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Journal of Crohn's & Colitis Apr 2024Ozanimod regulates lymphocyte egress from the spleen and lymph nodes into the systemic circulation. The histological changes which occur in the lymph nodes of patients...
INTRODUCTION
Ozanimod regulates lymphocyte egress from the spleen and lymph nodes into the systemic circulation. The histological changes which occur in the lymph nodes of patients on ozanimod is unknown.
MATERIALS AND METHODS
This retrospective study included patients with ulcerative colitis [UC] undergoing total colectomy for treatment-refractory disease who received ozanimod, and a cohort of patients with UC undergoing colectomy who did not have ozanimod exposure. Histology of the lymph nodes from the mesentery of colectomy specimens was reviewed and multiple features were scored by experienced pathologists.
RESULTS
Six [13%] ozanimod-treated patients with UC required surgery for treatment-refractory disease. Colectomy specimen data were available for five patients [one patient had surgery at an outside centre]. Lymph node specimens from six control patients with UC who had colectomy were examined. Histological examination of lymph nodes showed that patients treated with ozanimod had significantly greater extent of dilated sinuses [p = 0.03] and greater degrees of sinus histiocytosis [p = 0.03] compared with control patients. In addition, there was a trend towards more Castleman-like angiotrophic hyperplasia, plasma cell infiltration, and subcortical interfollicular expansion in ozanimod-treated patients.
CONCLUSION
This study identifies unique histological changes in the lymph nodes of patients with UC treated with ozanimod. The presence of sinus histiocytosis and dilated sinuses is in keeping with the known mechanism of action of ozanimod, and suggests that blocking lymphocyte egression from lymph nodes was insufficient to ameliorate disease severity in these patients. The possibility of Castleman-like features, identified in several of the cases, needs to be further investigated.
Topics: Humans; Colitis, Ulcerative; Colectomy; Male; Retrospective Studies; Female; Middle Aged; Lymph Nodes; Adult; Oxadiazoles; Aged; Sphingosine 1 Phosphate Receptor Modulators; Case-Control Studies; Indans
PubMed: 37879626
DOI: 10.1093/ecco-jcc/jjad174 -
Photodiagnosis and Photodynamic Therapy Dec 2023Rosai-Dorfman disease(RDD) is a rare benign histiocytic proliferative disorder. Cutaneous Rosai-Dorfman disease(CRDD)is a rare variant of RDD with lesions localized to...
Rosai-Dorfman disease(RDD) is a rare benign histiocytic proliferative disorder. Cutaneous Rosai-Dorfman disease(CRDD)is a rare variant of RDD with lesions localized to the skin. We report a case of patients with CRDD who was refractory to methotrexate, thalidomide, cyclosporine, glucocorticoid for local injection and external use, but resolved with fractional laser combined with 5-aminolevulinic acid photodynamic therapy (ALA-PDT). We believe that fractional laser technology combined with ALA-PDT for CRDD is a minimally invasive, effective and satisfactory treatment.
Topics: Humans; Histiocytosis, Sinus; Aminolevulinic Acid; Photochemotherapy; Photosensitizing Agents; Skin Diseases; Lasers
PubMed: 37858909
DOI: 10.1016/j.pdpdt.2023.103852 -
Journal of Cardiothoracic Surgery Oct 2023Rosai-Dorfman disease (RDD) is currently considered a group of neoplastic diseases of unknown etiology, with monoclonal proliferation of histiocytes, showing unique...
Rosai-Dorfman disease (RDD) is currently considered a group of neoplastic diseases of unknown etiology, with monoclonal proliferation of histiocytes, showing unique histopathologic features and varying clinical presentation. Primary thymic RDD is an extremely rare extranodal form of this disorder. In this study, we describe the case of an otherwise healthy 64-year-old Chinese man who presented with an isolated, asymptomatic soft tissue density lesion in the anterior mediastinum detected by computed tomography. Histology of the surgical specimen revealed infiltration of thymic tissue by sheets of large histiocytes with mixed lymphocytes and plasma cells, and background fibrosis. Immunohistochemical staining of the histiocytes was positive for S100, CD68, CD163, OCT2 and cyclin D1, but negative for CD1a and BrafV600E expression, thus supporting a diagnosis of RDD. Primary thymic RDD is extremely rare and may be a diagnostic challenge when presenting as mediastinal lesion.
Topics: Male; Humans; Middle Aged; Histiocytosis, Sinus; Histiocytes; Thymus Gland; Lymphocytes; Mediastinum; Musculoskeletal Diseases
PubMed: 37817188
DOI: 10.1186/s13019-023-02381-4 -
Skin Health and Disease Oct 2023Sinus histiocytosis or Rosai-Dorfman disease (RDD) is a rare disorder with severe lymphadenopathy and a limited clinical course, the aetiology of which is still...
Sinus histiocytosis or Rosai-Dorfman disease (RDD) is a rare disorder with severe lymphadenopathy and a limited clinical course, the aetiology of which is still controversial. The disease usually affects cervical nodes, with fever, polyclonal gammopathy, and leucocytosis with neutrophilia. Pure cutaneous involvement occurs as the only manifestation in only 3% of cases. Cutaneous RDD is often associated with infections, immunodeficiency, and autoimmune disorders. A 52-year-old patient presented with disseminated, recurrent, and relapsed pure cutaneous RDD that responded well to treatment with sirolimus and local infiltrations of methylprednisolone. The patient had multiple nonpainful nodular and tumour-like lesions, histiocytic infiltrates with emperipolesis were observed on histologic examination, and positive immunohistochemistry for CD68, and S100. There is no standardised treatment, then the patient was treated with various therapies, including systemic steroids, chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone), radiotherapy, and other immunosuppressive treatments. Some lesions were treated surgically, resulting in recurrence. Sirolimus and local infiltration with methylprednisolone were tried as salvage treatments, the patient responded well, reducing the incidence of new lesions during follow-up, and the size of the preexisting lesions.
PubMed: 37799357
DOI: 10.1002/ski2.273 -
The Pan African Medical Journal 2023Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by the accumulation of activated histiocytes within affected tissues. The... (Meta-Analysis)
Meta-Analysis
A meta-analysis of cases of Rosai Dorfman disease reported on the African continent and a description of two cases from a tertiary academic hospital in Johannesburg, South Africa.
Rosai-Dorfman-Destombes disease (RDD) is a rare non-Langerhans cell histiocytosis characterized by the accumulation of activated histiocytes within affected tissues. The original haematopathological description of RDD has links to the late South African born haematopathologist, Ronald Dorfman, with a descriptive account of two cases of the disease treated at Chris Hani Baragwanath Academic Hospital are described herein. Alongside the two case descriptions is a meta-analysis of 149 published cases from the African continent. Sequential literature searches were performed on Google Scholar and PubMed with the search terms "sinus histiocytosis with massive lymphadenopathy", "Rosai-Dorfman disease", "Rosai-Dorfman Destombes" and "lymphadenopathy" together with the name of each individual country on the African continent, from Algeria to Zimbabwe. All possible cases of RDD reported in published literature from Africa were captured on a Microsoft Excel spreadsheet recording details, where available, of demographics, nodal (nodal groups) or extra-nodal disease as well as treatment. Of the 54 African countries on the continent, published data was available from half of these countries (n=27). Nigeria (35), Tunisia (25) and South Africa (23) contributed the majority of cases for data collection with a clear paucity of reportable information available from Central Africa. Of the 149 cases from the African continent, the majority were from patients aged ten years and younger with a decrease in reported cases in patients with increasing age. The mean age at diagnosis was 25.66 years [95% CI: 21.81-29.51] with a median age of diagnosis of 24.5 years. The youngest patient in the series was 3 months old and the oldest patient aged 72 (range 71.75 years, IQR 31). The cases reported were fairly split between males and females with a male-to-female ratio of 1.07: 1. HIV seropositivity was reported in seven patients (4.8%) and no HIV results were available in 104 patients (71.2%). Disease presentation was split between nodal disease in 43% of patients (n=64), Extra nodal (EN) disease in 32.9% (n=32), mixed (nodal/EN) disease in 11.4% (n=17) and unknown in 12.8% (n=19). Fever was present in 18.1% (n=27) of cases. Hepatic enlargement was noted in nine patients (6%) and splenic enlargement in four patients (2.7%). Commonly ascribed sites of EN disease, in descending order, were skin and soft tissue, ocular, ear/nose/throat (ENT), abdominal organ(s), bone, lung/pleura, brain parenchyma (including dura), endocrine glands, spine, breast, pericardium, pseudotumour formation (unspecified site), joint(s), peripheral nerves and genitourinary tract disease. The upfront administration of glucocorticosteroids was seen in the majority of cases. Rosai-Dorfman-Destombes, although a rare disorder, should be considered as a differential diagnosis in patients with massive bilateral cervical lymphadenopathy and is confirmed with accompanying pathological changes on microscopic and immunohistochemical examination of biopsy specimens. The role of infection, particularly HIV infection, is considered to be a possible contributor to the pathogenesis of RDD and HIV testing in patients from areas of high HIV endemicity with co-existing RDD should be undertaken. Consideration for mycobacterium tuberculosis infection in patients with generalized significant lymphadenopathy still remains an important differential for massive lymphadenopathy and requires confirmation by appropriate microbiological investigations. The treatment landscape in RDD is limited in many resource-poor settings, with the upfront use of glucocorticosteroids employed routinely in the majority of cases.
Topics: Humans; Male; Female; Young Adult; Adult; Infant; Histiocytosis, Sinus; South Africa; HIV Infections; Lymphadenopathy; Bone and Bones; Hospitals
PubMed: 37790150
DOI: 10.11604/pamj.2023.45.130.40709 -
Gastric Cancer : Official Journal of... Nov 2023The status of regional tumour draining lymph nodes (LN) is crucial for prognostic evaluation in gastric cancer (GaC) patients. Changes in lymph node microarchitecture,... (Meta-Analysis)
Meta-Analysis Review
The clinical importance of the host anti-tumour reaction patterns in regional tumour draining lymph nodes in patients with locally advanced resectable gastric cancer: a systematic review and meta-analysis.
BACKGROUND
The status of regional tumour draining lymph nodes (LN) is crucial for prognostic evaluation in gastric cancer (GaC) patients. Changes in lymph node microarchitecture, such as follicular hyperplasia (FH), sinus histiocytosis (SH), or paracortical hyperplasia (PH), may be triggered by the anti-tumour immune response. However, the prognostic value of these changes in GaC patients is unclear.
METHODS
A systematic search in multiple databases was conducted to identify studies on the prognostic value of microarchitecture changes in regional tumour-negative and tumour-positive LNs measured on histopathological slides. Since the number of GaC publications was very limited, the search was subsequently expanded to include junctional and oesophageal cancer (OeC).
RESULTS
A total of 28 articles (17 gastric cancer, 11 oesophageal cancer) met the inclusion criteria, analyzing 26,503 lymph nodes from 3711 GaC and 1912 OeC patients. The studies described eight different types of lymph node microarchitecture changes, categorized into three patterns: hyperplasia (SH, FH, PH), cell-specific infiltration (dendritic cells, T cells, neutrophils, macrophages), and differential gene expression. Meta-analysis of five GaC studies showed a positive association between SH in tumour-negative lymph nodes and better 5-year overall survival. Pooled risk ratios for all LNs showed increased 5-year overall survival for the presence of SH and PH.
CONCLUSIONS
This systematic review suggests that sinus histiocytosis and paracortical hyperplasia in regional tumour-negative lymph nodes may provide additional prognostic information for gastric and oesophageal cancer patients. Further studies are needed to better understand the lymph node reaction patterns and explore their impact of chemotherapy treatment and immunotherapy efficacy.
Topics: Humans; Stomach Neoplasms; Hyperplasia; Histiocytosis, Sinus; Clinical Relevance; Lymph Nodes; Prognosis; Esophageal Neoplasms; Neoplasm Staging
PubMed: 37776394
DOI: 10.1007/s10120-023-01426-w -
Magyar Sebeszet Sep 2023Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy is a rare, benign non-Langerhans cell histiocytosis, that typically involves the...
CASE-REPORT
Rosai-Dorfman disease, also known as sinus histiocytosis with massive lymphadenopathy is a rare, benign non-Langerhans cell histiocytosis, that typically involves the lymph nodes, but may also involve extranodal sites. We present a 58- years- old female patient who complained of a palpable mass in her left breast surrounded by 15-20 livid cutaneous lesions, resembling malignant breast cancer with cutaneous metastasis. Despite of core biopsy of the tumor and excisional biopsy one of the lesions, correct diagnosis of RDD was achieved only by complete pathological examination of the whole lesion after surgical excision.
CONCLUSION
Rosai-Dorfman disease confined to the breast is extremely rare, that clinically may mimic breast cancer.
Topics: Humans; Female; Middle Aged; Histiocytosis, Sinus; Breast Neoplasms; Biopsy; Biopsy, Large-Core Needle; Breast
PubMed: 37747834
DOI: 10.1556/1046.2023.30003 -
Ear, Nose, & Throat Journal Sep 2023Rosai-Dorfman disease (RDD) is characterized by sinus histiocytosis with massive lymphadenopathy, and the tissue exhibits positive results for S100 and CD68. This lesion...
Rosai-Dorfman disease (RDD) is characterized by sinus histiocytosis with massive lymphadenopathy, and the tissue exhibits positive results for S100 and CD68. This lesion typically affects the lymph nodes and rarely involves the nasal soft tissues. This report aims to present a case of RDD involving the nasal soft tissues, presenting with nasal congestion and rhinorrhea as the primary complaints. The patient underwent surgery navigated by nasal endoscope did not report recurrence after operation.
PubMed: 37740427
DOI: 10.1177/01455613231195160 -
Leukemia Nov 2023
Topics: Humans; Histiocytosis, Sinus; Diagnosis, Differential
PubMed: 37731024
DOI: 10.1038/s41375-023-02032-6 -
Journal of Neurology Jan 2024
Liquid biopsy of cerebrospinal fluid enabling the detection and therapeutic targeting of the BRAF mutation in a patient with overlapping Erdheim-Chester/Rosai-Dorfman disease.
Topics: Humans; Erdheim-Chester Disease; Histiocytosis, Sinus; Liquid Biopsy; Mutation; Proto-Oncogene Proteins B-raf
PubMed: 37725109
DOI: 10.1007/s00415-023-11983-w