scholarly journals ROSAI DORFMAN DISEASE MANAGEMENT, ACTIVE INTERVENTION OR WAIT AND WATCH: A CASE SERIES

2021 ◽  
Vol Volume 9 (upjohns/volume9/Issue2) ◽  
pp. 47-50
Author(s):  
N. Vishnu S. Reddy
Keyword(s):  
Disease Management ◽  
Treatment Strategies ◽  
Case Series ◽  
Spontaneous Resolution ◽  
Separate Entity ◽  
Submandibular Region ◽  
Severity Of Disease ◽  
Massive Lymphadenopathy ◽  
First Case ◽  
Rosai Dorfman Disease

ABSTRACT Rosai-Dorfman disease (RDD) is a rare histiocytic disorder commonly manifested as histiocytic proliferation of lymph nodes and extra nodal tissues initially described as a separate entity by Rosai and Dorfman under the term sinus histiocytosis with massive lymphadenopathy (SHML) in 1969. The treatment strategies can be variable and based on the severity of disease, v ital organ invol vement and pos s ible complications. The first case was a 47 years old female presenting with nasal obstruction and submandibular lymphadenopathy since few days. The second case was a 8 year old girl presented with stridor and lymphadenopathy in the submandibular region. In both the cases spontaneous resolution was expected and intervention was done only if there werecomplications. Patients were followed up every three months for two years and both the cases have resolved completely without any sequelae of the disease.Here we present two cases of Rosai-Dorfman disease presented to our hospital, this case series adds to the growing knowledge of Rosai-Dorfman di sease, along with under s tanding of pathophysiology, clinical diagnosis, treatment strategies and recovery options in cases where complications occurred.

scholarly journals Rosai-Dorfman disease of the subdural spine with a long segment lesion: A case report and literature review

2017 ◽  
Vol 45 (2) ◽  
pp. 875-881 ◽  
Author(s):  
Ji Tu ◽  
Wen-Tian Li ◽  
Cao Yang
Keyword(s):  
Cervical Lymphadenopathy ◽  
Good Prognosis ◽  
Massive Lymphadenopathy ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Puncture Biopsy ◽  
Appropriate Therapy ◽  
Systemic Manifestations ◽  
Cluster Of Differentiation

Rosai-Dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare benign disorder usually characterized by massive painless cervical lymphadenopathy and systemic manifestations. Extranodal involvement, especially spinal involvement, is extremely rare. We report a 41-year-old man who presented with only intermittent dorsodynia. His condition was diagnosed as non-specific inflammatory disease on the basis of preoperative puncture biopsy results. We performed total surgical resection. Histopathological findings showed distinctive emperipolesis and immunohistochemistry results were positive for cluster of differentiation CD68 and S100 and negative for CD1a. A good prognosis was confirmed at the 3-month follow-up visit. This is the first case of RDD of the subdural spine with such a long segment lesion. There is still no consensus regarding appropriate therapy for this type of RDD and the preoperative diagnosis remains challenging. The unusual presentation of our case serves as a reference when diagnosing and treating RDD.

scholarly journals Diagnosis and treatment of Rosai-Dorfman disease of the spine: a systematic literature review

2021 ◽  
Vol 10 (1) ◽  
Author(s):  
Pan-pan Hu ◽  
Feng Wei ◽  
Xiao-guang Liu ◽  
Zhong-jun Liu
Keyword(s):  
Case Reports ◽  
Treatment Strategies ◽  
Diagnosis And Treatment ◽  
High Recurrence Rate ◽  
Imaging Features ◽  
Total Resection ◽  
Adjuvant Therapies ◽  
Massive Lymphadenopathy ◽  
Asymptomatic Patients ◽  
Rosai Dorfman Disease

Abstract Purpose To review and summarize the clinical features, diagnosis, treatment strategies, and prognosis of spinal Rosai-Dorfman disease (RDD). Methods RDD is also termed as sinus histiocytosis with massive lymphadenopathy. We searched the databases of PubMed, Elsevier ScienceDirect, SpringerLink, and OVID. The keywords were Rosai-Dorfman disease and spine/central nervous system. Research articles and case reports with accessibility to full texts regarding spinal RDD were eligible for the inclusion. A total of 62 articles were included, and they contained 69 cases. We extracted the information of interest and analyzed them using SPSS statistics package. Results The average age was 33.1 ± 18.3 years. The ratio of males to females was 1.9/1. Overall, 63 cases presented with spine-related symptoms. A total of 27 cases (39.1%) had multi-organ lesions, and 12 cases had records of massive lymphadenopathy. Among 47 cases who first manifested spine-related symptoms, 93.6% were preoperatively misdiagnosed. The disease had a predilection for cervical spine (38.8%) and thoracic spine (40.3%). 62.9% of lesions were dura-based. Surgery remained the mainstream treatment option (78.8%), with or without adjuvant therapies. Total lesion resection was achieved in 34.8% of cases. The rate of lesion recurrence/progression was 19.5%, which was marginally lower for total resection than for non-total resection. Conclusion Spinal RDD has no pathognomonic clinical and imaging features. Most cases first present with spine-relevant symptoms. Massive lymphadenopathy is not common, but a tendency for multi-organ involvement should be considered. Spinal RDD has a high recurrence rate; thus, total resection is the treatment of choice. Adjuvant therapies are indicated for multi-organ lesions and residual lesions. A wait and watch strategy is recommended for asymptomatic patients. Herein, a workflow of diagnosis and treatment of the spinal RDD is established.

Extranodal Rosai-Dorfman Disease With Mucosal Involvement of the Stomach in a Background of Autoimmune Atrophic Gastritis

2018 ◽  
Vol 26 (7) ◽  
pp. 671-675 ◽  
Author(s):  
Amanda Meindl ◽  
M. Sambasiva Rao ◽  
Guang-Yu Yang
Keyword(s):  
Risk Factors ◽  
Atrophic Gastritis ◽  
Sigmoid Colon ◽  
Disease Process ◽  
Massive Lymphadenopathy ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Mucosal Involvement ◽  
Pathologic Analysis ◽  
Autoimmune Atrophic Gastritis

Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, has been described involving both lymph nodes and extranodal sites, but extranodal RDD rarely involves the gastrointestinal tract. Although the etiology is unclear, several risk factors have been shown to be highly associated with this disease process, including viral infection and immune alterations. In this article, we present a case of a 79-year-old male with a history of autoimmune atrophic gastritis and multiple carcinoid tumors of the stomach presenting with a new stomach mass. An additional large sigmoid colon mass and adjacent enlarged lymph node was identified through imaging, prior to surgery. Through extensive pathologic analysis, we identified the first case of predominant extranodal RDD involving gastric mucosa and submucosa in a background of atrophic gastritis, with additional involvement of the sigmoid colon. Based on this case and literature review, we further discuss possible risk factors and pathogenesis of this disease process.

scholarly journals Sodium-Glucose Cotransporter-2 Inhibitors in Patients with Hereditary Podocytopathies, Alport Syndrome, and FSGS: A Case Series to Better Plan a Large-Scale Study

Cells ◽  
2021 ◽  
Vol 10 (7) ◽  
pp. 1815
Author(s):  
Jan Boeckhaus ◽  
Oliver Gross
Keyword(s):  
Glomerular Filtration ◽  
Alport Syndrome ◽  
Large Scale ◽  
Case Series ◽  
Hereditary Diseases ◽  
Glomerular Filtration Barrier ◽  
Early Effect ◽  
Filtration Barrier ◽  
First Case ◽  
Sodium Glucose Cotransporter

Hereditary diseases of the glomerular filtration barrier are characterized by a more vulnerable glomerular basement membrane and dysfunctional podocytes. Recent clinical trials have demonstrated the nephroprotective effect of sodium-glucose cotransporter-2 inhibitors (SGLT2i) in chronic kidney disease (CKD). SGLT2-mediated afferent arteriole vasoconstriction is hypothesized to correct the hemodynamic overload of the glomerular filtration barrier in hereditary podocytopathies. To test this hypothesis, we report data in a case series of patients with Alport syndrome and focal segmental glomerulosclerosis (FSGS) with respect of the early effect of SGLT2i on the kidney function. Mean duration of treatment was 4.5 (±2.9) months. Mean serum creatinine before and after SGLT-2i initiation was 1.46 (±0.42) and 1.58 (±0.55) mg/dL, respectively, with a median estimated glomerular filtration rate of 64 (±27) before and 64 (±32) mL/min/1.73 m2 after initiation of SGLT2i. Mean urinary albumin-creatinine ratio in mg/g creatinine before SGLT-2i initiation was 1827 (±1560) and decreased by almost 40% to 1127 (±854) after SGLT2i initiation. To our knowledge, this is the first case series on the effect and safety of SGLT2i in patients with hereditary podocytopathies. Specific large-scale trials in podocytopathies are needed to confirm our findings in this population with a tremendous unmet medical need for more effective, early on, and safe nephroprotective therapies.

scholarly journals Clinical, Laboratory and Histological Features of Dipeptidyl Peptidase-4 Inhibitor Related Noninflammatory Bullous Pemphigoid

2021 ◽  
Vol 10 (9) ◽  
pp. 1916
Author(s):  
Ágnes Kinyó ◽  
Anita Hanyecz ◽  
Zsuzsanna Lengyel ◽  
Dalma Várszegi ◽  
Péter Oláh ◽  
...  
Keyword(s):  
Bullous Pemphigoid ◽  
Clinical Laboratory ◽  
Case Series ◽  
Dipeptidyl Peptidase ◽  
Area Index ◽  
Histological Features ◽  
Dipeptidyl Peptidase 4 ◽  
First Case ◽  
Dipeptidyl Peptidase 4 Inhibitor ◽  
The Mean

Bullous pemphigoid (BP) is an autoimmune blistering disease of elderly patients that has shown increasing incidence in the last decades. Higher prevalence of BP may be due to more frequent use of provoking agents, such as antidiabetic dipeptidyl peptidase-4 inhibitor (DPP4i) drugs. Our aim was to assess DPP4i-induced bullous pemphigoid among our BP patients and characterize the clinical, laboratory and histological features of this drug-induced disease form. In our patient cohort, out of 127 BP patients (79 females (62.2%), 48 males (37.7%)), 14 (9 females and 5 males) were treated with DPP4i at the time of BP diagnosis. The Bullous Pemphigoid Disease Area Index (BPDAI) urticaria/erythema score was significantly lower, and the BPDAI damage score was significantly higher in DPP4i-BP patients compared to the nonDPP4i group. Both the mean absolute eosinophil number and the mean periblister eosinophil number was significantly lower in DPP4i-BP patients than in nonDPP4i cases (317.7 ± 0.204 vs. 894.0 ± 1.171 cells/μL, p < 0.0001; 6.75 ± 1.72 vs. 19.09 ± 3.1, p = 0.0012, respectively). Our results provide further evidence that DPP4i-associated BP differs significantly from classical BP, and presents with less distributed skin symptoms, mild erythema, normal or slightly elevated peripheral eosinophil count, and lower titers of BP180 autoantibodies. To our knowledge, this is the first case series of DPP4i-related BP with a non-inflammatory phenotype in European patients.

scholarly journals Challenging Presentations of Seven Cases of Gastrointestinal Basidiobolomycosis in Sudan: Clinical Features, Histology, Imaging, and Recommendations

2020 ◽  
Vol 12 (04) ◽  
pp. 281-284
Author(s):  
Sawsan A. Mohammed ◽  
Azza A. Abdelsatir ◽  
Mohamed Abdellatif ◽  
Suliman Hussein Suliman ◽  
Omer Mohammed Ibrahim Elbasheer ◽  
...  
Keyword(s):  
Gastrointestinal Tract ◽  
Fungal Infection ◽  
Clinical Features ◽  
Subcutaneous Tissue ◽  
Case Series ◽  
First Case

AbstractsBasidiobolomycosis is a fungal infection caused by Basidiobolus ranarum which affects the skin and subcutaneous tissue and rarely the gastrointestinal tract. We report seven cases of gastrointestinal basidiobolomycosis with interesting clinical, radiological, and histological presentations. To our knowledge, this is the first case series of abdominal basidiobolomycosis to be reported from Sudan.

FIRST CASE SERIES OF VENOUS SINUS STENTING IN IIH PATIENTS IN WALES

2015 ◽  
Vol 86 (11) ◽  
pp. e4.113-e4
Author(s):  
Gauhar Abbas Malik ◽  
Yogish Joshi
Keyword(s):  
Intracranial Hypertension ◽  
Case Series ◽  
Headache Disorder ◽  
Venous Sinus ◽  
First Line ◽  
Venous Stenting ◽  
First Case ◽  
Sinus Stenosis

BackgroundIdiopathic Intracranial Hypertension (IIH), is defined by increased cerebral spinal fluid (CSF) pressure in the absence of other causes of intracranial hypertension. There has been recent interest in the role of intracranial venous sinus stenosis in IIH. The raised pressures in IIH are argued to worsen by the secondary appearance of the venous sinus stenosis.Objective5 patients have undergone endovascular pressure measurement in Wales and their clinical details including history, examination, initial management, neuroimaging pre- and post venous stenting, and follow-up (6–24 months) to provide the first case study of patients undergoing Venous sinus stenting in Wales.Methods5 patients with IIH refractory to first line treatments underwent venography and manometry and 4 patients underwent stenting of the venous sinuses after this procedure had shown a pressure gradient proximal to stenosis in the lateral sinuses.ResultsThree patients were rendered asymptomatic, two were improved including one patient unmasking a different headache disorder following treatment.ConclusionsStenting in venous stenosis provides a further treatment option to patients refractory to first line treatments with IIH. This case series highlights in selected cases treatment is promising with good outcomes.

scholarly journals Treatment of digital ulcers in systemic sclerosis: Case series study of thirteen patients and discussion on outcome

2017 ◽  
Vol 63 (5) ◽  
pp. 422-426 ◽  
Author(s):  
Yahia Abuowda ◽  
Raquel Sousa Almeida ◽  
Ana Alves Oliveira ◽  
Petra Pego ◽  
Cristina Santos ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Treatment Protocol ◽  
Treatment Strategies ◽  
Case Series ◽  
Tissue Loss ◽  
Repeated Treatment ◽  
Digital Ulcers ◽  
Case Series Study ◽  
Clinical Description ◽  
Soft Tissue Loss

Summary Introduction: In systemic sclerosis (SSc), digital ulcers (DU) are debilitating and recurrent. They are markers of prognosis and are associated with disability and mortality. Treatment strategies have been developed to block the proposed mechanisms of this complication. Objective: Clinical description of a population of SSc patients with DU, treatment, complications and outcome. Method: Analysis of 48 SSc patients meeting 2013 ACR-EULAR criteria, followed between 1999-2015; 13 patients had DU. Treatment protocol applied included cycles of 21 days of alprostadil, which can be repeated in the absence of DU healing. After DU healing, bosentan was initiated. Results: DU healing was achieved with intravenous prostanoid in 12 patients; seven patients required repeated treatment for DU healing. Twelve patients were later treated with bosentan; three of them experienced recurrence of DU, while one was anti-B2-GPI positive. Four patients had soft tissue loss and three other suffered digital amputation, these being late diagnosis. Conclusion: Younger patients and early referrals had better outcomes. Endothelin receptor antagonist toxicity should be monitored, particularly in patients previously exposed to hepatotoxic drugs.

Cutaneous Rosai-Dorfman Disease With Increased Number of Eosinophils: Coincidence or Histologic Variant?

2011 ◽  
Vol 135 (12) ◽  
pp. 1597-1600 ◽  
Author(s):  
John J Cangelosi ◽  
Victor G Prieto ◽  
Doina Ivan
Keyword(s):  
Differential Diagnosis ◽  
Pituitary Gland ◽  
Plasma Cells ◽  
S100 Protein ◽  
Clinical Context ◽  
Cutaneous Involvement ◽  
Eosinophilic Infiltrate ◽  
First Case ◽  
Rosai Dorfman Disease ◽  
Skin Biopsies

Rosai-Dorfman disease (RDD) is characterized histologically by a dense histiocytic infiltrate with emperipolesis and associated lymphocytes, plasma cells, and neutrophils. Eosinophils are not commonly associated. We report a patient with initial thymus and pituitary gland involvement by RDD, who later developed papules on the groin and axilla. Skin biopsies showed admixed histiocytic infiltrates (lymphocytes, neutrophils, and plasma cells) without emperipolesis. A prominent eosinophilic infiltrate was also observed, a feature not, to our knowledge, previously reported. Immunohistochemistry revealed positivity for CD68 (most cells) and S100 protein (scattered cells) and was negative for anti-CD1a. The diagnosis of RDD was established in the clinical context after comparison with the thymic and pituitary lesions (similar histologic features, albeit with fewer eosinophils, and immunohistochemical profiles). We present the first case, to our knowledge, of multicentric RDD with cutaneous involvement and associated prominent eosinophilic infiltrate. Thus, RDD should be included in the differential diagnosis of mononuclear infiltrates containing eosinophils.

Sign in / Sign up

Export Citation Format

Share Document