scholarly journals SURG-34. MULTIDISCIPLINARY MANAGEMENT OF A PATIENT WITH OPTIC PATHWAY-HYPOTHALAMIC LANGERHANS CELL HISTIOCYTOSIS: A CASE REPORT

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii210-ii210
Author(s):  
John Emmanuel Custodio ◽  
Kevin Paul Ferraris ◽  
Joseph Erroll Navarro ◽  
Kenny Seng ◽  
Jose Carlos Alcazaren ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
S100 Protein ◽  
Visual Fields ◽  
Langerhans Cell ◽  
Normal Body Mass Index ◽  
Team Approach ◽  
Hypothalamic Region ◽  
Suprasellar Region ◽  
Increased Risk ◽  
History Of

Abstract Langerhans cell histiocytosis (LCH) of the Central Nervous System (CNS) is rare. Isolated involvement of the hypothalamic region is much more extremely rare with only 0.04 to 0.6% of the cases. We report a case of a 33 year-old female who presented with a one-year history of amenorrhea and a five-month history of intermittent headache, memory lapse, and somnolence. The patient was of normal body mass index with normal visual acuity and intact visual fields. Laboratory examinations revealed panhypopituitarism with central diabetes insipidus. Cranial magnetic resonance imaging showed a large lobulated mass measuring 1.9 x 2.2 x 2 cm in the suprasellar region which extended to the pituitary infundibulum, hypothalamus and retrochiasmatic region, with surrounding edema. The patient underwent right orbitozygomatic craniotomy and subtotal excision of the mass through subfrontal and transsylvian approaches. Histopathological examination of langerhans cells were observed with positive immunohistochemical stain for CD1a and S100 protein antigen markers establishing a diagnosis of CNS LCH. Thoracoabdominal computed tomography scan and bone scan were done postoperatively and showed no evidence of extracranial lesions. The patient had been receiving prednisone and vinblastine based chemotherapy regimen. She remains to be asymptomatic and on close surveillance. To date, there is no standardized treatment strategy for CNS LCH in the adult population. An accurate histopathologic diagnosis and a specialized multidisciplinary team approach especially involving Oncology, Neurosurgery, Ophthalmology and Endocrinology are critical to optimally tailor possible effective treatment options for patients with this similar disease. Long-term follow-up is crucial due to the increased risk of local recurrence and multisystemic involvement.

Progressive Mucinous Histiocytosis: Importance of Electron Microscopy to Confirm Diagnosis

2010 ◽  
Vol 14 (5) ◽  
pp. 245-248 ◽  
Author(s):  
Iman Hemmati ◽  
W. Alastair McLeod ◽  
Richard I. Crawford
Keyword(s):  
Electron Microscopy ◽  
Light Microscopy ◽  
Clinical Examination ◽  
Crucial Role ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
History Of ◽  
Characteristic Features ◽  
Immunohistochemical Studies

Background: Progressive mucinous histiocytosis (PMH) is a benign, non-Langerhans cell histiocytosis with characteristic ultrastructural features that can be used for diagnosis. Once an important tool in dermatologic diagnosis, electron microscopy has been largely replaced by immunohistochemistry and immunofluorescence techniques today. However, electron microscopy occasionally still plays a crucial role in the diagnosis of dermatologic conditions. We report a case of PMH as an example of a dermatologic disorder that requires electron microscopy for its diagnosis. Methods: A 60-year-old woman presented to our clinic with a history of small, sharply demarcated, skin-colored papules ranging from 2 to 5 mm in diameter distributed over the arms, forearms, and dorsal hands. The results of light microscopy, immunohistochemical studies, and clinical examination were inconclusive. Another biopsy for electron microscopy showed the characteristic features of PMH. Conclusion: This case demonstrates that a dermatopathology service still needs to have access to electron microscopy for diagnostic purposes to successfully diagnose a small number of rare conditions.

scholarly journals Revealing a rare inflammatory oral manifestation in a 6-year-old child

2019 ◽  
Vol 12 (7) ◽  
pp. e229483
Author(s):  
Samapika Routray ◽  
Amit Kumar Adhya ◽  
Joseph John ◽  
Punit Dikhit
Keyword(s):  
Bone Loss ◽  
Buccal Mucosa ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Oral Manifestation ◽  
Atypical Cells ◽  
History Of ◽  
Permanent Molars ◽  
Superficial Dermis ◽  
Distal Aspect

A 6-year-old child with an episodic history of ulcerations over buccal mucosa was found to have severe inflammation on the palatal aspect of permanent first molars with grade 2 mobility bilaterally. Radiographical features were suggestive of bone loss around permanent molars extending to the distal aspect of the deciduous first molars. The clinical and radiographical findings were indicative of periodontal degeneration without any apparent cause visible intraorally. Further biopsy was done from the rashes present on the malar prominences, which showed nodular aggregates of atypical cells in superficial dermis. These large histiocytic cells with vesicular nuclei and nuclear grooves were immunopositive for CD1a and S100, concluding the diagnosis of Langerhans cell histiocytosis. For treatment, patient was referred to Department of Haemato-oncology and chemotherapy was suggested as per protocol.

TNF-α-238GA, IL-1α-889CC and IL-10–819CC Polymorphisms Confere an Increased Risk to Develop Multisystem Langerhans Cell Histiocytosis.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 1331-1331
Author(s):  
Paola De Filippi ◽  
Cesare Danesino ◽  
Diego Ferrarese ◽  
Annalisa De Silvestri ◽  
Carla Badulli ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Bone Lesion ◽  
Multiple Organ ◽  
Langerhans Cell ◽  
Cytokine Network ◽  
Regional Lymph Nodes ◽  
Cytokines And Chemokines ◽  
Increased Risk ◽  
Tnf Α ◽  
Disseminated Disease

Abstract Langerhans cell Histiocytosis (LCH) (OMIM 604856) is a rare disorder affecting any age, with a highly variable clinical course, usually related to the number and type of organs affected at the time of presentation. The symptoms range from a solitary bone lesion that does not require treatment to a disseminated disease with multiple-organ involvement and a high mortality rate, despite aggressive treatment. The pathogenesis of LCH remains unclear, although deregulated growth, activity and trafficking of Langerhans cells (LC) are implicated. The ability of LC to migrate from the epidermis to regional lymph nodes is of pivotal importance for the induction of immune response and there is increasing evidence that both cytokines and chemokines are implicated in this function. We report the analysis of polymorphisms in genes coding for different cytokines in a population of patients with LCH in comparison to an Italian control population (n=140). We studied 40 patients, with a mean age at the disease of onset of 6.1 (±5.0) years; the 15 with single-system (SS) disease had a mean age of 7.1 (±3.9) years, while the 25 with multi-system (MS) disease had a mean age of 5.3 (±5.3) years. The Cytokine Genotyping Tray (Pel-Freez, Milwaukee, USA) was used for the detection of the following polymorphisms: Il-1α-889 C/T, Il-1β (−511 C/T; +3962 T/C), IL-1R pst1 1970 C/T, IL-RA mspa1 11100 T/C, IL-4Rα +1902 G/A, IL-12 -1188 C/A, γ IFN UTR 5644 A/T, TNF α (−308 G/A; −238 G/A), IL-2 (−330 T/G; +160 G/T), IL-4 (−1098T/G; −590 T/C; −33 T/C), IL-6 (−174 G/C; nt565 G/A), IL-10 (−1082 G/A; −819 C/T; −592 C/A). The following polymorphisms showed a different distribution in patients versus controls: IL-4–33 (p=0.048); IL-4–590 (p=0.005); IL-1α-889 (p=0.009). The correspondence analysis of these variables showed that genotypes IL-4 -33TT, IL-4 -590 TC and-1β+3962TT confer an increased risk of developing SS disease, while genotypes TNF-α-238GA, IL-1α-889CC and IL-10–819CC give an increased risk of developing MS disease. The present study supports the concept that constitutional variants affecting the cytokine network may induce susceptibility to develop LCH and also affect its manifestations. Figure Figure

The Frequency and Natural History of Diabetes Insipidus in Children with Langerhans-Cell Histiocytosis

1989 ◽  
Vol 321 (17) ◽  
pp. 1157-1162 ◽  
Author(s):  
David B. Dunger ◽  
Valerie Broadbent ◽  
Elizabeth Yeoman ◽  
Jonathan R. Seckl ◽  
Stafford L. Lightman ◽  
...  
Keyword(s):  
Natural History ◽  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
History Of

scholarly journals Use of the Robson classification to understand the increased risk of cesarean section in case of maternal obesity.

2020 ◽  
Author(s):  
Simon Crequit ◽  
Diane Korb ◽  
Cécile Morin ◽  
Thomas Schmitz ◽  
Olivier Sibony
Keyword(s):  
Body Mass Index ◽  
Risk Factor ◽  
Cesarean Section ◽  
Retrospective Cohort ◽  
Independent Risk Factor ◽  
Maternal Obesity ◽  
Normal Body Mass Index ◽  
Robson Classification ◽  
Increased Risk ◽  
History Of

Abstract Background: The aim of this study was to identify characteristics of pregnant women with obesity that contribute to increased cesarean rate.Methods: Retrospective cohort in a single academic institution between 2012 and 2019. Women who delivered during this period were classified according to the Robson classification. Women with normal body mass index (N=11797) and with obesity (N=2991) were compared. The contribution of each Robson group to the overall caesarean rate were compared.Results: The overall cesarean rate was higher for women with (28.1%) than without (14.2%, p < 0.001) obesity. This result came mainly from Robson group 5a (history of one cesarean). After adjustment for medical factors within this group, the association between maternal obesity and cesarean during labor was significant.Conclusions: The higher cesarean rate in women with obesity is explained by Robson group 5a in which obesity is an independent risk factor of in labor cesarean delivery.

scholarly journals Hemophagocytic Lymphohistiocytosis (HLH) in Langerhans Cell Histiocytosis (LCH): A Multicenter Retrospective Descriptional Study

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 707-707 ◽  
Author(s):  
Deepak Chellapandian ◽  
Rui Zhang ◽  
Michael Jeng ◽  
Cor Van Den Bos ◽  
Vicente Santa-María López ◽  
...  
Keyword(s):  
Diagnostic Criteria ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Langerhans Cell Histiocytosis ◽  
Conflicts Of Interest ◽  
Interleukin 2 ◽  
Langerhans Cell ◽  
Braf V600e ◽  
Bone Lesions ◽  
Normal Reference ◽  
Increased Risk

Abstract Introduction: Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia characterized by the accumulation of CD1a+ CD207+ histiocytes. Hemophagocytic lymphohistiocytosis (HLH), a non-malignant histiocytic disorder, is typified by the accumulation and activation of CD8+ T cells and macrophages, which secrete high levels of pro-inflammatory cytokines. The co-existence of LCH and HLH has been reported, albeit rarely, and is believed to be associated with a poorer outcome. To better understand the relationship between these two conditions, in this study we sought to describe the incidence, risk factors for development, and outcome of HLH when it develops in children and young adults with multisystem-LCH (MS-LCH). Methods: We conducted a retrospective study involving 14 centers and collected data on 384 MS-LCH patients aged less than 30 years and who were diagnosed between year 2000 and 2015. Data collected on the eligible patients included clinical information at the time of LCH diagnosis, clinical and laboratory parameters at HLH diagnosis (for those who developed HLH), treatment and disease outcome. Patients who developed HLH were classified as having "true-HLH", which was defined as disease fulfilling 5 of 8 HLH-2004 diagnostic criteria or as "HLH-like" disorder, which was defined as fulfilling <5 of 8 HLH diagnostic criteria but whose disease status was suggestive of HLH and treated with HLH- and/or LCH-directed therapy. Results: Of 384 MS-LCH patients, 44 (11%) were identified with HLH (29 with true HLH and 15 with an HLH-like disorder), ranging in age from 15 days to 20.6 years (median, 1.12 years). The majority of MS-LCH patients who also had HLH were females (n=27) and had accompanying risk organ (liver, spleen and/or hematopoietic system) involvement (RO+) (n=40), as opposed to non-HLH MS-LCH patients. Among nine HLH patients tested for BRAF V600E mutation status, eight were found to be positive. Twenty (45%) patients developed HLH (true or HLH-like) concurrent (±7 days) with LCH diagnosis, while 24 (55%) developed HLH >7 days before or after LCH diagnosis. The 3-year cumulative incidence of HLH (true or HLH-like) in MS-LCH was 16.8%. The 5-year overall survival of LCH patients without HLH was 98 ± 9%, while survival for those with an HLH-like disorder or true-HLH was 75 ± 12% and 70 ± 14%, respectively (P<0.0001). Age <2 years, female gender, RO+ and lack of bone involvement at LCH diagnosis were each independently associated with increased risk for HLH. Among 20 HLH patients with available data, the median soluble interleukin-2 receptor level (sIL-2R) was 16,220 U/mL (range, 1,149 to 60,420 U/mL) (normal reference <2,400 U/mL), ferritin was 505 ng/mL (range, 28 to 26,660 ng/mL) (normal reference <500 ng/mL), and sIL-2R/ferritin ratio was 42. Conclusion: The development of HLH in patients with MS-LCH was not uncommon and associated with a poorer prognosis. Young females with RO+ MS-LCH who lack bone lesions at LCH diagnosis were at increased risk of developing HLH. Ferritin levels appear to be lower in comparison to patients who develop HLH in other contexts. There are overlapping features between MS-LCH and HLH that make the clinical distinction between these disorders difficult. Accordingly, improved biomarkers are needed to facilitate the identification of HLH in patients with MS-LCH. It is anticipated that early identification of HLH and prompt intervention may improve the outcome for affected individuals. Future prospective studies are needed to better understand the underlying mechanisms and identify more effective therapies. Disclosures No relevant conflicts of interest to declare.

scholarly journals Pulmonary Langerhans cell histiocytosis with inguinal abscess: Unusual presentation

2020 ◽  
Vol 29 (1) ◽  
pp. 64-66
Author(s):  
Kamarul Naim Mohd Hirmizi ◽  
Nurul Yaqeen Mohd Esa ◽  
Mardiana Abdul Aziz ◽  
Nor Salmah Bakar ◽  
Mohammad Hanafiah
Keyword(s):  
Lymph Node ◽  
Langerhans Cell Histiocytosis ◽  
Respiratory Symptoms ◽  
Inguinal Lymph Node ◽  
Langerhans Cell ◽  
Histopathological Analysis ◽  
Intermittent Fever ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
History Of

We report a case of a 30-year-old man who was treated as recurrent right inguinal abscess following a 2-month history of right inguinal swelling and intermittent fever with no respiratory symptoms. Resection of his right inguinal lymph node and the histopathological analysis revealed the diagnosis of Langerhans cell histiocytosis. In addition, the CT of the thorax showed presence of bilateral cystic lung changes consistent with pulmonary Langerhans cell histiocytosis.

scholarly journals Use of the Robson classification to understand the increased risk of cesarean section in case of maternal obesity

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Simon Crequit ◽  
Diane Korb ◽  
Cécile Morin ◽  
Thomas Schmitz ◽  
Olivier Sibony
Keyword(s):  
Body Mass Index ◽  
Risk Factor ◽  
Cesarean Section ◽  
Retrospective Cohort ◽  
Independent Risk Factor ◽  
Maternal Obesity ◽  
Normal Body Mass Index ◽  
Robson Classification ◽  
Increased Risk ◽  
History Of

Abstract Background The aim of this study was to identify characteristics of pregnant women with obesity that contribute to increased cesarean rate. Methods Retrospective cohort in a single academic institution between 2012 and 2019. Women who delivered during this period were classified according to the Robson classification. Women with normal body mass index (N = 11,797) and with obesity (N = 2991) were compared. The contribution of each Robson group to the overall caesarean rate were compared. Results The overall cesarean rate was higher for women with (28.1%) than without (14.2%, p < 0.001) obesity. This result came mainly from Robson group 5a (history of one cesarean). After adjustment for medical factors within this group, the association between maternal obesity and cesarean during labor was significant. Conclusions The higher cesarean rate in women with obesity is explained by Robson group 5a in which obesity is an independent risk factor of in labor cesarean delivery.

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