{"doi":"10.1002/ajh.27065","title":"Ongoing symptoms following complete surgical excision in unicentric Castleman disease","abstract":"Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with shared lymph node (LN) histology.1 While multicentric CD (MCD) involves multiple enlarged LNs, systemic inflammation, and multi-organ failure, unicentric CD (UCD) involves a single enlarged LN or region of LNs and few to no symptoms.1 Though compressive symptoms are the best-described clinical manifestation of UCD, some patients can have an MCD-like inflammatory syndrome.2 In UCD, complete surgical excision (herein abbreviated “CLE”) of the enlarged LN(s) is reported to induce complete remission in ~96% of patients.1 Despite reports of high response rates to CLE, there have been anecdotal reports of unresolved symptoms post-CLE by patients in the Castleman Disease Collaborative Network (CDCN), a non-profit research and patient advocacy organization, as well as by CDCN-affiliated physicians. Unfortunately, there is limited published data on unresolved symptoms in UCD patients following complete surgical excision.3 ACCELERATE, a longitudinal CD natural history study, is well-positioned to systematically investigate the prevalence of ongoing symptoms in UCD patients after CLE. We investigated real-world data from ACCELERATE and utilized patient administered surveys to characterize UCD symptoms before and after CLE to determine the prevalence of ongoing symptoms. Patients included in the study enrolled into ACCELERATE between October 2016 and November 2020. Ninety-nine patients who self-reported UCD or were diagnosed with UCD by their physicians were reviewed by an expert panel of CD clinicians and pathologists to confirm the likelihood of a UCD diagnosis based on diagnostic pathology slides and extensive medical data. In total, 60 patients had a confirmed UCD diagnosis, 22 likely had another disease (i.e., another reactive/malignant process) and 17 possibly had UCD but lacked pathology slides for confirmation. Analyses were conducted on the 60 confirmed UCD patients. All 99 patients were also invited to participate in validated surveys (EQ-5D-5L, CD Symptom Score) and an additional questionnaire regarding ongoing symptoms. Among the 60 UCD patients, there were 15 males (25.0%) and 45 females (75.0%); patients were predominantly white (93.3%). Mean (standard deviation [SD]) age was 39.2 (12.9) years, and the mean (SD) follow-up was 3.0 (3.9) years. Most patients demonstrated hyaline-vascular histopathology (85.0%, n = 51; Table S1). Among 53 patients with assessable data at disease onset, 28 (52.8%) presented asymptomatically, including 22 (78.6%) with no abnormal labs. Twenty-five (47.2%) patients presented with constitutional symptoms, including fatigue (46.3%), night sweats (29.7%), unintentional weight loss (16.3%), and/or fever (9.8%). Apart from elevated erythrocyte sedimentation rate (ESR), which was abnormal in 4/7 (57.1%) patients in which it was measured, no other labs were abnormal in more than 50.0% of patients and only 30.0% (16/53) of patients had laboratory abnormalities at presentation. Of note, two patients demonstrated markedly elevated C-reactive protein (CRP) levels of 139.7 and 74.5 mg/L, and one patient had a markedly high ESR of 95 mm/h. We examined the efficacy of CLE in this cohort from medical record data, both clinically and radiologically. 55/60 (91.7%) received a CLE, of which 53 (96.4%) patients had sufficient data to assess a clinical and/or radiologic response (Figure 1A). While 41/44 (93.2%) patients demonstrated radiologic resolution to CLE, only 32/43 (74.4%) patients with sufficient clinical data had clinical response. Among the five patients where CLE was not performed due to the size/location of the mass, an incomplete surgical excision was performed instead, in which 4/5 (80.0%) patients did not achieve a clinical response (Table S2). We found a similar proportion of patients with symptoms at presentation between CLE responders (n = 32) and non-responders (n = 11; Table S3). A total of 7/11 (63.6%) non-respon","journal":"American Journal of Hematology","year":2023,"id":365999,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":6,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9634,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2023-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":187159,"name":"Saishravan Shyamsundar","orcid":"0000-0002-0160-8910","position":1,"is_corresponding":false},{"id":1121840,"name":"Freda Coren","orcid":"0000-0003-0281-5011","position":2,"is_corresponding":false},{"id":737143,"name":"Adam Bagg","orcid":"0000-0002-1669-468X","position":3,"is_corresponding":false},{"id":434075,"name":"Gordan Srkalović","orcid":"0000-0003-3519-0409","position":4,"is_corresponding":false},{"id":795051,"name":"Daisy Alapat","orcid":"0000-0002-3414-8375","position":5,"is_corresponding":false},{"id":61737,"name":"Frits van Rhee","orcid":"0000-0001-9959-1282","position":6,"is_corresponding":false},{"id":281051,"name":"Megan S. Lim","orcid":"0000-0002-0415-2867","position":7,"is_corresponding":false},{"id":447598,"name":"Mary Jo Lechowicz","orcid":"0000-0003-1997-1950","position":8,"is_corresponding":false},{"id":821294,"name":"Joshua D. Brandstadter","orcid":"0000-0003-0652-5791","position":9,"is_corresponding":false},{"id":295573,"name":"Sheila K. Pierson","orcid":"0000-0002-3736-1411","position":10,"is_corresponding":false},{"id":295575,"name":"David C. Fajgenbaum","orcid":"0000-0002-7367-8184","position":11,"is_corresponding":false},{"id":1044252,"name":"Mateo Sarmiento Bustamante","orcid":"0009-0002-6774-2714","position":0,"is_corresponding":true}],"reference_count":6,"raw_metadata":null,"created_at":"2026-07-19T01:14:50.885797Z","pmid":"37635628","pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}