Rituximab Treatment in a Child with Rosai-Dorfman Disease and Systemic Lupus Erythematosus

Alqanatish, Jubran; Houghton, Kristin; Bond, Mason; Senger, Christof; Tucker, Lori B.

doi:10.3899/jrheum.091275

Cited by 37 publications

(27 citation statements)

References 5 publications

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“…Review of the 400 patient SHML registry found that none of the 13% of patients with associated autoimmune phenomena achieved a complete remission and patients with autoimmunity represented more than half the registry's mortalities . Other reports have confirmed the poor outcome of these patients with SHML …”

Section: Introductionsupporting

confidence: 58%

Successful Treatment of Recurrent Autoimmune Cytopenias in the Context of Sinus Histiocytosis With Massive Lymphadenopathy Using Sirolimus

Cooper

Arceci

Gamper

et al. 2015

Pediatric Blood & Cancer

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Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai-Dorfman disease (RDD), is a non-neoplastic, lymphoproliferative disorder that usually resolves spontaneously or with minimal conventional chemotherapy. Rarely, SHML can be associated with autoimmune findings. Such cases are often treatment resistant and have high rates of morbidity and mortality. We present a case of a patient with long-standing autoimmunity in the context of SHML, dependent on standard-treatment until he was transitioned to novel monotherapy with sirolimus. Sirolimus treatment resulted in a complete remission, now sustained after discontinuation of all treatments for over 23 months, with no observable long-term sequelae.

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Section: Introductionsupporting

confidence: 58%

Successful Treatment of Recurrent Autoimmune Cytopenias in the Context of Sinus Histiocytosis With Massive Lymphadenopathy Using Sirolimus

Cooper

Arceci

Gamper

et al. 2015

Pediatric Blood & Cancer

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“…When this approach is not possible, according to the literature, roentgen therapy, chemotherapy, steroid therapy [11, 28], rituximab [29], interferon-alfa-2a [30], and immunosuppressive agents, such as azathioprine, methotrexate, and mercaptopurine [31–33], can be used.…”

Section: Discussionmentioning

confidence: 99%

“…Alqanatish et al described a case of a 7-year-old child with concomitant LES and RDD, who was treated with rutiximab (500 mg/m 2 /dose), with complete remission of the massive lymphadenopathy after 7 weeks of treatment [29]. Also high dose of interferon-alfa-2a, in particular, the pegylated form, has shown dramatic efficacy in cases of systemic RDD [30].…”

Section: Discussionmentioning

confidence: 99%

Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease

Arnao

Riolo

Savettieri

et al. 2016

Case Reports in Neurological Medicine

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Background. Rosai-Dorfmann disease (RDD) is a rare, idiopathic non-Langerhans cell histiocytosis, affecting children and young adults, that commonly presents as painless, massive cervical lymphadenopathy with fever, weight loss, and polyclonal hypergammaglobulinemia. Cervical lymphadenopathy and extranodal involvement are the main presentations. On the contrary, ophthalmic involvement and localisation in the central nervous system are rare. Case Report. An old man was admitted to our hospital for first seizure. Brain imaging studies revealed on the left an extra-axial thickening of the dura mater with enhancement and perilesional oedema, infiltrating the sphenoorbital fissure and an isointense mass with enhancement in the orbital region with dislocation of the optic nerve. Pathological and immunohistochemistry examination of the bioptical specimen was consistent with a diagnosis of RDD. Treatment with levetiracetam and steroids was started obtaining only remission of seizures. Because of the patient refusal of the surgical debulking, therapy with mercaptopurine was started, stopping disease progression. Conclusion. So far, very few cases of extranodal RDD with multiple CNS lesions involving the orbital region have been described. Our case is significant because it is the first case in which the efficacy of mercaptopurine treatment has been documented in an adult patient with isolated ocular and intracranial RDD.

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“…Tatsächlich können einige kutane lymphoproliferative Erkrankungen, wie das subkutane, einer Pannikulitis ähnliche T-Zell-Lymphom, einer LP ähneln [5]. Es wurde über Patienten mit RDS und systemischem Lupus erythematodes (SLE) berichtet [7,8] Die hauptsächlichen Unterscheidungsmerkmale in unserem Fall sind die Dichte des Infiltrats und das Vorhandensein großer Histiozyten mit reichlich Zytoplasma, Emperipolese und Expression von sowohl CD68-als auch S-100-Protein.…”

Section: Clinical Letterunclassified

A case of histiocytic lymphophagocytic panniculitis (Rosai‐Dorfman disease) with positive autoantibodies

Wang

Chen

Wu³

et al. 2015

J Deutsche Derma Gesell

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Sehr geehrte Herausgeber, das Rosai-Dorfman-Syndrom (RDS; auch bekannt als Sinushistiozytose mit massiver Lymphadenopathie) ist eine gutartige, häufig protrahierte, histiozytäre, proliferative Erkrankung. In der Regel tritt eine massive, aber schmerzlose, bilaterale, zervikale Lymphadenopathie auf, begleitet von Fieber, Anämie, erhöhter Senkungsgeschwindigkeit von Erythrozyten und polyklonaler Hypergammaglobulinämie [1]. Das am häufigsten betroffene Organ bei extranodalem RDS ist die Haut, mit verschiedenen kutanen Erscheinungsformen. Das kutane RDS wird in ca. 10 % der Fälle festgestellt [2]. Gleichwohl ist das RDS, welches nur das subkutane Fettgewebe betrifft, häufig als histiozytäre lymphophagozytische Pannikulitis bezeichnet, eine sehr seltene Form der Erkrankung. Wir stellen einen Fall von RDS, assoziiert mit Autoantikörpern, vor. Unsere Patientin zeigte eine dramatische subkutane Beteiligung ohne Lymphadenopathie und reagierte gut auf Prednisolon.Eine 20-jährige Frau stellte sich mit palpablen, jedoch unsichtbaren asymptomatischen Knoten an ihrer Taille und rechten Hüfte vor, die seit neun Monaten existierten. Die Läsionen waren sukzessive gewachsen. Die Patientin zeigte keine Symptome wie Fieber, Ermüdung, Arthralgien oder orale Ulzera.Bei der klinischen Untersuchung zeigten sich verschiedene subkutane Knoten, im Durchmesser etwa 1,5 cm groß, lokalisiert an Taille und am rechten Oberschenkel. Die darüberliegende Haut war normal (Abbildung 1). Beim Tasten waren die Knoten beweglich, nicht mit der darüber liegenden Haut verbunden, etwas fest und derb. Es lag keine palpable Lymphadenopathie oder Hepatosplenomegalie vor. Die Laboranalysen ergaben Folgendes: Das große Blutbild, die Erythrozyten-Senkungsgeschwindigkeit und Serumelektrophorese waren im normalen Bereich. Urin-Protein war negativ. Positive Autoantikörper umfassten antinukleäre Antikörper (ANA) 4,26 (normal < 1) und Anti-Doppelstrang-DNA-Antikörper

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Rituximab Treatment in a Child with Rosai-Dorfman Disease and Systemic Lupus Erythematosus

Cited by 37 publications

References 5 publications

Successful Treatment of Recurrent Autoimmune Cytopenias in the Context of Sinus Histiocytosis With Massive Lymphadenopathy Using Sirolimus

Successful Treatment of Recurrent Autoimmune Cytopenias in the Context of Sinus Histiocytosis With Massive Lymphadenopathy Using Sirolimus

Mercaptopurine Treatment in an Adult Man with Orbital and Intracranial Rosai-Dorfman Disease

A case of histiocytic lymphophagocytic panniculitis (Rosai‐Dorfman disease) with positive autoantibodies

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