Rituximab for refractory subcutaneous Sweet's syndrome in chronic lymphocytic leukemia: A case report

Hashemi, Seyed Mehdi; Fazeli, Seyed Amirhossein; Vahedi, Abdolbaset; Golabchifard, Reza

doi:10.3892/mco.2015.715

Cited by 8 publications

(3 citation statements)

References 22 publications

(24 reference statements)

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“…Significant levels of CD56, a Natural killer cell marker, CD40/CD40 ligand, and IFN-γ may indicate the role of antigen presenting cells, as well as a cross-link between the robust innate and adaptive immune response in SS ( 570 ). Further evidence of adaptive immunity involvement is suggested by SS remission following treatment with therapies targeting adaptive cell processes including corticosteroids, cyclosporine, IVIG, rituximab, and vedolizumab ( 121 , 132 , 571 – 576 ). Table 4 summarizes cytokines and inflammatory markers documented in SS.…”

Section: Pathogenesismentioning

confidence: 99%

Insights Into the Pathogenesis of Sweet's Syndrome

2019

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Sweet's syndrome, also known as Acute Febrile Neutrophilic Dermatosis, is a rare inflammatory condition. It is considered to be the prototype disease of neutrophilic dermatoses, and presents with acute onset dermal neutrophilic lesions, leukocytosis, and pyrexia. Several variants have been described both clinically and histopathologically. Classifications include classic Sweet's syndrome, malignancy associated, and drug induced. The cellular and molecular mechanisms involved in Sweet's syndrome have been difficult to elucidate due to the large variety of conditions leading to a common clinical presentation. The exact pathogenesis of Sweet's syndrome is unclear; however, new discoveries have shed light on the role of inflammatory signaling, disease induction, and relationship with malignancy. These findings include an improved understanding of inflammasome activation, malignant transformation into dermal infiltrating neutrophils, and genetic contributions. Continued investigations into effective treatments and targeted therapy will benefit patients and improve our molecular understanding of inflammatory diseases, including Sweet's syndrome.

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Section: Pathogenesismentioning

confidence: 99%

Insights Into the Pathogenesis of Sweet's Syndrome

2019

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“…Our report identifies obinutuzumab as a novel drug capable of causing a Sweet-like eruption. This stands in contrast to rituximab, which actually has been used to treat refractory Sweet syndrome . Thus, dermatologists should be aware of this new agent and its potential for neutrophilic inflammation, allowing for more timely recognition, diagnosis, and treatment.…”

Section: Discussionmentioning

confidence: 99%

Sweet-Like Eruption Associated With Obinutuzumab Therapy for Chronic Lymphocytic Leukemia

et al. 2017

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ously described. 3 Genomic DNA was extracted from peripheral blood, and exome sequencing was performed using the Ion Torrent system (BGI).Flow cytometric analysis of lesional skin revealed markedly high production of IL-17A from CD4+ T cells. Compared with age-, site-and sex-matched skin from a healthy donor, our patient had approximately 25-fold more IL-17Aproducing CD4+ T cells, with minimal differences in tumor necrosis factor, interferon γ, and IL-13. This information prompted treatment with the anti-IL-17 monoclonal antibody secukinumab at a dose of 150 mg subcutaneously. Rapid and robust clinical and laboratory response was then observed, with defervescence within 12 hours, cessation of new pustule formation within 24 hours, and discharge after 48 hours. His skin was nearly clear at follow-up 3 days after discharge (Figure , B). He continued treatment with subcutaneous secukinumab, 150 mg, at weeks 1, 2, 3, and 4 and then every 4 weeks thereafter as well as methotrexate, 5 mg/wk. At 1-year follow-up, he remained recurrence-free.Genetic analysis revealed a homozygous mutation within the IL36RN gene at position c.115+6T>C. This mutation has been shown to lead to a splicing defect resulting in exon skipping and a premature stop codon, leading to a truncated IL36Ra protein. 4 A heterozygous c.115+6T>C mutation in the patient's mother was confirmed by Sanger sequencing; DNA from the patient's brother, who had plaque psoriasis, and his unaffected father was not available for analysis.Discussion | DITRA is an autosomal recessive auto-inflammatory syndrome caused by mutations in the IL36RN gene. 1 Though the IL-1 receptor antagonist anakinra has been reported effective for patients with IL36RN mutations, it is not yet clear that IL-1 is active downstream of IL-36, possibly explaining lack of efficacy in our patient and others. 5 Inhibition of IL-17 resulted in rapid clinical response and sustained remission. To date, use of anti-IL17 agents has not been reported in pediatric patients with GPP, deficiency of the interleukin-1 receptor antagonist, or DITRA. Carrier et al 6 demonstrated that elevated IL-36 levels in psoriatic lesions correlated with increased levels of IL-17, but the relationship between IL-36 and T helper 17 cell (Th17) differentiation remains largely unknown. Our results suggest a pathomechanistic link between defective IL36RN and Th17 differentiation in the skin of patients with DITRA, and they identify IL-17 as a potential therapeutic target that warrants further investigation.

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“…Refractory cases usually require high doses of methylprednisolone (250 mg/24 h for a period of 3-5 days). Rituximab and anakinra are also postulated as favorable alternatives [2,[14][15][16][17].…”

Section: Systemic Treatmentmentioning

confidence: 99%

Treatment Strategies in Neutrophilic Dermatoses: A Comprehensive Review

Starita-Fajardo,

Lucena-López,

Ballester-Martínez

et al. 2023

IJMS

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Neutrophilic dermatoses (NDs) are a group of noninfectious disorders characterized by the presence of a sterile neutrophilic infiltrate without vasculitis histopathology. Their physiopathology is not fully understood. The association between neutrophilic dermatoses and autoinflammatory diseases has led some authors to propose that both are part of the same spectrum of diseases. The classification of NDs depends on clinical and histopathological features. This review focuses on the recent developments of treatments in these pathologies.

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Rituximab for refractory subcutaneous Sweet's syndrome in chronic lymphocytic leukemia: A case report

Cited by 8 publications

References 22 publications

Insights Into the Pathogenesis of Sweet's Syndrome

Insights Into the Pathogenesis of Sweet's Syndrome

Sweet-Like Eruption Associated With Obinutuzumab Therapy for Chronic Lymphocytic Leukemia

Treatment Strategies in Neutrophilic Dermatoses: A Comprehensive Review

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