scholarly journals Langerhans Cell Histiocytosis Presenting as Uncontrolled Asthma

2013 ◽  
Vol 2013 ◽  
pp. 1-4
Author(s):  
Frederic A. Rawlins ◽  
James E. Hull ◽  
Julia A. Morgan ◽  
Michael J. Morris
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Systemic Disease ◽  
Signs And Symptoms ◽  
Cor Pulmonale ◽  
Langerhans Cell ◽  
High Resolution Computed Tomography ◽  
Adult Smoker ◽  
Fourth Decade ◽  
Cystic Changes ◽  
Antigen Presenting

Langerhans cell histiocytosis (LCH) is an uncommon disorder affecting primarily young adult smokers. It is characterized by abnormal proliferation of Langerhans cells, specialized monocyte-macrophage lineage antigen-presenting cells. LCH can affect the lungs in isolation or as part of a systemic disease. Most commonly, the disease presents in the third or fourth decade without gender predominance. Symptoms typically include dyspnea and cough. Commonly, physical examination is unremarkable but cor pulmonale may be observed in advanced disease. The chest radiograph is typically abnormal with nodular or interstitial infiltrates and cystic changes. High-resolution computed tomography of the chest with these findings in the middle and upper lobes of an adult smoker is virtually diagnostic of LCH. Pulmonary function assessment is variable. Asthma has rarely been reported in association with this disorder. There are only three reported cases of the diagnosis of concomitant asthma which have been made in association with the diagnosis of LCH. We present a case in which our patient presented with signs and symptoms of asthma to include confirmatory findings of airway hyperresponsiveness. The diagnosis of LCH was established after the patient failed to respond to conventional treatment for asthma, and further evaluation was completed.

A CASE OF BURNT OUT LANGERHANS CELL HISTIOCYTOSIS PRESENTING AS POST-PARTUM HYPOPITUITARISM

2020 ◽  
Author(s):  
Susmita Reddy Karri ◽  
Amy Hsieh ◽  
John Binder ◽  
Vasant Shenoy
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Post Partum ◽  
Urine Osmolality ◽  
Open Lung Biopsy ◽  
Langerhans Cell ◽  
High Resolution Computed Tomography ◽  
Cystic Lung ◽  
Pulmonary Lymphangioleiomyomatosis ◽  
Cystic Changes ◽  
Systemic Pathology

Objective: Hypopituitarism in post-partum women is usually attributed to lymphocytic hypophysitis (LH), but can also be secondary to systemic pathology. We discuss a case of a lady presenting with central hypogonadism and diabetes insipidus, who further developed persistent cough, leading to an unexpected diagnosis of burnt-out Langerhans’ cell histiocytosis (LCH). Methods: Clinical and laboratory endocrine evaluation, magnetic resonance imaging (MRI), high-resolution computed tomography (HRCT) and open-lung biopsy results are discussed. Results: A 28 year old lady, presented at 10 months postpartum with polydipsia, polyuria and amenorrhoea for 3 months. Her results showed prolactin of 25 (reference,<23.5) μg/L , oestrogen of 24.8 (reference,30–49), follicular stimulating hormone 6 (reference, 2–20) IU/L and luteinizing hormone 6 (reference, 2–70) IU/L. Water deprivation test demonstrated sodium 148 (reference, 135–145) mEq/l, serum osmolality 310 (reference, 275–295) mmol/kg and urine osmolality 107 (reference,50–1450) mmol/kg improving to 142 mEq/L, 295mEq/L and 535 mEq/L respectively, after desmopressin. Gadolinium enhanced pituitary MRI demonstrated a markedly thickened stalk with uniform enhancement. Chest HRCT confirmed bilateral upper zone cystic lung disease suggestive of either pulmonary lymphangioleiomyomatosis (PLAM) or LCH. Eventual histology showed CD1a-positive burnt-out LCH. This differentiation was crucial as PLAM exacerbates with oestrogen therapy and pregnancy which she was able to successfully pursue without disease exacerbation. Conclusion: The patient’s initial presentation was considered as LH, but subsequent cystic changes on HRCT led to a unifying definitive diagnosis of burnt-out LCH. This case highlights the importance of investigating for uncommon secondary causes of hypophysitis.

Self-Healing Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption

2001 ◽  
Vol 5 (6) ◽  
pp. 486-489 ◽  
Author(s):  
Kelli W. Morgan ◽  
Jeffrey P. Callen
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Rare Condition ◽  
Signs And Symptoms ◽  
Male Infant ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Self Healing ◽  
Systemic Involvement ◽  
S 100 ◽  
Simplex Virus

Background: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by the eruption of multiple, disseminated, red-brown papules and nodules which may increase in size and number during the first few weeks of life. Systemic signs are usually absent except for occasional mild hepatomegaly. Objective: We present a 3.5-kg male infant who presented at birth with numerous diffuse, erythematous, crusted erosions. He was presumed to have congenital herpes simplex virus (HSV) and was started on IV acyclovir. Histopathologic examination revealed a mixed inflammatory infiltrate with numerous histiocytes which were S-100 and peanut agglutin positive consistent with CSHLCH. Further workup did not reveal any signs of systemic involvement. Conclusion: CSHLCH has rarely been reported to present as a papulovesicular eruption at birth. In these cases, a viral etiology is commonly entertained in the differential diagnosis. Despite the spontaneous regression of skin lesions in CSHLCH, close followup is required to evaluate for systemic signs and symptoms associated with latent Letterer–Siwe disease.

scholarly journals Unrecognized oral manifestations of Langerhans cell histiocytosis which progressed to systemic disease

2006 ◽  
Vol 42 (1) ◽  
pp. 10-13 ◽  
Author(s):  
I. Alajbeg ◽  
V. Vucicevic Boras ◽  
R. Femenic ◽  
A. Cekic-Arambasin ◽  
M. Anicic ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Systemic Disease ◽  
Langerhans Cell ◽  
Oral Manifestations

scholarly journals Cutaneous neonatal Langerhans cell histiocytosis: a systematic review of case reports

F1000Research ◽  
2019 ◽  
Vol 8 ◽  
pp. 13
Author(s):  
Victoria Venning ◽  
Evelyn Yhao ◽  
Elizabeth Huynh ◽  
John W. Frew
Keyword(s):  
Systematic Review ◽  
Langerhans Cell Histiocytosis ◽  
Case Reports ◽  
Systemic Disease ◽  
Langerhans Cell ◽  
Multiple Lesions ◽  
Risk Of Mortality ◽  
Cochrane Database ◽  
Chi Squared ◽  
Worse Prognosis

Background: Cutaneous langerhans cell histiocytosis (LCH) is a rare disorder characterized by proliferation of cells with phenotypical characteristics of Langerhans cells. Although some cases spontaneously resolve, no consistent variables have been identified that predict which cases will manifest with systemic disease later in childhood. Methods: A systematic review (Pubmed, Embase, Cochrane database and all published abstracts from 1946-2018) was undertaken to collate all reported cases of cutaneous LCH in the international literature. This study was registered with PROSPERO (CRD42016051952). Descriptive statistics and correlation analyses were undertaken. Bias was analyzed according to GRADE criteria. Results: A total of 83 articles encompassing 128 cases of cutaneous LCH were identified. Multiple lesions were weakly associated with an increased length of survival (R=0.304 (p<0.05)), Worse prognosis was associated with internal organ involvement with a statistically significant chi squared statistic (χ2=14.96, 2DF p<0.001) and an elevated odds ratio ((OR)= 12.30 95% CI=2.67-56.74). Vesicular lesions (OR=10.8 95% CI=2.83-41.26), but not ulceration (OR=0.53 95% CI 0.12-2.05) were associated with greater risk of mortality. Conclusions: Congenital and neonatal LCH most commonly presents as multiple lesions in multiple anatomical sites at birth. Significant differences, including the associations of mortality with lesion morphology and number were seen in this neonatal cohort compared to overall pediatric LCH. These findings require validation in a large prospective cohort.

scholarly journals Pediatric Langerhans Cell Histiocytosis (LCH) Clinical Outcome in Hospital Civil De Guadalajara, México

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 4951-4951 ◽  
Author(s):  
Katterine Rojas Rodríguez ◽  
Veronica Soto ◽  
Carlos Rodriguez-Galindo ◽  
Paola M. Friedrich ◽  
Edwin Guzmán ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Langerhans Cell Histiocytosis ◽  
Disease Risk ◽  
Conflicts Of Interest ◽  
Systemic Disease ◽  
Giant Cells ◽  
Langerhans Cell ◽  
Induction Treatment ◽  
Multisystem Disease ◽  
Clonal Proliferation

Abstract Pediatric Langerhans cell histiocytosis (LCH) clinical outcome in Hospital Civil de Guadalajara, México. Introduction : LCH results from clonal proliferation of functionally and immunophenotyped inmature round Langerhans cells along with eosinophiles, machrophages, lymphocytes and ocasionally multinucleated giant cells (1). Its incidence is 2-10 cases by million of children below 15 yr in US (2). Our objective was to describe the clinical characteristics and treatment outcome of patients with LCH at Departement of Hematology-Oncology of Hospital Civil de Guadalajara México. Methods: It was a retrospective design and 41 pediatric patients below 18 yr were included. The diagnosis was corroborated by pathology and immunohistochemistry. Variables as age, gender, localised vs systemic disease, risk organ commitment, global survival (GS) and event free survival (EFS) were analysed. We used descriptive and inferencial statistics with SPSS program. Results: There were included 41 patients from January 1st 2012 to December 31st 2017. Relation male:female was 1.1:1. Mean presentation was localised disease (58%). Bone was the principal affected structure (34%) and it was 71% to be combined with lung, lymph node and CNS compromise. Risk organ commitment was presented in 32%, being more frecquent bone marrow and liver in 22% each one. Time induction treatment was equal or below 12 weeks in 66% of patients. The 25% of patients had reactivation of LCH, with similar lesions to the beggining in 19.5%. We found statistically significant differences between dead patients (DP) (14.6%) and not dead patients (NDP) (85.4%) in clinical presentation: localised (0% in DP vs 69% in NDP) and systemic disease (100% vs 31%) (p=0,003) and risk organ commitment (100% in DP vs 20% in NDP) (p=0,000). Median age of 13 vs 24 months was for DP and NDP respectively. Conclusion: Dead patients were younger than 13 months old, with systemic disease, and risk organ commitment. We found a later asking of medical advice in DP (6 months) vs NDP (2 months). Keys words: langerhans cell histiocytosis, multisystem disease, risk organ Figure. Figure. Disclosures No relevant conflicts of interest to declare.

scholarly journals Smoking and Other Interstitial Lung Diseases

2011 ◽  
Vol 2011 ◽  
pp. 1-7
Author(s):  
Carpio Carlos ◽  
Gómez-Carrera Luis ◽  
Álvarez-Sala Rodolfo
Keyword(s):  
Computed Tomography ◽  
Smoking Cessation ◽  
Langerhans Cell Histiocytosis ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Langerhans Cell ◽  
Lung Disorder ◽  
High Resolution Computed Tomography ◽  
Pulmonary Langerhans Cell Histiocytosis

Cigarette smoking has been implicated in the development of some uncommon respiratory interstitial diseases. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung diseases are characterized by a diffuse alveolar and peribronchiolar filling with macrophages, respectively. Pulmonary Langerhans' cell histiocytosis is a rare interstitial lung disorder characterized by the proliferation of Langerhans' cell forming interstitial infiltrates and nodules that could progress to cavitary nodules. The treatment of these disorders involves smoking cessation and sometimes the use of steroids. High-resolution computed tomography is essential for the characterization of these smoking-related interstitial lung diseases, but frequently it is necessary to create a workgroup composed by pulmonologists, pathologists, and radiologists to diagnosis and treat patients affected with these pathologies.

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