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.

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.

Pulmonary Langerhans Cell Histiocytosis

2020 ◽  
Vol 41 (02) ◽  
pp. 269-279
Author(s):  
Brian Shaw ◽  
Michael Borchers ◽  
Dani Zander ◽  
Nishant Gupta
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Treatment Options ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Mitogen Activated Protein Kinase ◽  
Myeloid Neoplasm ◽  
Cystic Lung ◽  
Pulmonary Langerhans Cell Histiocytosis ◽  
Mitogen Activated Protein ◽  
The Central Nervous System

AbstractPulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. A subset of patients with PLCH may have extrapulmonary involvement, typically involving the skeletal system in the form of lytic lesions, skin lesions, or the central nervous system most commonly manifesting in the form of diabetes insipidus. Smoking cessation is the cornerstone of treatment in patients with PLCH and can lead to disease regression or stabilization in a substantial proportion of patients. Further insight into the underlying molecular pathogenesis of PLCH has paved the way for the future development of disease-specific biomarkers and targeted treatment options directed against the central disease-driving mutations.

scholarly journals Lung cysts in chronic paracoccidioidomycosis

2013 ◽  
Vol 39 (3) ◽  
pp. 368-372 ◽  
Author(s):  
Andre Nathan Costa ◽  
Edson Marchiori ◽  
Gil Benard ◽  
Mariana Sponholz Araujo ◽  
Bruno Guedes Baldi ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Retrospective Analysis ◽  
Interstitial Pneumonia ◽  
Langerhans Cell Histiocytosis ◽  
Lung Diseases ◽  
Lung Parenchyma ◽  
Langerhans Cell ◽  
Lung Cysts ◽  
Cystic Lung ◽  
Lymphocytic Interstitial Pneumonia

On HRCT scans, lung cysts are characterized by rounded areas of low attenuation in the lung parenchyma and a well-defined interface with the normal adjacent lung. The most common cystic lung diseases are lymphangioleiomyomatosis, Langerhans cell histiocytosis, and lymphocytic interstitial pneumonia. In a retrospective analysis of the HRCT findings in 50 patients diagnosed with chronic paracoccidioidomycosis, we found lung cysts in 5 cases (10%), indicating that patients with paracoccidioidomycosis can present with lung cysts on HRCT scans. Therefore, paracoccidioidomycosis should be included in the differential diagnosis of cystic lung diseases.

scholarly journals PULMONARY AND INTRACRANIAL RADIOGRAPHIC PRESENTATIONS OF LANGERHANS CELL HISTIOCYTOSIS

2019 ◽  
Vol 5 (1) ◽  
pp. 21-25
Author(s):  
D. Mohammed ◽  
S. B. Patel
Keyword(s):  
Case Report ◽  
Rare Disease ◽  
Langerhans Cell Histiocytosis ◽  
Open Lung Biopsy ◽  
Langerhans Cell ◽  
X Rays ◽  
Osteolytic Lesions ◽  
Systemic Manifestations ◽  
Systemic Symptoms ◽  
Radiographic Presentation

Background. Langerhans Cell Histiocytosis is a rare disease that affects 1 to 2 adults per million worldwide and often consists of systemic manifestations including pulmonary, intracranial and osteolytic lesions and endocrinologic abnormalities such as Diabetes Insipidus. Objective. The objective of this case report was to expand the medical literature of this rare disease. Methods. A case report of a 51-year-old female patient presenting with systemic symptoms as a result of Langerhans Cell Histiocytosis is presented. Results. A 51-year-old female presented with epistaxis, fatigue, polydipsia, polyuria, headaches and dyspnea. After initial x-rays showed multiple lung and liver nodules and the patient suffered subsequently from a unilateral pneumothorax, an open lung biopsy was recommended. On a pathological basis, the patient was diagnosed with Langerhans Cell Histiocytosis. This report focuses on the radiological presentations of the manifestations of Langerhans Cell Histiocytosis, particularly the presentations in the lung and intracranial regions. Conclusions. Langerhans Cell Histiocytosis is an incredibly rare disease that presents systemically. Recognizing and differentiating radiographic presentation of these patients is important to determine the need for confirmation by biopsy and early chemotherapeutic intervention.

scholarly journals Isolated Langerhans cell histiocytosis in the hypothalamic-pituitary region: a case report

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Weibin Zhou ◽  
Jia Rao ◽  
Chengjiang Li
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Symptoms ◽  
Elevated Serum ◽  
Urine Osmolality ◽  
Langerhans Cell ◽  
Lymphocytic Hypophysitis ◽  
Pituitary Stalk ◽  
High Signal ◽  
Cns Disorders ◽  
Hormonal Evaluation

Abstract Background Langerhans cell histiocytosis (LCH) is a rare disease that mainly affects children, but this disease is significantly rarer in patients who are older than 15 years. In this disease, any organ can be involved. The skeleton, skin and lung are commonly affected, and isolated hypothalamic-pituitary (HP) involvement is relatively rare. Here we report a 17-year-old adolescent with isolated HP-LCH of enlarged pituitary stalk presented with central diabetes insipidus (CDI). Case presentation A 17-year-old male adolescent with polydipsia and polyuria accompanied with elevated serum sodium level and low urine osmolality for 3 weeks was referred to our hospital. After admission, hormonal evaluation showed that his growth hormone (GH) was slightly elevated, and serum osmolality and glucose were normal. The fluid deprivation-vasopressin test demonstrated CDI. Imaging examination showed an obvious thickening of the pituitary stalk. Lymphocytic hypophysitis, sarcoidosis and granulation tissue lesions were suspected. After oral 1-deamino-8-Darginine vasopressin (DDAVP) and prednisone were administered for 2 months, symptoms were relieved, and he discontinued taking the drugs by himself. On reexamination, imaging revealed changes in the size and shape of the pituitary stalk, with thickened nodules. Then, a diagnostic biopsy of the pituitary stalk lesion was performed. Immunohistochemistry confirmed the definitive diagnosis of LCH. The clinical symptoms subsided with oral hormone replacements. Conclusion CDI is a rare symptom in children and adolescents. Most of the causes are idiopathic, while others are caused by central nervous system (CNS) disorders. Meanwhile, lymphocytic hypophysitis, germinoma, LCH and other CNS disorders can all present as thickening of the pituitary stalk, diffuse enlargement of the pituitary gland, and weakening of high signal intensity in the neurohypophysis on magnetic resonance imaging (MRI). The differential diagnosis among these diseases depends on immunohistochemistry evidence.

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 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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