Eruptive Telangiectasia as a Presenting Manifestation of Intravascular NK-cell Lymphoma: a Rare Case Report and Review of the Literature


  • Apasee Sooksamran Institute of Dermatology–Ministry of Public Health, Bangkok
  • Poonawis Sudtikoonaseth Institute of Dermatology–Ministry of Public Health, Bangkok


Epstein-Bar virus, NK-cell lymphoma, Intravascular lymphoma, Telangiectasia


Intravascular NK-cell lymphoma (IVNKL) is a very rare neoplasm. Although intravascular B-cell lymphoma is recognized as a common variant of mature B-cell neoplasm, IVNKL has not even been categorized by the World Health Organization (WHO) classification. To date, a few cases of IVNKL have been reported. The most common presentation is erythematous patches and plaques on trunk and extremities. Only a single case presented with blanching erythematous patches that resemble telangiectasia. Here, we report a case of IVNKL presented with eruptive telangiectasia, papules and plaques on the upper trunk and extremities accompanied with progressive weight loss. Skin histopathology showed atypical mononuclear cell infiltrations within lumina of blood vessels. Immunohistochemical study revealed NK cell phenotype with PCR and DNA sequencing for EBV were positive.. Thorough investigations were done and showed no internal organ involvement. Treatment was started with prednisolone. Unfortunately, the patient passed away 2 months after the diagnosis because of tumor lysis syndrome.


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1. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood 2016; 127: 2391-405.

2. Nguyen GH, Yassin AH, Magro CM. Unusual variants of intravascular malignant hematopoietic neoplasms: a report of 4 cases and review of the literature. The Am J Dermatopathol 2015; 37: 360-7.

3. Nakamichi N, Fukuhara S, Aozasa K, Morii E. NKcell intravascular lymphomatosis--a mini-review. Eur J Haematol 2008; 81: 1-7.

4. Röglin J, Böer A. Skin manifestations of intravascular lymphoma mimic inflammatory diseases of the skin. The Br J Dermatol 2007; 157: 16-25.

5. Bi Y, Huo Z, Liang Z, et al. Intravascular NK-cell lymphoma: a case report and review of the literature. Diagn Pathol 2015; 10: 84.

6. Wu H, Said JW, Ames ED, et al. First reported cases of intravascular large cell lymphoma of the NK cell type: clinical, histologic, immunophenotypic, and molecular features. Am J Clin Pathol 2005; 123: 603-11.

7. Santucci M, Pimpinelli N, Massi D, et al. Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop. Cancer 2003; 97: 610-27.

8. Jiang L, Xie JL, Zhou XG. [Intravascular NK-cell lymphoma: a clinicopathologic study and literature review]. Zhonghua Bing Li Xue Za Zhi 2011; 40: 689-93.

9. Liao JB, Hsieh PP, Hwang YC, Lin SL, Wu CS. Cutaneous intravascular natural killer-cell lymphoma: a rare case and review of the literature. Acta Derm Venereol 2011; 91: 472-3.

10. Wang L, Chen S, Ma H, et al. Intravascular NK/Tcell lymphoma: a report of five cases with cutaneous manifestation from China. J Cutan Pathol 2015; 42: 610-7.

11. Alhumidi A. Cutaneous Intravascular NK/T-cell lymphoma mimic panniculitis clinically, case report and literature brief review. Diagn Pathol 2015;10:107.

12. Kuo TT, Chen MJ, Kuo MC. Cutaneous intravascular NK-cell lymphoma: report of a rare variant associated with Epstein-Barr virus. Am J Surg Pathol 2006; 30: 1197-201.

13. Song DE, Lee MW, Ryu MH, Kang DW, Kim SJ, Huh J. Intravascular large cell lymphoma of the natural killer cell type. J Clin Oncol 2007; 25: 1279-82.

14. Chen H, Zhong LS, Wei ZP, Jin X, Quan S, Liu YQ. Case of intravascular natural killer cell lymphoma. J Dermatol 2013; 40: 573-4.

15. Marin GR, Majek E. [Acute kidney injury secondary to steroid-induced tumor lysis in an adolescent with acute lymphoblastic leukemia: role of urinary alkalinisation and peritoneal dialysis]. Archi Argent Pediatr 2012; 110: e118-22.

16. Kim JO, Jun DW, Tae HJ, et al. Low-dose steroidinduced tumor lysis syndrome in a hepatocellular carcinoma patient. Clin Mol Hepatol 2015; 21: 85-8.

17. Gupta R, Gautam RK, Bhardwaj M, Chauhan A. A clinical approach to diagnose patients with localized telangiectasia. Int J Dermatol 2015; 54: e294-301.

18. Pongpairoj K, Rerknimitr P, Wititsuwannakul J, Asawanonda P. Eruptive telangiectasia in a patient with fever and haemophagocytic syndrome. Clin Exp Dermatol 2016; 41: 696-8.

19. Cerroni L, Massone C, Kutzner H, Mentzel T, Umbert P, Kerl H. Intravascular large T-cell or NKcell lymphoma: a rare variant of intravascular large cell lymphoma with frequent cytotoxic phenotype and association with Epstein-Barr virus infection. Am J Surg Pathol 2008; 32: 891-8.

20. López-Gil F, Roura M, Umbert I, Umbert P. Malignant proliferative angioendotheliomatosis or angiotropic lymphoma associated with a softtissue lymphoma. J Am Acad Dermatol 1992; 26: 101-4.

21. Gebauer N, Nissen EJ, Driesch P, Feller AC, Merz H. Intravascular natural killer cell lymphoma mimicking mycosis fungoides: a case report and review of the literature. Am J Dermatopathol 2014; 36: e100-4.

22. Liu Y, Zhang W, An J, Li H, Liu S. Cutaneous intravascular natural killer-cell lymphoma: a case report and review of the literature. Am J Clin Pathol 2014; 142: 243-7.




How to Cite

Sooksamran, A., & Sudtikoonaseth, P. (2017). Eruptive Telangiectasia as a Presenting Manifestation of Intravascular NK-cell Lymphoma: a Rare Case Report and Review of the Literature. Thai Journal of Dermatology, 33(4), 275–285. Retrieved from