Abstract
Castleman disease (CD) represents a heterogeneous group of hematologic disorders characterized by distinct histopathological features. While CD is regarded as a pre-lymphomatous condition, its reactive or neoplastic nature is still a matter of controversy. Although rare, lymphoma has been reported in patients with CD, most commonly B-cell lymphomas. Here, we present a case of a 69-year-old man initially diagnosed with CD who subsequently developed angioimmunoblastic T-cell lymphoma (AITL) during treatment. To elucidate the potential biological relationship between these two disease entities, we performed whole-exome sequencing to compare genomic alterations between the two disease states. Additionally, we provide a concise review of published cases documenting lymphoma occurring after or coexisting with CD, summarizing patients’ characteristics and the various treatment strategies employed thus far.
Supplementary Information
The online version contains supplementary material available at https://doi.org/10.1007/s00277-026-07199-3.
Keywords: Castleman’s disease, Angioimmunoblastic T-cell lymphoma, Lymphoma development; immune dysregulation, Genomic alterations
Introduction
Castleman’s disease (CD) is a heterogeneous group of hematologic diseases with characteristic histopathological abnormalities [1–3]. According to the most recent WHO classification of hematolymphoid tumors (WHO-HAEM5), Castleman disease is categorized under ‘tumour-like lesions with B-cell predominance’. It is classified as either unicentric (UCD) or multicentric (MCD) depending on the involved lymph node (LN) stations [4]. Human herpesvirus 8 (HHV-8) was found to be one of the etiologic agent of MCD, hence MCD has been classified into HHV-8 associated MCD and HHV-8 negative MCD. The latter is referred to as idiopathic multicentric Castleman disease (iMCD), which encompasses a heterogeneous group of HHV-8-negative cases characterized by systemic inflammation and multicentric lymphadenopathy.” [5].
The diagnosis of CD requires the exclusion of other conditions that may present with similar LNs pathology, among which lymphoma is of paramount importance. However, few studies have reported that patients with CD could develop lymphomas during treatment [6]. Most of these cases involved B-cell lymphomas and only < 5% cases were develop to T-cell lymphomas. The mechanisms underlying the association between CD and B-cell lymphomas are at least partially understood, particularly in HHV-8-associated cases, where HHV-8 infection of plasmablasts and immunodeficiency are thought to play key roles in lymphomagenesis. However, the biological relationship between CD and T-cell lymphomas remains currently unknown.
In this study, we report a case of a patient who was firstly diagnosed as CD and subsequently developed AITL during treatment. Only 2 such cases have been reported where patients with CD subsequently developed PTCL [12, 15], and our case is the first reported case in which a patient with CD subsequently developed angioimmunoblastic T-cell lymphoma (AITL). This distinction is particularly important, as lymphomas such as AITL may occasionally mimic Castleman disease, especially in early stages.We further performed whole exome sequencing to compare the difference between the status of these two diseases. We also provide a brief review of published cases of lymphoma occurring after or coexisting with CD, summarizing the characteristics of patients and the different treatment modalities used so far. In addition, we present the role of P2RY8, KMT2D and STK11 in lymphoma, the mutation genes identified in our patient.
Materials and methods
Case presentation
A previously healthy 69-year-old man was presented to our hospital two years ago with eyelids and lower extremity edema accompanied by hypertension. Renal biopsy suggested Minimal Change Disease, which improved with diuretic treatment. Laboratory tests revealed an ESR of 32 mm/1 h, CRP < 10 mg/L, Hb 121 g/L, ALB 18 g/L, 24-hour urine protein 4.9 g/day, PLT 189 × 10^9/L, eGFR 65 mL/min/1.73 m², and IgG 1096 mg/dL. During hospitalization, superficial lymph node ultrasound revealed multiple enlarged lymph nodes in the bilateral cervical, inguinal, and axillary regions. Abdominal CT showed enlarged lymph nodes adjacent to the left iliac vessels and in the left inguinal region. A subsequent cervical lymph node biopsy was performed, and a large number of plasma cells were found proliferating in the LN. The immunohistochemistry results are shown in (Fig. 1): CD20 (B-cell zone+), CD3 (T-cell zone+), CD10 and BCL-6 (germinal center+), Ki-67 (30%+ in extrafollicular areas), CD30 (scattered+), CD21 (FDC+), Bcl-2 (germinal center-), EBER (-), Cyclin D1 (-), Kappa (+), Lambda (+), CD138 (plasma cells+), IgG (plasma cells+), IgG4 (plasma cells+, up to 100/HPF), IgG4/IgG < 40%, CD38 (plasma cells+), MUM1 (plasma cells+), CD56 (-), EMA (plasma cells+), HHV8 (-). The pathological diagnosis was lymph node lymphoid hyperplasia with prominent plasma cell infiltration and increased IgG4-positive plasma cells. Given a serum IgG4 level of 0.211 g/L, IgG4-related autoimmune disease was ruled out, leading to a final diagnosis of HHV8-negative iMCD, not otherwise specified. Bone marrow examination showed no evidence of lymphoma involvement.
Fig. 1.
Pathological findings of Castleman disease in the neck lymph node
To further exclude the possibility of an underlying T-cell lymphoma at initial diagnosis, additional immunohistochemical and molecular analyses were performed on the initial lymph node specimen (Fig. 1). Staining for follicular helper T-cell (TFH) markers showed that PD1- and ICOS-positive cells were present but sparse and scattered, without evidence of clustering, perifollicular aggregation, or meshwork-like expansion.EBER in situ hybridization revealed only rare scattered positive cells, without evidence of EBV-positive B-cell proliferation.Furthermore, T-cell receptor (TCR) gene rearrangement analysis did not demonstrate a clonal T-cell population, indicating the absence of a clonal T-cell population at that time point (Fig. 2). These findings, including preserved nodal architecture, prominent interfollicular plasmacytosis, polytypic plasma cell proliferation, and absence of TFH-cell expansion or T-cell clonality, supported a diagnosis of Castleman disease rather than T-cell lymphoma at that time.
Fig. 2.
T-cell receptor (TCR) gene rearrangement analysis of the initial lymph node biopsy
The patient was administered tocilizumab/predisone every two weeks for 6 months and LNs were significantly shrunken. This was followed by maintenance by rituximab every three months.
However, during the follow-up, on December 5, 2023, lymphadenopathy recurred, and a repeat cervical lymph node biopsy led to the diagnosis of peripheral T-cell non-Hodgkin lymphoma, angioimmunoblastic type. The immunohistochemistry results are shown in (Fig. 3): CD2, CD3, CD4, CD5, CD7, TIA1, PD1, and ICOS all positive; CD10 and BCL6 partially positive; MUM1 (plasma cells+); CD21 and CD23 (disrupted FDC networks+); CD30 (20%+); Ki-67 (60%+); EBER (scattered+); CD20, CD56, CXCL13, and ALK negative. TCR gene rearrangement was positive. We also performed whole exome sequencing (WES) on the specimens of the CD and the AITL lymph node biopsies, with the results presented in (Supplementary Fig. 1). Comparative analysis revealed differential somatic mutations in STK11, P2RY8 and KMT2D between the two disease states. And WES identified a TET2 mutation (TET2:NM_001127208.2: exon 10) in both the initial and subsequent samples, whereas no RHOA or IDH2 mutations were detected, further whereas no RHOA or IDH2 mutations were detected, further supporting the absence of evidence for an underlying T-cell lymphoma at the initial diagnosis.
Fig. 3.
Pathological findings of Angioimmunoblastic T-cell Lymphoma in the neck lymph node
After definitive diagnosis, the patient received bortezomib, cyclophosphamide and dexamethasone (BCD) regimen chemotherapy. This time, he was evaluated by Fluorine-18 labeled fluorodeoxyglocose (FDG) positron emission tomography (PET)/CT. As seen in (Fig. 4A), PET-CT showed multiple hyper-metabolic and enlarged LNs along the bilateral iliac vascular and right inguinal region. Furthermore, bone marrow involvement was under consideration.
Fig. 4.
Serial PET/CT imaging during the disease progression
From January 9, 2024, AZA plus cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) was administered for four cycles. Nonetheless, follow-up PET-CT (Fig. 4B) showed many new and hypermetabolic LNs developed above and below the diaphragm as well as a new hypermetabolic mass diffusely involving the spleen, which reminded us that the disease was persistently progressing.
Because of continued clinical disease progression, a repeat lymph node biopsy was performed, which again confirmed the diagnosis of AITL. Subsequent therapies included chidamide/thalidomide/azacitidine, chidamide/siltuximab, and siltuximab/bortezomib, achieving transient disease stabilization. In January 2025, the patient was re-admitted with recurrent lymphadenopathy and later succumbed to infection.
Search strategy
A systematic search of the literature on the PubMed database was conducted in January 2025 using the following search terms: Castleman’s disease, lymphoma, non-Hodgkin’s lymphoma, Hodgkin’s disease. The inclusion criteria in this study were English language reports of patients who were initially diagnosed with CD firstly and subsequently developed lymphoma or had coexisting lymphoma published between 1991 and 2025. The search yielded 11 case reports and 4 reviews that met the inclusion criteria. We analyzed the 11 case reports that included a total of 17 patients.
Results
The characteristics of the 17 patients are summarized in Table 1. All patients were diagnosed as having Castleman’s disease through pathological reports and clinical symptoms. Among them, 13 were classified as MCD and 4 were UCD, with MCD patients exhibiting a poorer prognosis. According to the disease progression described in the case reports, 13 patients developed lymphoma secondary to CD, while 4 patients had concurrent CD and lymphoma. DLBCL and PTCL cases were all occurred after the diagnosis of CD, whereas primary effusion lymphoma (PEL) and Hodgkin’s disease (HD) cases included both transformed and coexisting instances. However, this does not necessarily indicate a definitive pattern, as some cases were quite old, and some lacked detailed descriptions of the disease course or pathological changes, such as the three cases in the 10th case report. In contrast, our case included paired lymph node biopsies obtained at the CD and AITL stages, with whole-exome sequencing demonstrating distinct genomic alterations between the two disease states.
Table 1.
The characteristics of the patients
| No. | Age | Sex | HHV8 | Subsequent / Concurrent | Transformed Subtype | CD treatment | Lymophoma treatment | Follow up |
|---|---|---|---|---|---|---|---|---|
| 1 | 58 | Male | + | Subsequent | Large B-cell lymphoma (plasmablastic) | / | / | NA [7] |
| 2 | 68 | Female | NA | Subsequent | PTCL-U | Chinese Medicine | Chidamid、GemOx、GDP | Survival [8] |
| 3 | 58 | Male | NA | Concurrent | HD, nodular sclerosis | VBM + Radiotherapy | NA [9] | |
| 4 | 43 | Male | + | Concurrent | PEL | Restarted cART, declined proposed chemotherapy of rituximab | NA [10] | |
| 5 | 25 | Male | + | Concurrent | PEL | V-Epoch, ICE + R, allo-HCT, daratumumab+Gemcitabine+Oxaliplatin | Death [11] | |
| 6 | 37 | Male | NA | Subsequent | PTCL、LDCHL | CHOP | IVAM, BEAM, auto-PBSCT for PTCL, ABVD for HD, DHAP for both | Death [12] |
| 7 | 73 | Male | + | Subsequent | PEL | cyclophosphamide and prednisone | doxorubicin, vincristine and steroiss | Death [13] |
| 8 | 57 | Female | NA | Concurrent | Primary cutaneous MZBL | watch and wait for 2 years and CHOP | Death [14] | |
| 9 | 46 | Male | NA | Subsequent | DLBCL | 2-CDA | anthracyclin-based chemotherapy, DHAP, MINE | Death [15] |
| 62 | Male | + | Subsequent | PTCL | 2-CDA | Chlorambucil, prednisone | Death [15] | |
| 10 | 30 | Male | NA | Subsequent | HD, mixed cellularity | radiotherapy | chemotherapy | Survival [16] |
| 18 | Male | NA | Subsequent | HD, nodular sclerosis | Chemotherapy, Radiotherapy | chemotherapy | Survival [16] | |
| 40 | Male | NA | Subsequent | HD, nodular sclerosis | radiotherapy | chemotherapy | Survival [16] | |
| 11 | 48 | Male | + | Subsequent | PEL | HAART | early death | Death [17] |
| 67 | Male | + | Subsequent | PEL | HAART | early death | Death [17] | |
| 41 | Male | + | Subsequent | PEL | HAART | early death | Death [17] | |
| 41 | Male | + | Subsequent | PEL | HAART, Splenectomy | CHOP | Death [17] | |
HHV8 is almost always universally positive in classic PEL. Our review of case reports also identified HHV8 positivity in patients who subsequently developed PTCL or large B-cell lymphoma. Notably, some of these patients were HIV-positive, suggesting that immunocompromised individuals may be more prone to developing lymphoma.
Regarding treatment, management strategies for patients with lymphoma varied: CD-specific approaches included watch and wait, CHOP chemotherapy, radiotherapy, traditional Chinese medicine, and 2-Chloro-deoxyadenosine (2-CDA). After the diagnosis of lymphoma, targeted therapies such as EPOCH, ICE and autologous transplantation were initiated. However, both our case and previous reports indicate that lymphoma occurring after or coexisting with CD generally have a poor prognosis.
Discussion
Castleman’s disease (CD) is a non-malignant polyclonal lymphoid proliferation of unknown etiology [18]. It is considered a pre-lymphomatous state, whose reactive or neoplastic nature is still a matter of controversy. Although reported cases are limited, both lymphoma occurring after CD have been indicated, with PEL being the most frequently reported subtype.
In this article, we report a case initially diagnosed with MCD via cervical lymph node biopsy. The patient received over one year of tocilizumab and rituximab therapy without disease control. A repeat cervical lymph node biopsy revealed subsequently confirmed AITL, and subsequent treatments included BCD regimen, AZA+CHOP, and chidamide plus siltuximab. While transient disease stabilization was achieved, the patient ultimately experienced relapse and died of infectious complications. This represents the first reported case in which a patient with MCD subsequently developed AITL based on the currently available literature. In addition, to our knowledge, this is the first study worldwide to perform whole-exome sequencing on paired CD and AITL specimens from the same patient. Comparative analysis revealed differential somatic mutations in STK11, P2RY8 and KMT2D between the two disease states.
STK11 is a critical tumor suppressor gene, playing roles in cellular energy regulation and DNA damage repair [19]. The mutations of STK11 lead to aberrant mTOR activation via its downstream substrate AMPK. Studies have demonstrated that the STK11/AMPK/TSC/mTOR pathway contributes to the proliferation and metastasis of mantle cell lymphoma (MCL) [20]. Furthermore, P2RY8 was reported to have oncogenic potential. Somatic mutations in P2RY8 lead to the oncogenesis of diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL), because of the loss of its growth-suppressive function and disruption of germinal center (GC) B cell migration via Gα13 inhibition [21]. As for KMT2D, it is one of the most frequently mutated genes in GC-derived B-cell malignancies and has been recognized as a tumor suppressor [22]. Notably, preclinical studies suggest that chidamide combined with decitabine may target KMT2D deficiency [23], potentially explaining the transient disease stabilization observed in our case. In T-cell lymphomas, KMT2D mutations are associated with shorter overall survival, suggesting that KMT2D-related epigenetic dysregulation may have contributed to the development of AITL in our patient. However, the relevant mechanism needs to be further elucidated.
Our literature review found that most reported cases of lymphoma occurring after or coexisting with CD involved PEL or HD, while AITL occurring after CD has been extremely rare. Our case represents the first reported case in which a patient with CD subsequently developed AITL. Regarding whether these cases represent concurrent CD and lymphoma or the subsequent development of lymphoma after CD, the distinction remains unclear due to limited historical data: many older reports lacked detailed testing or descriptions, posing limitations. However, in our patient, we definitively confirmed the development of AITL following the diagnosis of CD. Multiple sequential lymph node biopsies during disease progression revealed complete architectural disruption, and WES further demonstrated distinct genomic alterations between the CD and AITL stages. A key diagnostic challenge in this case is distinguishing subsequent development of AITL from an unrecognized early AITL. Early (pattern I) AITL may exhibit subtle histological features and overlap with reactive conditions. However, even in such cases, at least partial evidence of TFH-cell expansion, emerging clonality, or lymphoma-associated molecular alterations is typically present. In contrast, the initial biopsy in our case showed no evidence of TFH-cell expansion (PD1/ICOS), no EBV-positive B-cell proliferation, and no clonal T-cell population by TCR rearrangement analysis. In addition, although a TET2 mutation was identified, it was present in both disease stages and occurred in isolation, without accompanying RHOA or IDH2 mutations, which are more characteristic of TFH lymphomas. These findings do not meet the criteria for even early (pattern I) AITL and instead support a reactive or Castleman disease-like process at initial presentation. The subsequent emergence of overt AITL with characteristic immunophenotypic and molecular features is therefore most consistent with disease evolution rather than a missed diagnosis at baseline.Taken together, the absence of TFH expansion, lack of T-cell clonality, and non-specific molecular findings at baseline strongly argue against an early AITL, supporting the subsequent development of AITL during follow-up.
The etiology underlying the occurrence of lymphoma in patients with CD remains uncertain. Our literature analysis suggests that HHV8 is not only associated with PEL but may also play a role in development of PTCL. Thus, viral infections (e.g., HHV8 or EBV) may contribute to this process. Additionally, reported HIV-positive cases more frequently developed lymphoma after CD or concurrent diagnoses, implying that immunosuppression may be a risk factor. Notably, one case series described lymphoma in 2 of 3 CD patients treated with 2-CDA, and suggested that certain medications might also induce malignant progression. Taking our genomic findings as a basis, whether KMT2D, P2RY8 and STK11 directly contribute to the subsequent development of AITL in patients with CD requires further investigation.
The relationship between Castleman disease (CD) and angioimmunoblastic T-cell lymphoma (AITL) remains incompletely understood. Both diseases are characterized by immune dysregulation and remodeling of the lymphoid microenvironment, resulting in overlapping clinicopathological features in some cases [24]. Early AITL may exhibit Castleman-like changes, including plasmacytosis, vascular proliferation, and abnormal follicular dendritic cell (FDC) networks, making distinction from CD challenging [25]. Therefore, evaluation of TFH-cell expansion, T-cell clonality, and lymphoma-associated molecular alterations is essential for accurate diagnosis [26]. Although lymphoma occurring in patients with CD has been reported, most cases involve B-cell lymphomas, particularly HHV8-associated lymphoproliferative disorders, whereas AITL following CD is exceedingly rare. Current evidence does not support CD as a direct precursor of AITL; instead, these cases may reflect shared immune dysregulation or an evolving lymphoid neoplastic process. In our patient, the absence of TFH-cell expansion, T-cell clonality, and characteristic AITL-associated molecular alterations at initial diagnosis, together with the subsequent emergence of overt AITL, supports the development of AITL during follow-up rather than an initially missed lymphoma.
As for prognosis, both our case and the reviewed literature indicate poor prognosis after lymphoma develops, likely due to the prolonged disease course and compromised immunity. Several patients died of liver failure or sepsis.
In summary, this study presents the first reported case of a patient with MCD subsequently developed AITL, supported by WES of the patient’s lymphoid tissue revealing mutations in STK11, P2RY8 and KMT2D. The findings suggest a potential role for these genes in the development of AITL, warranting further mechanistic investigation. We also conducted a comprehensive review of cases involving either lymphoma occurring after or coexisting with CD, providing critical insights into the association between CD and lymphomagenesis.
a-c: H&E showed (a) Reactive follicular hyperplasia with focal germinal center atrophy and loss, accompanied by dilated lymphatic sinuses (25x). (b) Diffuse interfollicular plasma cell proliferation and hyalinization of small blood vessels; characteristic “onion-skin” structures are inconspicuous (200x). (c) Higher magnification shows prominent proliferation of high endothelial venules (1000x). d: CD20, demonstrates expanded mantle zones surrounding multiple hyperplastic lymphoid follicles, with focal germinal center atrophy (100x). e: CD38, diffusely positive in the interfollicular plasma cells (200x). f: CD21, shows Disorganized follicular dendritic cell networks with both hyperplastic and atrophic germinal centers (200x).g-h: Polytypic expression of κ (g) and λ (h) without light chain restriction (400x). i–k: Immunohistochemical and in situ hybridization studies performed on the initial biopsy: ICOS (i) and PD1 (j) highlight sparse, scattered TFH cells without clustering or perifollicular expansion; EBER (k) shows only rare scattered positive cells without evidence of EBV-positive B-cell proliferation (×100).
Multiplex PCR-based analysis of (A) TCRβ, (B) TCRγ, and (C) TCRδ gene rearrangements was performed and analyzed by capillary electrophoresis. The electropherograms demonstrate a polyclonal pattern with multiple low-amplitude peaks and no dominant clonal peak, consistent with a reactive T-cell population and absence of T-cell clonality.
a-b: H&E showed (a) The lymph node demonstrates effaced architecture with indistinct capsular borders, showing infiltration by aggregates of pale-staining polygonal cells into pericapsular adipose tissue (200x). (b) Characteristic branching proliferation of high endothelial venules with architectural distortion. The neoplastic lymphocytes exhibit small-to-medium size, irregular nuclear contours, and pale cytoplasm (1000x). c: CD3, Diffuse positive (200x). d: CD21, Disorganized and expanded follicular dendritic cell networks with typical “windblown” pattern (200x). e: Ki67, High proliferative activity in neoplastic foci (200x). f-h: CD10, ICOS, and PD-1, the markers of follicular helper T-cell markers are positive (200x).i: EBER, positive (400x).
a.Initial FDG PET/CT taken on January 2024 showed multiple hyper-metabolic and enlarged LNs along the bilateral iliac vascular and right inguinal region. b. FDG PET/CT taken on April 2024 showed many new and hypermetabolic LNs developed above and below the diaphragm as well as a new hypermetabolic mass diffusely involving the spleen.
Electronic Supplementary Material
Below is the link to the electronic supplementary material.
Author contributions
W.W. and R.X. were responsible for conceptualization, methodology, and software; X.H. and D.Z. handled data curation and wrote the original draft; J.W. performed visualization and investigation; Z.C. supervised the research; X.H., R.X., and J.H. were involved in writing - reviewing and editing, and all authors reviewed the final manuscript.
Funding
This work was supported by the National Natural Science Foundation of China (Project no. 82300231).
Data availability
No datasets were generated or analysed during the current study.
Declarations
Ethics approval
This study was performed in line with the principles of the Declaration of Helsinki. Approval was granted by the Ethics Committee of the first affiliated hospital, School of Medicine, Zhejiang University. Informed consent was obtained from the individual participant included in the study.
Consent to participate
Informed consent was obtained from the individual participant included in the study.
Consent to publish
Written informed consent was obtained for publication of the case report and any accompanying images from the patient or their legal representative.
Competing interests
The authors declare no competing interests.
Footnotes
Publisher’s note
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References
- 1.Dispenzieri A, Fajgenbaum DC (2020) Overview of Castleman disease. Blood 135(16):1353–1364. 10.1182/blood.2019000931 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Carbone A, Borok M, Damania B et al (2021) Castleman disease. Nat Rev Dis Primers 7(1):84 Published 2021 Nov 25. 10.1038/s41572-021-00317-7 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Chen LYC, Zhang L, Fajgenbaum DC (2025) Expert perspective: Diagnosis and treatment of castleman disease. Arthritis Rheumatol Published online June 2. 10.1002/art.43269 [DOI] [PMC free article] [PubMed]
- 4.Lang E, van Rhee F (2024) Idiopathic multicentric Castleman disease: An update in diagnosis and treatment advances. Blood Rev 64:101161. 10.1016/j.blre.2023.101161 [DOI] [PubMed] [Google Scholar]
- 5.Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Lim MS (2017) International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood 129(12):1646–1657. 10.1182/blood-2016-10-746933 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 6.Larroche C, Cacoub P, Soulier J et al (2002) Castleman’s disease and lymphoma: report of eight cases in HIV-negative patients and literature review. Am J Hematol 69(2):119–126. 10.1002/ajh.10022 [DOI] [PubMed] [Google Scholar]
- 7.Montroni I, Sabattini E, Ramadori E et al (2014) Small bowel obstruction caused by a large B-cell lymphoma in a patient with multicentric Castleman’s disease: An unusual occurrence. Int J Surg Pathol 22(5):434–437. 10.1177/1066896913507599 [DOI] [PubMed] [Google Scholar]
- 8.Liu S, Wang Y, Hu T, Zhang C, Zheng Z (2020) Peripheral T cell lymphoma coexisting with Castleman’s disease: A case report and literature review. Med (Baltim) 99(2):e18650. 10.1097/MD.0000000000018650 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 9.Saletti P, Ghielmini M, Scali G, Pedrinis E, Guéneau M, Cavalli F (1999) Hodgkin’s and Castleman’s disease in a patient with systemic mastocytosis. Ann Hematol 78(2):97–100. 10.1007/s002770050482 [DOI] [PubMed] [Google Scholar]
- 10.Scott MJ, Alhariri BC, Farghaly H, Perez R, Arnold FW (2014) Castleman’s disease and primary effusion lymphoma in a HIV-positive patient. Int J STD AIDS 25(6):455–457. 10.1177/0956462413512805 [DOI] [PubMed] [Google Scholar]
- 11.Caballero JC, Pardo L, Rodriguez-Pinilla MS et al (2023) Primary Effusion Lymphoma, Multicentric Castleman’s Disease, and Kaposi’s Sarcoma in an HHV-8 and HIV-Positive Patient: A Case Report. Med (Kaunas) 59(6):1049 Published 2023 May 30. 10.3390/medicina59061049 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 12.Park J, Lee JE, Kim M et al (2013) Discordant lymphocyte-depleted classical Hodgkin’s and peripheral T-cell lymphoma arising in a patient 11 years after diagnosis of multicentric Castleman’s disease. Int J Hematol 98(1):114–121. 10.1007/s12185-013-1358-0 [DOI] [PubMed] [Google Scholar]
- 13.Teruya-Feldstein J, Zauber P, Setsuda JE et al (1999) Expression of human herpesvirus-8 oncogene and cytokine homologues in an HIV-seronegative patient with multicentric Castleman’s disease and primary effusion lymphoma [published correction appears in Lab Invest. ;79(7):835]. Lab Invest. 1998;78(12):1637–1642 [PubMed]
- 14.Tsukamoto N, Kojima M, Uchiyama T et al (2007) Primary cutaneous CD5 + marginal zone B-cell lymphoma resembling the plasma cell variant of Castleman’s disease. Case Rep APMIS 115(12):1426–1431. 10.1111/j.1600-0463.2007.00797.x [DOI] [PubMed] [Google Scholar]
- 15.Colleoni GW, Duarte LC, Kerbauy FR et al (2003) 2-Chloro-deoxyadenosine induces durable complete remission in Castleman’s disease but may accelerate its transformation to non-Hodgkin’s lymphoma. Acta Oncol 42(7):784–787. 10.1080/02841860310003527 [DOI] [PubMed] [Google Scholar]
- 16.Maheswaran PR, Ramsay AD, Norton AJ, Roche WR (1991) Hodgkin’s disease presenting with the histological features of Castleman’s disease. Histopathology 18(3):249–253. 10.1111/j.1365-2559.1991.tb00833.x [DOI] [PubMed] [Google Scholar]
- 17.Ascoli V, Signoretti S, Onetti-Muda A et al (2001) Primary effusion lymphoma in HIV-infected patients with multicentric Castleman’s disease. J Pathol 193(2):200–209. 10.1002/1096-9896(200102)193:2<200:AID-PATH773>3.0.CO;2-L [DOI] [PubMed] [Google Scholar]
- 18.El-Osta HE, Kurzrock R (2011) Castleman’s disease: from basic mechanisms to molecular therapeutics. Oncologist 16(4):497–511. 10.1634/theoncologist.2010-0212 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 19.Skoulidis F, Heymach JV (2019) Co-occurring genomic alterations in non-small-cell lung cancer biology and therapy. Nat Rev Cancer 19(9):495–509. 10.1038/s41568-019-0179-8 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 20.Jiang C, Bi C, Jiang X et al (2019) The miR-17 ~ 92 cluster activates mTORC1 in mantle cell lymphoma by targeting multiple regulators in the STK11/AMPK/TSC/mTOR pathway. Br J Haematol 185(3):616–620. 10.1111/bjh.15591 [DOI] [PubMed] [Google Scholar]
- 21.Muppidi JR, Schmitz R, Green JA et al (2014) Loss of signalling via Gα13 in germinal centre B-cell-derived lymphoma. Nature 516(7530):254–258. 10.1038/nature13765 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 22.Li J, Chin CR, Ying HY et al (2024) Loss of CREBBP and KMT2D cooperate to accelerate lymphomagenesis and shape the lymphoma immune microenvironment. Nat Commun. ;15(1):2879. Published 2024 Apr 3. 10.1038/s41467-024-47012-1 [DOI] [PMC free article] [PubMed]
- 23.Ji MM, Huang YH, Huang JY et al (2018) Histone modifier gene mutations in peripheral T-cell lymphoma not otherwise specified. Haematologica 103(4):679–687. 10.3324/haematol.2017.182444 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 24.Fajgenbaum DC, Uldrick TS, Bagg A et al (2017) International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood 129(12):1646–1657. 10.1182/blood-2016-10-746933 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 25.Rodriguez-Justo M, Attygalle AD, Munson P, Roncador G, Marafioti T, Piris MA (2009) Angioimmunoblastic T-cell lymphoma with hyperplastic germinal centres: a neoplasia with origin in the outer zone of the germinal centre? Clinicopathological and immunohistochemical study of 10 cases with follicular T-cell markers. Mod Pathol 22(6):753–761. 10.1038/modpathol.2009.12 [DOI] [PubMed] [Google Scholar]
- 26.Bouska A, Zhang W, Sharma S et al (2025) Integrative Genomic and Transcriptomic Analysis Reveals Targetable Vulnerabilities in Angioimmunoblastic T-Cell Lymphoma. Am J Hematol 100(9):1486–1501. 10.1002/ajh.27736 [DOI] [PMC free article] [PubMed] [Google Scholar]
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Supplementary Materials
Data Availability Statement
No datasets were generated or analysed during the current study.