University of Utah Health Salt Lake City, UT, United States
Keegan Colletier, MD1, Dan McEntire, MD2 1University of Utah Health, Salt Lake City, UT; 2University of Utah, Salt Lake City, UT
Introduction: Castleman disease is a rare non-clonal lymphoproliferative disorder characterized by non-neoplastic lymphadenopathy that can affect any region of the body. Castleman disease adenopathy can either be localized to a single site, or more widespread. In the latter case, Multicentric Castleman Disease (MCD) is further classified as either HHV-8 negative/idiopathic MCD or HHV-8 associated MCD. MCD can present aggressively with systemic manifestations, and treatment differs based on the presence of Kaposi Sarcoma (KS). In this report, we describe a patient with HIV/AIDS that was found to have diffuse gastrointestinal KS and was ultimately diagnosed with HHV-8 associated MCD.
Case Description/Methods: A 44 year old male with recently diagnosed HIV/AIDS and cutaneous KS presented with complaints of night sweats, dizziness, weight loss, and diarrhea. He reported waxing and waning lymphadenopathy present for at least one year. Physical examination was notable for diffuse lymphadenopathy and an enlarged spleen. Initial laboratory studies were remarkable for CD4 count of 70, Hgb 6.0g/dL, platelets 91 k/uL, sodium of 121mmol/L. CT of the abdomen and pelvis demonstrated diffuse lymphadenopathy as well as marked splenomegaly. CT of the chest showed thoracic lymphadenopathy, and MRI Brain revealed cervical chain lymphadenopathy. GI was consulted to evaluate for systemic/GI KS or other lesions to guide definitive treatment. EGD and colonoscopy demonstrated nodules in the stomach, ascending colon, and rectum all of which were biopsied and revealed KS. An excisional biopsy of an inguinal lymph node was obtained, pathology revealed Castleman lymphadenopathy, plasma cell variant. Oncology recommended that the patient be started on rituximab and liposomal doxorubicin for treatment of HHV-8 associated MCD with concurrent Kaposi Sarcoma.
Discussion: We present a rare case of HHV-8 associated MCD with concomitant KS found diffusely in the GI tract and skin. There was initially significant diagnostic uncertainty until lymph node biopsy was performed. Interestingly, therapeutic recommendations for MCD depend on the presence or absence of KS, and gastrointestinal KS can be present without cutaneous KS. MCD without KS is treated with four weeks of rituximab, and if KS is present, doxorubicin or etoposide is included. This case highlights the potential for GI involvement of KS in MCD, and in particular the need to pursue endoscopy in patients with HHV-8 associated MCD to evaluate for KS and guide treatment decisions.
Figure: Kapsoi Sarcoma in the gastric antrum (a), gastric body (b), sigmoid colon (c), and rectum (d).
Disclosures: Keegan Colletier indicated no relevant financial relationships. Dan McEntire indicated no relevant financial relationships.
Keegan Colletier, MD1, Dan McEntire, MD2. P1484 - Diffuse Gastrointestinal Kaposi Sarcoma in a Patient With HHV-8 Associated Multicentric Castleman Disease, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.