Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

Emile, Jean François; Abla, Oussama; Fraïtag, Sylvie; Horne, AnnaCarin; Haroche, Julien; Donadieu, Jean; Requena-Caballero, Luís; Jordan, Michael B.; Abdel‐Wahab, Omar; Allen, Carl E.; Charlotte, Frédéric; Diamond, Eli L.; Egeler, R. Maarten; Fischer, Alain; Herrera, J; Henter, Jan‐Inge; Janků, Filip; Mérad, Miriam; Picarsic, Jennifer; Rodríguez‐Galindo, Carlos; Rollins, Barret J.; Tazi, Abdellatif; Vassallo, Robert; Weiss, Lawrence M.

doi:10.1182/blood-2016-01-690636

Cited by 1,219 publications

(1,434 citation statements)

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“…CD207 is a type II transmembrane C-type lectin associated with the formation of Birbeck granules. 16 This test is now the Gold Standard in the correct clinical setting. 17,18 Erdheim-chester disease (ECD) is a rare disease, frequently seen in adults.…”

Section: Discussionmentioning

confidence: 99%

An Unusual Intracranial Inflammatory Process of Unknown Origin

Badran¹,

Abdelsadq²,

Abbas³

et al. 2017

Neuro – Open Journal

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We present an unusual case of steroid responsive inflammatory condition, involving sellar suprasellar region with further ependymal lesions. This is complicated by previous surgery due to pituitary adenoma, not thought to related to inflammatory process. The patient responded well to steroids, but deteriorated due to development of hydrocephalus caused by obstruction due to adhesions. Despite extensive literature review and consideration of all known pathological conditions, it was concluded that the condition represented another inflammatory entity not yet fully characterised. The case also highlights that despite the steroid responsive nature of the condition, the ependymal involvement can result in progressive acute obstructive hydrocephalus with clinical deterioration. This case also suggests close follow-up and early imaging for early detection and treatment of this complication. CASE REPORTThis case report pertains to a 46-year-old male Caucasian who had endoscopic treatment for a pituitary adenona three years previously. He required replacement hydrocortisone and testosterone. Prior to the surgery, he was not known to have any other neurological issues.A year after surgery following minor trauma, he underwent a computed tomography (CT) scan of the head. This confirmed no residual pituitary tumour (Figure 1). He was somewhat non-compliant with endocrine follow-up. Surveillance magnetic resonance imaging (MRI) imaging 2 years following initial surgery demonstrated post-up change with some enhancing tissue scaling the pituitary stalk and hypothalamic region; this was not clearly seen on the previous CT scan, possibly due to different modalities and absence of contrast imaging. Two months later, he developed polydipsia, fatigue and a partial left-sided homonymous visual field deficit. There was progressive confusion with fluent dysphasia and inattention. He was pyrexial. Gaze evoked nystagmus was present to the right. The rest of his examination was unremarkable. MRI brain demonstrated significant progression of the abnormalities seen on the earlier scan with increased enhancing tissue involving the pituitary stalk, hypothalamic area and floor of the 3 rd ventricle, also including the optic chiasm (Figure 2). Associated signal

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Section: Discussionmentioning

confidence: 99%

An Unusual Intracranial Inflammatory Process of Unknown Origin

Badran¹,

Abdelsadq²,

Abbas³

et al. 2017

Neuro – Open Journal

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“…It is characterized by the accumulation of macrophages, dendritic cells or monocytederived cells in various tissues and organs [6]. It accounts for less than 1% of all cases of haemato-lymphoid neoplasms and it has predominance in children and young adult males [7].…”

Section: Discussionmentioning

confidence: 99%

Extensive Mucocutaneous Histiocytic Sarcoma raised from an Acute B Lymphocytic Leukemia: A Case Report at Hospital Mexico, Costa Rica

Pineda¹,

Chaverri²,

García³

et al. 2017

JLRDT

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“…1,2 Heterogeneous clinical manifestations depend on site and degree of involvement. Skeletal involvement with sclerotic lesions and radiotracer uptake is common, often asymptomatic and incidentally detected by radiological studies.…”

Section: Introductionmentioning

confidence: 99%

Tocilizumab in patients with multisystem Erdheim–Chester disease

et al. 2017

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Treatment of Erdheim-Chester disease (ECD), a rare non-Langerhans histiocytosis, relies on interferon-a, chemotherapeutic agents such as purine analogs, cytokine-blocking agents and BRAF inhibitors. Since interleukin (IL)-6 levels are elevated in serum and lesions of ECD patients, we evaluated the therapeutic efficacy and safety of IL-6 blockade with tocilizumab. We conducted an open-label, single-arm, phase II, prospective study of tocilizumab in three patients with multisystem ECD and poor tolerance/contraindications to IFN-a. Modifications of symptoms attributed to ECD represented the criteria for evaluation of clinical response. Changes at positron emission tomography scan, computed tomography scan, and magnetic resonance imaging at month 6 represented the main criteria for the evaluation of radiological response.Sustained complete clinical response and partial radiological improvement were observed in two patients, paralleled by modulation of systemic pro-inflammatory mediators. In spite of disease stabilization or improvement at extra-neurological sites, a third patient experienced a radiologic and clinical progression of central nervous system involvement, mirrored by a dramatic increase of circulating IL-6 and related cytokines. These findings indicate that IL-6 inhibition can be effective in ECD, but caution is advisable in patients with neurologic involvement. IL-6 emerges as a central mediator in ECD pathogenesis.

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Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages

Cited by 1,219 publications

References 93 publications

An Unusual Intracranial Inflammatory Process of Unknown Origin

An Unusual Intracranial Inflammatory Process of Unknown Origin

Extensive Mucocutaneous Histiocytic Sarcoma raised from an Acute B Lymphocytic Leukemia: A Case Report at Hospital Mexico, Costa Rica

Tocilizumab in patients with multisystem Erdheim–Chester disease

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