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Castleman disease, plasma cell variant with classic Hodgkin lymphoma involving lymph node (a–c). The overall lymph node architecture is well-preserved with focal fibrosis and marked plasmacytosis (b). Focally, a few follicles are small and have hyaline vascular features (a). Large atypical cells morphologically consistent with Hodgkin and Reed-Sternberg (HRS) cells are seen (c). These cells are present in a mixed inflammatory background composed of eosinophils, small lymphocytes, plasma cells, and histiocytes. The lymph node has a thickened capsule, and some fibrous bands, and scattered large atypical cells have retracted cytoplasm resembling lacunar cells. Immunohistochemical studies show that the HRS cells are positive for PAX-5 (weak) (d), CD30 (strong) (e), and CD15 (f), and are negative for CD20, CD45, OCT-2, and BOB.1 (not shown)
Source publication
Hodgkin lymphoma is a B cell neoplasm characterized by Hodgkin and Reed-Sternberg (HRS) cells in an inflammatory background. Classic Hodgkin lymphoma (CHL) accounts for approximately 90% of all cases of HL and four types are recognized in the World Health Organization (WHO) classification: lymphocyte-rich, nodular sclerosis, mixed cellularity, and...
Citations
... However, Hodgkin's lymphoma with coexisting Castleman disease shows diffuse histological findings of CD as seen in our case. Most of the cases of HL reported are of the interfollicular pattern and coexisted with the multicentric plasma cell variant of CD [7,9,16]. In our report also, one of the cases was of a plasma cell variant of CD with concomitant interfollicular HL with HHV-8-negative morphology on lymph node biopsy. ...
... Lyapichev et al. described three cases of CD with concurrent HL where the pattern of involvement was focal and involved only a minor portion of the lymph node biopsy specimen, contrary to our case where the pattern of involvement of HL was diffuse [16]. From the diagnostic point of view, it is important for the pathologists to be aware of the possible occurrence of HL foci in cases of CD, so as to avoid missing the diagnosis of malignancy. ...
... Whether or not HL overlaps with iMCD remains an open question. Indeed, there are several reports regarding the coexistence of HL and iMCD (17)(18)(19). In the current case, we believe our diagnosis was HL-compatible, as chemotherapy was effective for whole lesions and improved clinical symptoms, including B symptoms. ...
Hodgkin lymphoma (HL) and idiopathic multicentric Castleman disease (iMCD) are markedly different conditions. However, in some cases, histological similarities caused by elevated cytokines, including IL-6, can lead to a misdiagnosis of HL as CL. We herein report a patient with HL who had been diagnosed with Castleman disease by an expert panel and for whom an additional biopsy was useful for determining the correct diagnosis. Furthermore, we analyzed the positron emission tomography/computed tomography findings at the diagnosis and found that the maximum standardized uptake value was useful for distinguishing HL from iMCD.
... UCD and HL usually coexist in the same lymph node and is of the PC variant, while NHL is usually found in a distant anatomical site and is of the HV subtype [7]. In a recent review by Lyapichev et al., a total of 37 cases of CD and cHL were reported in the literature [8]. We report a case of UCD with cHL coexistent in the same lymph node that had a sustained remission following treatment with the standard cHL chemotherapy and consolidative radiotherapy. ...
... Interleukin-6 (IL-6) has been implicated in the pathogenesis of cHL and CD [9,10]. Furthermore, RS has been shown to produce IL-6, as evidenced by being positive for IL-6 and the fact that a higher IL-6 level can be used to predict relapse [8,11,12]. ...
... The presentation of our patient with systemic symptoms is not unusual, as about one half of patients with CD coexistent with cHL were reported to have B symptoms [13]. However, only 11% of patients were reported to have involvement of the axillary lymph node [8]. ...
Castleman disease (CD) is a rare lymphoproliferative disorder that is associated with an increased risk for lymphoma. The association between CD and classical Hodgkin lymphoma (HL) is rare. The patient described here is a 44-year-old, HIV-seronegative male who presented with significant weight loss, fever, night sweats, and right axillary swelling. Imaging showed bulky infraclavicular, subpectoral, and axillary lymph nodes. A biopsy revealed classical HL on the background of a human herpesvirus-8 (HHV-8)-negative plasma cell variant of CD. The patient had a complete remission after six cycles of doxorubicin, bleomycin, vincristine, and dacarbazine (ABVD) that were followed by consolidative radiotherapy and continued to be disease-free for more than two years.
... In these cases, the cHL involves the interfollicular areas and the Hodgkin and Reed-Sternberg (HRS) cells have a typical immunophenotype (CD15 þ/À , CD30 þ , PAX5 þ , CD45 À ). [48][49][50] In one case series of UCD associated with cHL, the HRS cells and plasma cells expressed IL6 in 2 cases, 48 suggesting that the interfollicular variant of cHL and UCD may be distinct entities with a common pathogenesis, possibly related to IL6 dysregulation. Less often, lymph nodes involved by cHL may show hyaline-vascular changes in follicles resembling hyalinevascular UCD or mixed/plasmacytic with hyaline-vascular features. ...
... In these cases, the cHL involves the interfollicular areas and the Hodgkin and Reed-Sternberg (HRS) cells have a typical immunophenotype (CD15 þ/À , CD30 þ , PAX5 þ , CD45 À ). [48][49][50] In one case series of UCD associated with cHL, the HRS cells and plasma cells expressed IL6 in 2 cases, 48 suggesting that the interfollicular variant of cHL and UCD may be distinct entities with a common pathogenesis, possibly related to IL6 dysregulation. Less often, lymph nodes involved by cHL may show hyaline-vascular changes in follicles resembling hyalinevascular UCD or mixed/plasmacytic with hyaline-vascular features. ...
Context.—:
Unicentric Castleman disease (UCD) is a dynamic entity with a wide spectrum of morphologic findings. UCD can be further subdivided into hyaline-vascular and mixed/plasmacytic variants. Hyaline-vascular UCD has both follicular and interfollicular (stromal) changes, and occasionally these lesions show a skewed representation of either the follicular or stromal compartments. Plasmacytosis is usually minimal in hyaline-vascular variant. Mixed/plasmacytic variant of UCD is composed of sheets of plasma cells often associated with a variable number of follicles with regressive changes.
Objective.—:
To illustrate the differential diagnosis of UCD, as it is quite broad and includes lymphomas, plasma cell neoplasms and stromal neoplasms such as follicular dendritic cell sarcoma and vascular neoplasms, immunoglobulin G4-related disease, infections, and other rare lesions. An additional objective is to enhance awareness of the morphologic features of UCD in excisional and in small core-needle biopsy specimens, the latter of which may inadvertently target follicle- or stroma-rich areas, causing diagnostic challenges.
Data sources.—:
In this review, we provide the readership a concise illustration of the morphologic spectrum of UCD that we have encountered in our practice and a brief discussion of entities in the differential diagnosis.
Conclusions.—:
UCD exhibits a broad spectrum of morphologic changes, and awareness of these morphologic variations is key to avoid misdiagnosis.
... In the index case, our smear does not have prominent capillaries. Malzone et al. describe the imaging findings of a hypervascular mass with a welldelineated margin, which is hypoechogenic [14]. ...
Castleman disease (CD) is a rare benign disorder presents as a lymph nodal mass in mediastinum, cervical, axillary or abdomen. Due to the presence of dysplastic dendritic cell in a background mature lymphocyte and plasma cell, it mimics Hodgkin disease (HD). Synchronous and metachronous occurrence in HD and CD can also occur. An 11-year-old male presented with cervical lymphadenopathy (3.5 × 3.5 cm). Fine needle aspiration shows atypical binucleate cell in a background of small lymphocytes, a diagnosis of Hodgkin disease is suggested. Excisional biopsy showed classical features of Hyaline vascular Castleman disease. Careful cytological evaluation and clinical correlation is required for definitive diagnosis.
... Castleman disease coexisting with cHL has been repeatedly reported in the literature, and some researchers consider it as a distinct entity [8]. Although Castleman disease and cHL were not diagnosed concomitantly in the presented case, we speculate that the presented case may have also been one of such cases. ...
Chemotherapy for hemodialysis (HD) patients is a challenging situation because HD patients are generally frail, and the pharmacokinetics and pharmacodynamics of most chemotherapeutics in HD patients are unknown. We report a classical Hodgkin lymphoma (cHL) patient successfully treated with 34 courses of brentuximab vedotin (BV) monotherapy, of which 30 courses were carried out during HD. Although grade 2 peripheral sensory neuropathy and one occasion of febrile neutropenia were observed, treatment was well-tolerated overall and effective. This is the first report of successful BV administration in a cHL patient on HD, and also the first to report efficacy and safety of extended courses of BV in an HD patient. Treatment options for cHL in the HD patient are limited, and extended courses of BV monotherapy may be an optimal treatment approach for some patients.
... CD can also be associated with several malignancies, including lymphoma [9,10]. However, the relationship between lymphoma and CD, specifically iMCD, is poorly understood. ...
Castleman disease (CD) is a rare lymphoproliferative disorder known to represent at least four distinct clinicopathologic subtypes. Large advancements in our clinical and histopathologic description of these diverse diseases have been made, resulting in subtyping based on number of enlarged lymph nodes (unicentric versus multicentric), according to viral infection by human herpes virus 8 (HHV-8) and human immunodeficiency virus (HIV), and with relation to clonal plasma cells (POEMS). In recent years, significant molecular and genetic abnormalities associated with CD have been described. However, we continue to lack a foundational understanding of the biological mechanisms driving this disease process. Here, we review all cases of CD with molecular abnormalities described in the literature to date, and correlate cytogenetic, molecular, and genetic abnormalities with disease subtypes and phenotypes. Our review notes complex karyotypes in subsets of cases, specific mutations in PDGFRB N666S in 10% of unicentric CD (UCD) and NCOA4 L261F in 23% of idiopathic multicentric CD (iMCD) cases. Genes affecting chromatin organization and abnormalities in methylation are seen more commonly in iMCD while abnormalities within the mitogen-activated protein kinase (MAPK) and interleukin signaling pathways are more frequent in UCD. Interestingly, there is a paucity of genetic studies evaluating HHV-8 positive multicentric CD (HHV-8+ MCD) and POEMS-associated CD. Our comprehensive review of genetic and molecular abnormalities in CD identifies subtype-specific and novel pathways which may allow for more targeted treatment options and unique biologic therapies.
Clinicians are sometimes confronted with the diagnostic difficulties of the idiopathic form of Castleman's Disease (iMCD). As this review reports with demonstrative clinical cases, iMCD can mimic various serious systemic pathologies such as certain autoimmune diseases, Still's disease, POEMS syndrome, and malignant lymphoproliferations, sharing a very similar histology and identical symptoms. To make a diagnosis of iMCD, the clinician must eliminate all the pathologies mentioned above, but he must first think of it and evoke this diagnosis of rare disease before the first symptoms but also know how to evoke this diagnosis again even after several years of evolution of a disease like those mentioned above whose evolution is not favorable. © 2022 Published by Elsevier Masson SAS on behalf of Société nationale française de médecine interne (SNFMI).
Résumé
Le terme de « maladie de Castleman » regroupe plusieurs entités très différentes sur le plan clinique, biologique, anatomopathologique et physiopathologique. Nous décrivons, dans cet article, les caractéristiques de la maladie de Castleman unicentrique, de la maladie de Castleman multicentrique associée à HHV8 et de la maladie de Castleman multicentrique idiopathique associée ou non au syndrome de TAFRO (« thrombocytopenia, anasarca, fever, reticulin myelofibrosis and/or renal insufficiency, organomegaly »). Nous détaillons également les diagnostics différentiels de ces différentes entités.
