Document Type : Case Reports


Department of Pathology, Cancer Institute, Imam Khomeini Hospital Complex, Tehran University of Medical Sciences, Tehran, Iran


One of the rare variants of extranodal large B-cell lymphoma is intravascular large B-cell lymphoma (IVLBCL). Characteristics of IVLBCL include intraluminal selective proliferation of atypical lymphoid cells in small to medium-sized vessels. The etiologic of IVLBCL is unknown, but due to the growth pattern of this tumor, it is speculated that IVLBCL is caused by a defect in homing receptor of tumor cells. IVLBCL can involve any organ but central nervous system, lungs, and skin are the most involved sites. IVLBCL does not usually involve lymph nodes. IVLBCL mainly occurs in the middle aged to elderly population with a slight male predominance. Generally, IVLBCL is aggressive and rapidly fatal if left untreated.
We here reported two cases of IVLBCL who succumbed to the disease at initial phase of treatment to emphasize the difficulty in diagnosis of IVLBCL due to its exclusive intravascular growth pattern and fulminant clinical course.


Main Subjects

  1. Swerdlow SH, et al. WHO classification of tumors of haematopoietic and lymphoid tissues. 2016
  2. Jaffe ES, Arber DA, Campo E, Harris NL, Quintanilla-Fend L. Hematopathology e-book. Elsevier Health Sciences; 2016.
  3. Kinoshita M, Izumoto S, Hashimoto N, Kishima H, Kagawa N, Hashiba T, et al. Immunohistochemical analysis of adhesion molecules and matrix metalloproteinases in malignant CNS lymphomas: a study comparing primary CNS malignant and CNS intravascular lymphomas. Brain Tumor Pathol. 2008 Nov;25(2):73. [DOI:10.1007/s10014-008-0232-x] [PMID]
  4. Ponzoni M, Arrigoni G, Gould VE, Del Curto B, Maggioni M, Scapinello A, et al. Lack of CD 29 (β1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis. Hum Pathol. 2000;31(2):220-226. [DOI:10.1016/S0046-8177(00)80223-3]
  5. Phoon YW, Lin X, Thirumoorthy T, Tan SY, Lee HY, Lim ST, et al. Intravascular large B-cell lymphoma presenting as panniculitis clinically: a case report. Singapore Med J. 2018 Mar;59(3):163. [DOI:10.11622/smedj.2018030] [PMID] [PMCID]
  6. Shen Q, Duan X, Feng W, Nguyen N, Lapus A, Brown RE, et al. Intravascular large B-cell lymphoma: report of three cases and analysis of the mTOR pathway. Int J Clin Exp Pathol. 2011 Nov;4(8):782.
  7. Fischer M, Iglseder S, Grams A, Matosevic B, Moser P, Stockhammer G, et al. Intravascular large B-cell lymphoma mimicking central nervous system vasculitis. Hum Pathol Case Rep. 2017 Jun;8:3-8. [DOI:10.1016/j.ehpc.2016.11.002]
  8. Orwat DE, Batalis NI. Intravascular large B-cell lymphoma. Arch Pathol Lab Med. 2012 Mar;136(3):333-338. [DOI:10.5858/arpa.2010-0747-RS] [PMID]
  9. Masaki Y, Dong L, Nakajima A, Iwao H, Miki M, Kurose N, et al. Intravascular large B cell lymphoma: proposed of the strategy for early diagnosis and treatment of patients with rapid deteriorating condition. Int J Hematol. 2009 Jun;89(5):600-610. [DOI:10.1007/s12185-009-0304-7] [PMID]
  10. Murase T, Yamaguchi M, Suzuki R, Okamoto M, Sato Y, Tamaru J, et al. Intravascular large B-cell lymphoma (IVLBCL): a clinicopathologic study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood. 2007 Jan;109(2):478-485. [DOI:10.1182/blood-2006-01-021253] [PMID]
  11. Alizadeh AA, Eisen MB, Davis RE, Ma C, Lossos IS, Rosenwald A, Boldrick JC, Sabet H, Tran T, Yu X, Powell JI. Distinct types of diffuse large B-cell lymphoma identified by gene expression profiling. Nature. 2000 Feb;403(6769):503-11. [DOI:10.1038/35000501] [PMID]
  12. Berglund M, Thunberg U, Amini RM, Book M, Roos G, Erlanson M, Linderoth J, Dictor M, Jerkeman M, Cavallin-Ståhl E, Sundström C. Evaluation of immunophenotype in diffuse large B-cell lymphoma and its impact on prognosis. Modern Pathol. 2005 Aug;18(8):1113-20. [DOI:10.1038/modpathol.3800396] [PMID]
  13. De Paepe P, Achten R, Verhoef G, Wlodarska I, Stul M, Vanhentenrijk V, Praet M, De Wolf-Peeters C. Large cleaved and immunoblastic lymphoma may represent two distinct clinicopathologic entities within the group of diffuse large B-cell lymphomas. J Clin Oncol. 2005 Oct 1;23(28):7060-8. [DOI:10.1200/JCO.2005.15.503] [PMID]
  14. Kanda M, Suzumiya J, Ohshima K, Tamura K, Kikuchi M. Intravascular large cell lymphoma: clinicopathological, immuno-histochemical and molecular genetic studies. Leukemia & lymphoma. 1999 Jan 1;34(5-6):569-80. [DOI:10.3109/10428199909058485] [PMID]
  15. Yegappan S, Coupland R, Arber DA, Wang N, Miocinovic R, Tubbs RR, Hsi ED. Angiotropic lymphoma: an immunophenotypically and clinically heterogeneous lymphoma. Modern Pathology. 2001 Nov;14(11):1147-56. [DOI:10.1038/modpathol.3880450] [PMID]