A fatal case of Rosai–Dorfman disease

Bulyashki, Daniel; Brady, Zarina; Arif, Shahswar; Tsvetkov, Nikolay; R, Radev

doi:10.1002/ccr3.1036

Cited by 6 publications

(5 citation statements)

References 2 publications

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“…Specifically, the erythrocyte sedimentation rate (ESR) is elevated in nearly 90% of RDD cases and polyclonal hypergammaglobulinemia is present in approximately 80–90% of cases according to several studies [ 1 , 4 – 8 ]. Other less common laboratory findings associated with RDD include elevated C-reactive protein (CRP), anemia, neutrophilia, leukocytosis, thrombocytopenia, arthropathy, arthritis, and elevated rheumatoid factor [ 1 , 4 , 5 , 7 , 8 , 10 , 14 , 16 ]. The types of anemias are many and may include normocytic or microcytic varieties [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

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Rosai-Dorfman Disease: Self-Resolving Unilateral Lymphadenopathy and a Brief Review of Literature

Feriante

Lee

2018

Case Reports in Oncological Medicine

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Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a disease of histiocytic proliferation with no known pathogenesis. This disease is defined by histological and cytological characteristics, including emperipolesis and positive S100 and CD68 markers. Although the disease typically presents clinically with massive bilateral lymphadenopathy due to sinus expansion from excessive histiocytosis, only unilateral lymphadenopathy was observed in this patient. The case involves a 40-year-old Caucasian male from the Netherlands presenting with unilateral lymphadenopathy suspicious for malignancy. Subsequent histological and laboratory testing led to the rare diagnosis of RDD. The lymphadenopathy resolved spontaneously over the course of several weeks following the initial presentation. Rosai-Dorfman disease reportedly has a benign prognosis with approximately 20% of patients experiencing spontaneous disease resolution (as was the case for this patient) with 70% experiencing chronic symptoms that may last years but not require intervention. We therefore advocate observation as a mainstay of treatment for most cases of this rare disease with intervention only being pursued in symptomatic cases. A review of recent literature regarding pathogenesis, epidemiology, diagnostic factors, prognosis, and treatment is provided and discussed.

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Section: Discussionmentioning

confidence: 99%

“…Moreover, spontaneous resolution appears to favor cases with nodal involvement only [ 5 ]. Mortality from diagnosed RDD or SHML is associated with an extra nodal development of disease in vital organs such as the CNS and kidneys or from complications associated with immunosuppression or amyloidosis [ 2 , 16 ].…”

Section: Discussionmentioning

confidence: 99%

Rosai-Dorfman Disease: Self-Resolving Unilateral Lymphadenopathy and a Brief Review of Literature

Feriante

Lee

2018

Case Reports in Oncological Medicine

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“…13 It is often unnecessary to intervene until and unless airways are obstructed or some major organ is compressed. 14 Due to its rare incidence, no standard treatment has yet been defined for RDD. However, the condition is mostly selflimiting.…”

Section: Discussionmentioning

confidence: 99%

Rosai Dorfman disease: a case report with nodal involvement

Silky

Singh

Kumar

et al. 2022

Int J Otorhinolaryngol Head Neck Surg

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<p class="abstract">Rosai Dorfman disease is characterised by sinus histiocytosis with massive lymphadenopathy. Here, we present a case report of 33-year female with low grade fever and multiple swellings of variable size in cervical region. Laboratory examinations showed erythrocyte sedimentation rate to be increased up to 40 mm / hour. Histopathological examination revealed a number of histiocytes containing lymphocytes and few plasma cells in their cytoplasm showing emperipolesis of plasma cells and lymphocytes. Immunohistochemistry showed positivity for CD68 with marked sinus expansion by histiocytic cells and emperipolesis. Treatment with tapering dose of systemic corticosteroids over 1 month gave excellent results.</p>

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“…The literature review indicates 20% of RDD patients can be self-healing, 70% are in stable condition, only 10% arise have aggressive local lesions or systemic disease [ 21 ]. RDD could be fatal for a very small percentage of patients if the giant enlarged lymph nodes compress vital organs, with the combination of mass formation and cellular infiltration [ 22 , 23 ]. For symptomatic RDD patients or those with involvement of critical organs, the treatments including surgery should be given [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

An extremely rare case of Rosai-Dorfman disease in the spleen

et al. 2021

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Background Rosai-Dorfman disease (RDD) is a rare, multisystemic histiocytic disorder, and commonly manifesting as lymphadenopathy in the young male. Abdominal manifestations of RDD are extremely rare. Case presentation In August 2018, a 42-year-old man underwent an abdominal ultrasonography examination due to his weight loss of 10 kg in only three months and found a giant solid tumor was found in his spleen. Then, he was admitted to our hospital and diagnosed as a splenic mass via abdominal enhanced CT and MRI. Laparoscopic splenectomy was administrated within six days of admission due to the clear surgical indications. The pathogenesis of RDD remained poorly understood and the disease should be diagnosed based on histopathology and immunohistochemistry (IHC). The mutations in ATM and NFKBIA were observed using next generation sequencing (NGS). Conclusion We reported a case of splenic involvement of RDD with NGS genetic testing, indicating the difficulty of making a diagnosis before surgery. This extremely rare case offers new references for the understanding of abdominal viscera RDD.

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A fatal case of Rosai–Dorfman disease

Cited by 6 publications

References 2 publications

Rosai-Dorfman Disease: Self-Resolving Unilateral Lymphadenopathy and a Brief Review of Literature

Rosai-Dorfman Disease: Self-Resolving Unilateral Lymphadenopathy and a Brief Review of Literature

Rosai Dorfman disease: a case report with nodal involvement

An extremely rare case of Rosai-Dorfman disease in the spleen

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