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Cervical Lymphadenopathy in an Elderly Man

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A White man in his 70s with a medical history notable only for mitral valve repair was incidentally found to have cervical lymphadenopathy on magnetic resonance imaging while undergoing workup for shoulder pain. A fine-needle biopsy of an enlarged left-sided cervical lymph node yielded benign results. One month later, he presented to a local emergency department with diffuse abdominal pain. Symptoms began 6 weeks earlier and had been intermittent in nature. He had also experienced fatigue, night sweats, decreased appetite, and weight loss for 6 weeks. He otherwise denied having fevers, myalgias, nausea, vomiting, diarrhea, or easy bruising. Physical examination revealed bilateral cervical, axillary, and inguinal lymphadenopathy in addition to mild diffuse abdominal pain. Laboratory results revealed a hemoglobin level of 13.1 g/dL (to convert to g/L, multiply by 10), a white blood cell count of 6.4 × 103/μL, and a platelet count of 404 × 103/μL (to convert to × 109/L, multiply by 1) in addition to negative results for HIV, hepatitis B virus, and hepatitis C assays. Additional workup results were notable for elevated levels of interleukin (IL) 10 (1091 pg/mL), IL-6 (3.1 pg/mL), C-reactive protein (4.9 mg/dL [to convert to mg/L, multiply by 10]), and erythrocyte sedimentation rate (79 mm/h). A computed tomography scan of the neck, chest, abdomen, and pelvis further revealed extensive cervical, supraclavicular, retroperitoneal, pelvic, and inguinal lymphadenopathy, with extensive inflammation and fat stranding adjacent to these enlarged nodes. Positron emission tomography–computed tomography further demonstrated fluorodeoxyglucose-avid lymph nodes above and below the diaphragm. The patient then underwent excisional biopsy of an enlarged right cervical lymph node (Figure).

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B. Multicentric Castleman disease

A microscopic analysis of the biopsy revealed focal concentric mantle cell zone architecture (or onion-skin morphology; Figure). Further, the capsule was noted to be focally thickened, with the presence of vascular proliferation in addition to substantial intermedullary plasmacytosis. Immunohistochemical staining demonstrated human herpes virus–8 (HHV8) positivity in the spindle cells in areas of vascular proliferation (diagnostic of Kaposi sarcoma; Figure) and the round large lymphoid cells in the follicles (diagnostic of coexisting multicentric Castleman disease [MCD]).1 A peripheral blood sample also demonstrated HHV8 positivity, and a diagnosis of MCD was made. There were no granulomas or necrosis seen, making granulomatous diseases, including tuberculosis and sarcoidosis, unlikely. Moreover, the preserved nodal architecture was not consistent with lymphoma.

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Article Information

Corresponding Author: Ashutosh Kacker, MD, Department of Otolaryngology–Head & Neck Surgery, New York-Presbyterian Hospital/Weill Cornell Medicine, 1305 York Ave, 5th Floor, New York, NY 10024 (ask9001@med.cornell.edu).

Published Online: May 12, 2022. doi:10.1001/jamaoto.2022.0829

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Dispenzieri  A , Fajgenbaum  DC .  Overview of Castleman disease.   Blood. 2020;135(16):1353-1364. doi:10.1182/blood.2019000931PubMedGoogle ScholarCrossref
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Wang  H-W , Pittaluga  S , Jaffe  ES .  Multicentric Castleman disease: where are we now?   Semin Diagn Pathol. 2016;33(5):294-306. doi:10.1053/j.semdp.2016.05.006PubMedGoogle ScholarCrossref
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Fajgenbaum  DC , Shilling  D .  Castleman disease pathogenesis.   Hematol Oncol Clin North Am. 2018;32(1):11-21. doi:10.1016/j.hoc.2017.09.002PubMedGoogle ScholarCrossref
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Pria  AD , Pinato  D , Roe  J , Naresh  K , Nelson  M , Bower  M .  Relapse of HHV8-positive multicentric Castleman disease following rituximab-based therapy in HIV-positive patients.   Blood. 2017;129(15):2143-2147. doi:10.1182/blood-2016-10-747477PubMedGoogle ScholarCrossref
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Dossier  A , Meignin  V , Fieschi  C , Boutboul  D , Oksenhendler  E , Galicier  L .  Human herpesvirus 8–related Castleman disease in the absence of HIV infection.   Clin Infect Dis. 2013;56(6):833-842. doi:10.1093/cid/cis1009PubMedGoogle ScholarCrossref
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Lurain  K , Yarchoan  R , Uldrick  TS .  Treatment of Kaposi sarcoma herpesvirus–associated multicentric Castleman disease.   Hematol Oncol Clin North Am. 2018;32(1):75-88. doi:10.1016/j.hoc.2017.09.007PubMedGoogle ScholarCrossref
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