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At least 19 recordsLinked to original sources
PubMed2025

The Involvement of PI3K-Akt Signaling in the Clinical and Pathological Findings of Idiopathic Multicentric Castleman Disease-Thrombocytopenia, Anasarca, Fever, Reticulin Fibrosis, and Organomegaly and Not Otherwise Specified Subtypes.

Idiopathic multicentric Castleman disease is a rare lymphoproliferative disorder that is clinically classified into idiopathic plasmacytic lymphadenopathy (IPL); thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO); and not otherwise specified (NOS). Although each subtype shows varying degrees of hypervascularity, no statistical data on the degree of vascularization have been reported. Additionally, the mechanisms underlying vascularization in each clinical subtype are poorly understood. Here, we aimed to clarify these mechanisms by evaluating the histopathological characteristics of each clinical subtype across 37 patients and performing a whole-transcriptome analysis focusing on angiogenesis-related gene expression. Histologically, TAFRO and NOS exhibited a significantly higher degree of vascularization than IPL (IPL vs TAFRO, P < .001; IPL vs NOS, P = .002). In addition, the germinal centers (GCs) were significantly more atrophic in TAFRO than in IPL. In TAFRO and NOS, "whirlpool vessels" in GCs were seen in most cases (TAFRO, 9/9, 100%; NOS, 6/8, 75%) but not in IPL (IPL vs TAFRO, P < .001; IPL vs NOS, P = .007). Likewise, immunostaining for Ets-related gene revealed higher levels in endothelial cells of GCs in TAFRO than in IPL (P = .014), and TAFRO and NOS were associated with a significantly higher number of endothelial cells in interfollicular areas compared with that in IPL (TAFRO vs IPL, P < .001; NOS vs IPL, P = .002). Gene expression analysis revealed that the PI3K-Akt signaling pathway was significantly enriched in the TAFRO and NOS (TAFRO/NOS) groups. This pathway, which may be activated by vascular endothelial growth factor A and some integrins, is known to affect angiogenesis by increasing vascular permeability, which may explain the clinical manifestations of anasarca and/or fluid retention in TAFRO/NOS. These results suggest that the PI3K-Akt pathway plays an important role in the pathogenesis of TAFRO/NOS.

Humans↗
PubMed1992

[A case of Castleman's disease with a variable neuropsychiatric symptomatology].

A case of 44-year-old woman who had shown psychiatric symptoms before and during the course of Castlemans' disease was presented. For four years, she first suffered from a paranoid-hallucinatory state and then a depressive one episodically. In the course of the latter, severe anemia developed. She was diagnosed as Castleman's disease, because the increased serum level of gamma-globulin and interleukin-6 (IL-6), and multiple lymphomata were evidenced. A paranoid-hallucinatory state relapsed about one year later from this episode. At last, some bulbar and cerebellar symptoms, and a delirium suddenly occurred. The ischemic changes at the level of the pons and midbrain were revealed by the magnetic resonance imaging (MRI) examination. It is certainly that both neurological and psychiatric symptoms were related to the lesions. This ischemic lesions may have resulted from the anoxia secondary to the severe anemia and/or hyperviscosity syndrome in the disease. On the other hand, the increased serum level of IL-6 as well as the ischemic lesions might have caused psychiatric symptoms in this case, as the interferone which is one of the analogues of IL-6, is known to induce emotional and behavioral symptoms.

Adult↗
PubMed1978

[A case of pseudo-tumoral lymphoid hyperplasia or Castleman's disease].

The relatively rare pseudotumoral lymphoid hyperplasia (benigh lymphoid or reactive lymphoreticular hyperplasia; pseudolymphoma; Castleman's disease) arises from the lymph nodes, usually in the mediastinum. The disease assumes tow histologically and clinically different forms: the first, angiofollicular, is often symptomless, while the second, lymphoplasmocytic, is sometimes associated with a marked chronic inflammatory syndrome. The etiology is unknown, but is probably a reaction of the lymphoplasmocytic system to an antigen, possibly of viral origin. Diagnosis is based on histology. Surgical treatment is curative. A rare form of this unusual disease is reported with reference to the morphological differential diagnosis; the similarities with immunoblastic lymphadenopathy are emphasized.

Adult↗
PubMed1978

Giant mediastinal lymph node hyperplasia (Castleman's disease): angiographic and clinical features.

The angiographic and clinical features of two cases of mediastinal angiofollicular lymph node hyperplasia (Castleman's disease) are presented. The masses were angiographically and microscopically extremely hypervascular. Both patients had profuse bleeding at initial surgery. In one patient, angiographic embolization after an initial attempt at resection made complete excision possible. These lesions behave in a benign fashion, and complete surgical excision is curative.

Adult↗
PubMed1979

Castleman's disease. Angiofollicular benign lymph-node hyperplasia. Radiological features in the differential diagnosis of vascular tumours of the pelvis.

An unusual case of benign lymph-node hyperplasia (Castleman) in the pelvis is reported and its clinical, histological and radiographic characteristics discussed. The angiographic appearances in this situation have to our knowledge not been described before in the literature. The angiographic features are not diagnostic, being mimicked by a wide variety of vascular tumours in the pelvis. The benign nature of the condition is stressed. BLH should be considered in the differential diagnosis of vascular tumours in the pelvis.

Angiography↗
PubMed1992

Membranoproliferative glomerulonephritis associated with multicentric angiofollicular lymph node hyperplasia. Case report and review of the literature.

A 14-year-old boy presented with fever, anemia, hepatosplenomegaly, generalized lymphadenopathy and nephrotic syndrome. Lymph node biopsy showed angiofollicular lymph node hyperplasia (generalized Castleman's disease) of the plasma cell type. Kidney biopsy showed membranoproliferative glomerulonephritis type 1. Complete remission was achieved with corticosteroid treatment and repeat kidney biopsy 22 months later showed complete resolution of the renal pathology. The association between membranoproliferative glomerulonephritis and multicentric angiofollicular lymph node hyperplasia, plasma cell type, has not previously been reported.

Adolescent↗
PubMed1975

Angiofollicular lymph node hyperplasia of the lung simulating carcinoma: pseudolymphoma with inflammatory changes.

Angiofollicular lymph node hyperplasia of the lung developed in a 45-year-old man who was a heavy smoker. Slow growth of the lesion 3 years after biopsy and the finding of atypical cells in the sputum and bronchial washings strongly suggested a malignant lesion. There were no systemic manifestations other than dyspnea, which may have been related to allergy. The lesion was angiofollicular lymph node hyperplasia of the hyaline-vascular type. Invasion by lymphoid cells of both blood vessels and bronchioles suggested that the pathogenesis was probably infectious or inflammatory; also, the nature of the infiltrate indicated an immune mechanism. The course of the disease, as in other cases reported, has been benign, though a patchy density has developed in contralateral lung 4 years after lobectomy.

Castleman Disease↗
PubMed1992

Angiofollicular lymph node hyperplasia: a comparison of unicentric, multicentric, hyaline vascular, and plasma cell types of disease by morphometric and clinical analysis.

A morphometric and clinical analysis of 47 cases of angiofollicular lymph node hyperplasia (AFLH) demonstrated morphometric differences between plasma cell (28 cases) and hyaline vascular (19 cases) AFLH. Twenty-one AFLH cases were unicentric, and 26 cases were multicentric. Plasma cell types of AFLH, defined by standard morphologic and objective morphometric assessment, were associated with systemic clinical and laboratory abnormalities. Unicentric or hyaline vascular AFLH was amenable to surgical therapy, and multicentric or plasma cell AFLH without neuropathy was amenable to steroid treatment or chemotherapy with good prognoses. Multicentric or plasma cell AFLH with neuropathy was associated with resistance to steroids and chemotherapy and with a poor prognosis.

Blood Vessels↗
PubMed2025

KSHVbook: An Information-Sharing Database for Kaposi's Sarcoma-Associated Herpesvirus.

Kaposi's sarcoma-associated herpesvirus (KSHV) is a double-stranded DNA virus belonging to the &#x3b3;-herpesvirus subfamily. KSHV is the causative agent of Kaposi's sarcoma (KS), primary effusion lymphoma (PEL), multicentric Castleman's disease (MCD), and KSHV inflammatory cytokine syndrome (KICS). Since its discovery, research on KSHV has rapidly progressed, but existing information platforms relatively lack comprehensiveness and do not provide efficient analysis tools tailored for KSHV. To further promote the research on KSHV more effectively, we have developed KSHVbook (http://www.kshvbook.com), a specialized information-sharing database dedicated to KSHV. This platform offers extensive information on genes, coding sequences, proteins, and the gene regulatory region. Besides, the KSHVbook includes about 35&#x2009;010 transcription factor binding sites (TFBSs), 342&#x2009;010 pairs of KSHV miRNA-host target gene relationships, protein structures predicted by AlphaFold3, qPCR primers, and so on. We also develop analytical tools for viral genome regions, TFBSs, and KSHV miRNA target genes to discover previously unknown biological functions of KSHV. These analytical tools can effectively identify the potential regulatory relationships between host transcription factors and viral genes. Overall, this platform provides a centralized data resource for KSHV research by integrating multiple databases, offering accessible analysis tools, and simplifying data acquisition. The KSHVbook will continue to be updated, and more features can be found on the website.

Herpesvirus 8, Human↗
PubMedDate not supplied

Retroperitoneal Castleman's tumors of hyaline vascular type: imaging study. Case report.

We report two retroperitoneal Castleman's tumors (giant lymph node hyperplasia) of hyaline vascular type. One was a large tumor situated in the pararenal region, the other was located in the pancreas head. On computed tomography (CT), both tumors were solid and moderately enhanced after administration of contrast material. On angiography, both tumors were hypervascular with dilated feeding arteries, and showed capillary blush. In an appropriate clinical setting, a retroperitoneal tumor showing these findings should include Castleman's tumor in the differential diagnosis.

Capillaries↗
PubMed1992

[Castleman tumor].

A case of a mediastinal benign tumor in a 56 year old male is presented. The tumor was detected during a routine chest radiographic examination. The patient was asymptomatic. During thoracotomy two tumors were found. One was localized in the middle mediastinum the other in the posterior mediastinum. Both tumors were removed completely. Basing on the results of the clinical examination, of the laboratory data and the histopathological examination of the resected tumors a diagnosis of Castleman's tumor of the plasma-cell type was made.

Castleman Disease↗
PubMed1992

[Peripheral neuropathy associated with Castleman's lymphoma].

Castleman's lymphoma is a rare clinicopathological entity and is often difficult to classify nosographically. From the histopathological point of view two variants are recognized, one hyalino-vascular and one plasma cellular. Moreover Castleman's lymphoma may be multifocal or unifocal (located within the thorax in 70% of patients). The plasma cellular variant is often associated with systemic symptoms such as fever, sweating, organomegaly, polyneuropathy, and mono- or polyclonal gammopathy. We describe a rare case of unifocal, plasma cellular-type Castleman's lymphoma located in the mediastinum, asymptomatic at onset but later dominated by neurological symptoms (chronic isolated polyneuropathy whose clinical expression was predominantly motor). A brief review is presented of the main clinico-histological characteristics of Castleman's lymphoma together with some hypotheses about the pathogenesis of the associated neuropathy.

Adult↗