Histiocytosis

2020 ◽  
pp. 5259-5262
Author(s):  
Chris Hatton

The histiocytoses are disorders derived from the dendritic cell and monocyte/macrophage lineages, with the classification of this group of disorders relating to the underlying cell of origin. Dendritic cell disorders—there has been much debate about the nature of these conditions, and their status as neoplastic or primary inflammatory diseases; for Langerhans’ cell histiocytosis in particular, there is increasing evidence of their clonal nature, as manifest by recurrent BRAF mutations. Clinical features and diagnosis—these are highly variable and dependent on the sites affected by histiocytic infiltration. Symptoms and signs may include rashes, bony pain, lymphadenopathy, hepatomegaly and splenomegaly, cough and dyspnoea, features of marrow failure, and endocrine presentations (classically diabetes insipidus). Diagnosis typically follows imaging and biopsy, with the demonstration of a histiocytic infiltrate confirmed by immunostaining. Treatment and prognosis—the rarity and heterogeneity of these diseases has made it difficult to achieve a consensus on treatment. For localized disease, curettage, steroid injections, or targeted radiotherapy may be helpful. For more systemic disease, combination chemotherapy is typically used. Treatment schedules differ between adults and children. Prognosis is dependent mainly on the site(s) of involvement. Our expanding appreciation of the molecular basis of these conditions also provides some justification for the use of BRAF inhibitors and other targeted small molecule therapies. Macrophage-related disorders—these include haemophagocytic lymphohistiocytosis, a collection of macrophage-activating syndromes which may be either reactive to underlying inflammatory, infective, or neoplastic disease, or consequent upon a primary genetic lesion affecting cytotoxic T-cell killing function. Rosai–Dorfman disease is a separate macrophage proliferation syndrome, thought to be non-neoplastic, which causes massive cervical lymphadenopathy, usually in children.

2021 ◽  
pp. 112067212110676
Author(s):  
Ahmad Abdel-Aty ◽  
Ninani Kombo

Chronic inflammatory diseases can cause significant psychosocial stress in affected patients. Few studies have examined the psychological effects of ocular inflammatory disease and no studies have examined the psychological effects of scleritis. In this study we evaluate the prevalence of mental health disorders in scleritis patients and we conduct a comprehensive review of the literature on the mental health effects of ocular inflammatory diseases. 162 patients (195 eyes) presenting to a tertiary care center with scleritis were identified. At least one comorbid mental health disorder was diagnosed in 35 patients (21.6%), most commonly major depression in 11.7%, generalized anxiety disorder in 9.3%, and substance use disorder in 6.2%. There were no significant differences in the length of an episode of scleritis or in the probability of symptom resolution between patients with a mental health disorder and other patients. In a review of the literature, 30 manuscripts met the inclusion criteria. The majority of manuscripts (83.3%) were focused on uveitis patients. Eight of these studies were focused on patients with uveitis in the context of systemic disease. The most commonly reported mental health disorders reported were anxiety and depression. An average of 31.3% of patients with ocular inflammatory disease had depression and 35.0% had anxiety. Similar to other chronic illnesses, ocular inflammatory disease may be a significant psychosocial stressor. Future studies will further elucidate the relationship between these diseases and mental health.


2012 ◽  
Vol 48 (3) ◽  
pp. 176-181 ◽  
Author(s):  
Kelli Smith ◽  
Robert O'Brien

In this retrospective study, radiographically enlarged sternal lymph nodes (LNs) were evaluated in 71 dogs and 13 cats for average size, location, and most representative radiographic view. Concurrent clinical diagnoses were also noted and grouped into one of three following categories: neoplastic, inflammatory, or hematologic. There were no statistically significant differences in LN size between lateral views within each species. Enlarged sternal LNs were more cranially positioned in dogs than cats. No statistical difference was noted between right and left laterals, as to on which projection the enlarged sterna lymph nodes was seen best. Neoplastic disease (78.9%) was the most prevalent condition seen in association with LN enlargement in dogs, followed by primary infectious or inflammatory diseases (14.1%) and various hematologic conditions (7.0%). In cats, neoplasia was also most common (69.2%), followed by inflammatory diseases (30.8%). No hematologic conditions were noted in cats. The most common etiologic agent seen concurrently with enlarged sternal LNs in both dogs (33.8%) and cats (38.5%) was malignant lymphoma. The results of this study provide a clinically useful representation of the average size and location of radiographically enlarged sternal LNs for dogs and cats. The diseases represented demonstrate the wide spectrum of potential causes of sternal lymphadenopathy.


Blood ◽  
2012 ◽  
Vol 120 (11) ◽  
pp. 2259-2268 ◽  
Author(s):  
Charles-Antoine Dutertre ◽  
Sonia Amraoui ◽  
Annalisa DeRosa ◽  
Jean-Pierre Jourdain ◽  
Lene Vimeux ◽  
...  

Abstract HIV infects activated CD4+ T cells and induces their depletion. Progressive HIV infection leading to AIDS is fueled by chronic immune hyperactivation, mediated by inflammatory cytokines like TNFα. This has been related to intestinal epithelial damage and microbial LPS translocation into the circulation. Using 11-color flow cytometry, cell sorting, and cell culture, we investigated the numbers and TNFα production of fully defined circulating dendritic cell and monocyte populations during HIV-1 infection. In 15 viremic, untreated patients, compared with 8 treated, virologically suppressed patients or to 13 healthy blood donors, circulating CD141 (BDCA-3)+ and CD1c (BDCA-1)+ dendritic cell counts were reduced. Conversely, CD14+CD16++ monocyte counts were increased, particularly those expressing M-DC8, while classical CD14++CD16−M-DC8− monocyte numbers were unchanged. Blood mononuclear cells from viremic patients produced more TNFα in response to LPS than those from virologically suppressed patients. M-DC8+ monocytes were mostly responsible for this overproduction. Moreover, M-DC8+ monocytes differentiated in vitro from classical monocytes using M-CSF and GM-CSF, which is increased in viremic patient's plasma. This M-DC8+ monocyte population, which is involved in the pathogenesis of chronic inflammatory diseases like Crohn disease, might thus be considered as a major actor in the immune hyperactivation fueling HIV infection progression.


1992 ◽  
Vol 59 (1_suppl) ◽  
pp. 57-59
Author(s):  
S. Valerio ◽  
G.F. Fornasiero ◽  
G. Antoniazzi ◽  
V. Petterle ◽  
E. Bassi

Xanthogranulomatous pyelonephritis is a particular form of renal infection that frequently mimics a neoplastic disease and other inflammatory diseases. The Authors present their experience with three cases of xanthogranulomatous pyelonephritis and discuss the clinical and pathological features.


Heart ◽  
2019 ◽  
Vol 106 (7) ◽  
pp. 541-544 ◽  
Author(s):  
Massimo Imazio ◽  
Andrea Biondo ◽  
Davide Ricci ◽  
Massimo Boffini ◽  
Emanuele Pivetta ◽  
...  

ObjectiveBiochemical analysis of pericardial fluid (PF) is commonly performed for the initial assessment of PF, and the results are usually interpreted according to Light’s traditional criteria for the differential diagnosis of transudates versus exudates. However, Light’s criteria have been formulated for the biochemical analysis of pleural fluid. The aim of the present paper is to evaluate the normal composition of PF in candidates for cardiac surgery.MethodsCohort study with analysis of PF from candidates for cardiac surgery. Exclusion criteria were previous pericardial disease or cardiac surgery, prior myocardial infarction within 3 months, systemic disease (eg, systemic inflammatory diseases, uremia) or drug with potentiality to affect the pericardium.ResultsFifty patients (mean age was 67 years; 95% CI 64 to 71, 29 males, 58.0%) were included in the present analysis. Levels of small molecules were similar in blood and PF. Total proteins in PF was, on average, 0.5 times lower than corresponding plasma levels (p=0.041), while the level of pericardial lactate dehydrogenase was, on average, 1.06 times higher than plasma (p=0.55). Moreover, mononuclear cells were more concentrated in PF than plasma (p=0.17). Traditional Light’s criteria misclassified all PFs as exudates.ConclusionsTraditional Light’s criteria misclassified normal PFs in candidates for cardiac surgery as exudates. This study suggests their futility for the biochemical analysis of PF in clinical practice.


2021 ◽  
pp. 112067212110280
Author(s):  
Jean-Baptiste Ducloyer ◽  
Romain Marignier ◽  
Sandrine Wiertlewski ◽  
Pierre Lebranchu

Optic neuritis (ON) can be associated with inflammatory disease of the central nervous system or can be isolated, with or without relapse. It can also be associated with infectious or systemic disease. These multiple associations based on a variety of clinical, radiological, and biological criteria that have changed over time have led to overlapping phenotypes: a single ON case can be classified in several ways simultaneously or over time. As early, intensive treatment is often required, its diagnosis should be rapid and precise. In this review, we present the current state of knowledge about diagnostic criteria for ON aetiologies in adults and children, we discuss overlapping phenotypes, and we propose a homogeneous classification scheme. Even if distinctions between typical and atypical ON are relevant, their phenotypes are largely overlapping, and clinical criteria are neither sensitive enough, nor specific enough, to assure a diagnosis. For initial cases of ON, clinicians should perform contrast enhanced MRI of the brain and orbits, cerebral spinal fluid analysis, and biological analyses to exclude secondary infectious or inflammatory ON. Systematic screening for MOG-IgG and AQP4-IgG IgG is recommended in children but is still a matter of debate in adults. Early recognition of neuromyelitis optica spectrum disorder, MOG-IgG-associated disorder, and chronic relapsing idiopathic optic neuritis is required, as these diagnoses require therapies for relapse prevention that are different from those used to treat multiple sclerosis.


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