Intravascular Large B-Cell Lymphoma: A Continuing Clinical Enigma
Keywords:
lymphoma, large B cell, intravascular, fever of unknown originAbstract
Intravascular large B-cell lymphoma (IVLBCL) is a rare form of extranodal diffuse large B-cell lymphoma characterized by the proliferation of lymphoma cells in the lumen of small blood vessels. Clinical presentation varies among reported cases and diagnosis can be challenging for both clinicians and pathologists. We report a case of a 64-year-old female with a history of prolonged fever. Diagnosis was suspected clinically due to thrombocytopenia and elevated serum lactate dehydrogenase after exhausting work-up for an infectious etiology. IVLBCL was established on post-mortem examination upon finding infiltration of CD20-positive neoplastic cells in several organs in the absence of lymph node involvement.
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References
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4. Ponzoni M, Arrigoni G, Gould VE, et al. Lack of CD29 (beta1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis. Hum Pathol. 2000;31(2):220-6. PMID: 10685637.
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6. Zuckerman D, Seliem R, Hochberg E. Intravascular lymphoma: the oncologist's "great imitator". Oncologist. 2006;11(5):496-502. PMID: 16720850. https://doi.org/10.1634/theoncologist.11-5-496.
7. Ponzoni M, Ferreri AJ, Campo E, et al. Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol. 2007;25(21):3168-73. PMID: 17577023. https://doi.org/10.1200/JCO.2006.08.2313.
8. Wang J, Ding W, Gao L, et al. High frequency of bone marrow involvement in intravascular large B-cell lymphoma: a clinicopathological study of 13 cases in China. Int J Surg Pathol. 2016;25(2):118-26. PMID: 27553679. https://doi.org/10.1177/1066896916665203.
9. Fiegl M, Greil R, Pechlaner C, Krugmann J, Dirnhofer S. Intravascular large B cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem. Ann Oncol. 2002;13(9):1503-6. PMID: 12196378.
10. Khanna R, Sarin S. Non-cirrhotic portal hypertension--diagnosis and management. J Hepatol. 2014;60(2):421-41. PMID: 23978714. https://doi.org/10.1016/j.jhep.2013.08.013.
11. Yasuda, H, Ando J, Matsumoto T, et al. Intravascular large B cell lymphoma with hepatic portal vein, splenic vein and mesenteric vein tumour embolism. Histopathology. 2010;57(4):648-50. PMID: 20955393. https://doi.org/10.1111/j.1365-2559.2010.03660.x.
12. Matsue K, Asada N, Odawara J, et al. Random skin biopsy and bone marrow biopsy for diagnosis of intravascular large B cell lymphoma. Ann Hematol. 2011;90(4):417-21. PMID: 20957365. https://doi.org/10.1007/s00277-010-1101-3.
13. Orwat DE, Batalis NI. Intravascular large B-cell lymphoma. Arch Pathol Lab Med. 2012;136(3):333-8. PMID: 22372911. https://doi.org/10.5858/arpa.2010-0747-RS.
14. Sur M, Ross C, Moens F. Intravascular large B-cell lymphoma of the uterus: a diagnostic challenge. Int J Gynecol Pathol. 2005;24(2):201-3. PMID: 15782078.
15. Shigematsu Y, Matsuura M, Tsuyama N, et al. Intravascular large B-cell lymphoma of the bilateral ovaries and uterus in an asymptomatic patient with a t(11;22)(q23;q11) constitutional translocation. Intern Med. 2016;55(21):3169-74. PMID: 27803414. PMCID: PMC5140869. https://doi.org/ 10.2169/internalmedicine.55.6578.
16. Fournier M, Hourseau M, Chis C, Luton D, Koskas M. Uterine presentation of an intravascular large B-cell lymphoma diagnosed by PET/CT and histology. Case Rep Clin Pathol. 2014;1(2):105-8. https://doi.org/10.5430/crcp.v1n2p105.
17. Wahie S, Dayala S, Husain A, Summerfield G, Hervey V, Langtry JA. Cutaneous features of intravascular lymphoma. Clin Exp Dermatol. 2011;36(3):288–91. PMID: 21418271. https://doi.org/10.1111/j.1365-2230.2010.03934.x.
2. Murase T, Yamaguchi M, Suzuki R, et al. Intravascular large B-cell lymphoma (IVLBCL): a clinicopathological study of 96 cases with special reference to the immunophenotypic heterogeneity of CD5. Blood. 2007;109(2):478-85. PMID: 16985183. https://doi.org/10.1182/blood-2006-01-021253.
3. Nakamura S, Ponzoni M, Campo E. Intravascular large B-cell lymphoma. In S. Swerdlow, E. Campo, Harris NL, editors. WHO classification of tumours of haematopoeitic and lymphoid tissues, 4th ed. Lyon: IARC. 2008, pp.252-3.
4. Ponzoni M, Arrigoni G, Gould VE, et al. Lack of CD29 (beta1 integrin) and CD 54 (ICAM-1) adhesion molecules in intravascular lymphomatosis. Hum Pathol. 2000;31(2):220-6. PMID: 10685637.
5. Holmes NE, Gordon CL, Lightfoot N, et al. Intravascular large B cell lymphoma: an elusive cause of pyrexia of unknown origin diagnosed postmortem. Clin Infect Dis. 2010; 51(9): e61-4. PMID: 20868278. https://doi.org/10.1086/656684.
6. Zuckerman D, Seliem R, Hochberg E. Intravascular lymphoma: the oncologist's "great imitator". Oncologist. 2006;11(5):496-502. PMID: 16720850. https://doi.org/10.1634/theoncologist.11-5-496.
7. Ponzoni M, Ferreri AJ, Campo E, et al. Definition, diagnosis, and management of intravascular large B-cell lymphoma: proposals and perspectives from an international consensus meeting. J Clin Oncol. 2007;25(21):3168-73. PMID: 17577023. https://doi.org/10.1200/JCO.2006.08.2313.
8. Wang J, Ding W, Gao L, et al. High frequency of bone marrow involvement in intravascular large B-cell lymphoma: a clinicopathological study of 13 cases in China. Int J Surg Pathol. 2016;25(2):118-26. PMID: 27553679. https://doi.org/10.1177/1066896916665203.
9. Fiegl M, Greil R, Pechlaner C, Krugmann J, Dirnhofer S. Intravascular large B cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem. Ann Oncol. 2002;13(9):1503-6. PMID: 12196378.
10. Khanna R, Sarin S. Non-cirrhotic portal hypertension--diagnosis and management. J Hepatol. 2014;60(2):421-41. PMID: 23978714. https://doi.org/10.1016/j.jhep.2013.08.013.
11. Yasuda, H, Ando J, Matsumoto T, et al. Intravascular large B cell lymphoma with hepatic portal vein, splenic vein and mesenteric vein tumour embolism. Histopathology. 2010;57(4):648-50. PMID: 20955393. https://doi.org/10.1111/j.1365-2559.2010.03660.x.
12. Matsue K, Asada N, Odawara J, et al. Random skin biopsy and bone marrow biopsy for diagnosis of intravascular large B cell lymphoma. Ann Hematol. 2011;90(4):417-21. PMID: 20957365. https://doi.org/10.1007/s00277-010-1101-3.
13. Orwat DE, Batalis NI. Intravascular large B-cell lymphoma. Arch Pathol Lab Med. 2012;136(3):333-8. PMID: 22372911. https://doi.org/10.5858/arpa.2010-0747-RS.
14. Sur M, Ross C, Moens F. Intravascular large B-cell lymphoma of the uterus: a diagnostic challenge. Int J Gynecol Pathol. 2005;24(2):201-3. PMID: 15782078.
15. Shigematsu Y, Matsuura M, Tsuyama N, et al. Intravascular large B-cell lymphoma of the bilateral ovaries and uterus in an asymptomatic patient with a t(11;22)(q23;q11) constitutional translocation. Intern Med. 2016;55(21):3169-74. PMID: 27803414. PMCID: PMC5140869. https://doi.org/ 10.2169/internalmedicine.55.6578.
16. Fournier M, Hourseau M, Chis C, Luton D, Koskas M. Uterine presentation of an intravascular large B-cell lymphoma diagnosed by PET/CT and histology. Case Rep Clin Pathol. 2014;1(2):105-8. https://doi.org/10.5430/crcp.v1n2p105.
17. Wahie S, Dayala S, Husain A, Summerfield G, Hervey V, Langtry JA. Cutaneous features of intravascular lymphoma. Clin Exp Dermatol. 2011;36(3):288–91. PMID: 21418271. https://doi.org/10.1111/j.1365-2230.2010.03934.x.
Published
08/23/2017
How to Cite
Lo, R., & Lique, I. C. (2017). Intravascular Large B-Cell Lymphoma: A Continuing Clinical Enigma. PJP, 2(2), 31. Retrieved from https://philippinejournalofpathology.org/index.php/PJP/article/view/74
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Autopsy Vault
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The Philippine Journal of Pathology is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License. Based on works made open access at http://philippinejournalofpathology.org
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