. Nat Med 1995;1:707-8.  Back to cited text no. 2
Staskus KA, Zhong W, Gebhard K, Herndier B, Wang H, Renne R, et al. Kaposi's sarcoma-associated herpesvirus gene expression in endothelial (spindle) tumor cells. J Virol 1997;71:715-9.  Back to cited text no. 3
Stürzl M, Blasig C, Schreier A, Neipel F, Hohenadl C, Cornali E, et al. Expression of HHV-8 latency-associated T0.7 RNA in spindle cells and endothelial cells of AIDS-associated, classical and African Kaposi's sarcoma. Int J Cancer 1997;72:68-71.  Back to cited text no. 4
Fossati S, Boneschi V, Ferrucci S, Brambilla L. Human immunodeficiency virus negative Kaposi sarcoma and lymphoproliferative disorders. Cancer 1999;85:1611-5.  Back to cited text no. 5
Iscovich J, Boffetta P, Winkelmann R, Brennan P. Classic Kaposi's sarcoma as a second primary neoplasm. Int J Cancer 1999;80:178-82.  Back to cited text no. 6
Kose F, Kocer NE, Sumbul AT, Sezer A, Yilkan O. Kaposi's sarcoma following chronic lymphocytic leukemia: A rare entity. Case Rep Oncol 2012;5:271-4.  Back to cited text no. 7
Bartik MM, Welker D, Kay NE. Impairments in immune cell function in B cell chronic lymphocytic leukemia. Semin Oncol 1998;25:27-33.  Back to cited text no. 8
Mellemgaard A, Geisler CH, Storm HH. Risk of kidney cancer and other second solid malignancies in patients with chronic lymphocytic leukemia. Eur J Haematol 1994;53:218-22.  Back to cited text no. 9
Hisada M, Biggar RJ, Greene MH, Fraumeni JF Jr, Travis LB. Solid tumors after chronic lymphocytic leukemia. Blood 2001;98:1979-81.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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