Abstract
Lymphoproliferative lesions with plasmablastic/plasmacytoid appearance associated with KSHV/HHV8 in HIV patients most frequently found include multicentric Castleman disease (MCD), KSHV/ HHV8-associated diffuse large B-cell lymphoma (KSHV/HHV8+ DLBCL), primary effusion lymphoma, extracavitary primary effusion lymphoma, and more recently described, the KSHV inflammatory cytokine syndrome (KICS). These, more than separate entities, are considered a spectrum of clinical manifestations associated with KSHV/HHV8 infection. Patients with Kaposi's sarcoma are at higher risk of developing MCD and, in turn, those with
plasmablastic aggregates are more likely to progress to frank lymphoma associated with KSHV/HHV8. We present the clinical case of a 42-year-old male patient with HIV/AIDS and Kaposi's sarcoma who was later found to have histopathologically proven areas of MCD with KSHV/HHV8+ DLBCL. Additionally, a literature review of the differential diagnosis of this spectrum of entities is provided.
References
Chadburn A, Said J, Gratzinger D, Chan JKC, de Jong D, Jaffe ES et al. HHV8/KSHV-Positive Lymphoproliferative Disorders and the Spectrum of Plasmablastic and Plasma Cell Neoplasms: 2015 SH/EAHP Workshop Report—Part 3. Am J Clin Pathol. 2017;147(2):171-87.
Ramla S, Guy J. An unusual posttransplant EBV-associated lymphoproliferative disorder exhibiting plasmacytic features in leukemic phase. Blood. 2015;125(10):1672.
Carbone A, Cesarman E, Spina M, Gloghini A, Schulz TF. HIV-associated lymphomas and gamma-herpesviruses. Blood. 2009;113(6):1213-24. 4. Natkunam Y, Gratzinger D, Chadburn A, Goodlad JR, Chan JKC, Said J et al. Immunodeficiency-associated lymphoproliferative disorders: time for reappraisal? Blood. 2018;132(18):1871-8.
Polizzotto MN, Uldrick TS, Wyvill KM, Aleman K, Marshall V, Wang V et al. Clinical Features and Outcomes of Patients With Symptomatic Kaposi Sarcoma Herpesvirus (KSHV)-associated Inflammation: Prospective Characterization of KSHV Inflammatory Cytokine Syndrome (KICS). Clin Infect Dis Off Publ Infect Dis Soc Am. 2016;62(6):730-8.
Piñeiro F, Climent F, Imaz A, Gudiol C, Niubó J, Palomar-Muñoz A et al. Clinical and pathological features of Kaposi sarcoma herpesvirus-associated inflammatory cytokine syndrome. AIDS Lond Engl. 2020;34(14):2097-101.
Alaggio R, Amador C, Anagnostopoulos I, Attygalle AD, Araujo IB de O, Berti E et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Lymphoid Neoplasms. Leukemia. 2022;36(7):1720-48.
Dupin N, Diss TL, Kellam P, Tulliez M, Du MQ, Sicard D et al. HHV-8 is associated with a plasmablastic variant of Castleman disease that is linked to HHV-8-positive plasmablastic lymphoma. Blood. 2000;95(4):1406-12.
Du MQ, Diss TC, Liu H, Ye H, Hamoudi RA, Cabeçadas J et al. KSHV- and EBV-associated germinotropic lymphoproliferative disorder. Blood. 2002;100(9):3415-8.
Oksenhendler E, Boulanger E, Galicier L, Du MQ, Dupin N, Diss TC et al. High incidence of Kaposi sarcoma-associated herpesvirus-related non-Hodgkin lymphoma in patients with HIV infection and multicentric Castleman disease. Blood. 2002;99(7):2331-6.
Pan ZG, Zhang QY, Lu ZB (Jim), Quinto T, Rozenvald IB, Liu LT et al. Extracavitary KSHV-associated Large B-Cell Lymphoma: A Distinct Entity or a Subtype of Primary Effusion Lymphoma? Study of 9 Cases and Review of an Additional 43 Cases. Am J Surg Pathol. 2012;36(8):1129.
Brimo F, Michel RP, Khetani K, Auger M. Primary effusion lymphoma. Cancer Cytopathol. 2007;111(4):224-33.
Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/ idiopathic multicentric Castleman disease. Blood. 2017;129(12):1646-57.
Ramaswami R, Lurain K, Polizzotto MN, Ekwede I, Waldon K, Steinberg SM et al. Characteristics and outcomes of KSHV-associated multicentric Castleman disease with or without other KSHV diseases. Blood Adv. 2021;5(6):1660-70
Gonzalez-Farre B, Martinez D, Lopez-Guerra M, Xipell M, Monclus E, Rovira J et al. HHV8-related lymphoid proliferations: a broad spectrum of lesions from reactive lymphoid hyperplasia to overt lymphoma. Mod Pathol. 2017;30(5):745-60.
Nador R, Cesarman E, Chadburn A, Dawson D, Ansari M, Sald J et al. Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi’s sarcoma-associated herpes virus. Blood. 1996;88(2):645- 56
Chadburn A, Hyjek E, Mathew S, Cesarman E, Said J, Knowles DM. KSHV-Positive Solid Lymphomas Represent an Extra-Cavitary Variant of Primary Effusion Lymphoma. Am J Surg Pathol. 2004;28(11):1401.
Bruce-Brand C, Rigby J. Kaposi sarcoma with intravascular primary effusion lymphoma in the skin: A potential pitfall in HHV8 immunohistochemistry interpretation. Int J Surg Pathol [Internet]. 2020;28(8):868-71.
Knowles D, Inghirami G, Ubriaco A, Dalla-Favera R. Molecular genetic analysis of three AIDS-associated neoplasms of uncertain lineage demonstrates their B-cell derivation and the possible pathogenetic role of the Epstein-Barr virus. Blood. 1989;73(3):792-9
Walts AE, Shintaku P, Said JW. Diagnosis of Malignant Lymphoma in Effusions from Patients with AIDS by Gene Rearrangement. Am J Clin Pathol. 1990;94(2):170-5.
Said JW, Shintaku IP, Asou H, deVos S, Baker J, Hanson G et al. Herpesvirus 8 Inclusions in Primary Effusion Lymphoma: Report of a Unique Case With T-Cell Phenotype. Arch Pathol Lab Med. 1999;123(3):257-60.
All material published in the journal HEMATOLOGÍA (electronic and print version) is transferred to the Argentinean Society of Hematology. In accordance with the copyright Act (Act 11 723), a copyright transfer form will be sent to the authors of approved works, which has to be signed by all the authors before its publication. Authors should keep a copy of the original since the journal is not responsible for damages or losses of the material that was submitted. Authors should send an electronic version to the email: revista@sah.org.ar
