Logo American Journal of Case Reports

Call: 1.631.629.4328
Mon-Fri 10 am - 2 pm EST

Contact Us

Logo American Journal of Case Reports Logo American Journal of Case Reports Logo American Journal of Case Reports

30 December 2017 : Case report  Japan

Autopsy Findings in A Case of Pulmonary Langerhans Cell Histiocytosis-Associated Pulmonary Hypertension

Rare disease

Daisuke Taniyama12ABCDEFG*, Hirofumi Kamata1ABCDE, Keisuke Miyamoto1ABCDE, Shuko Mashimo1ABCDE, Fumio Sakamaki13ABCDEFG

DOI: 10.12659/AJCR.905860

Am J Case Rep 2017; 18:1401-1406

Abstract

BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) can be associated with pulmonary hypertension, although this association is more prevalent with other interstitial lung diseases. However, the diagnosis and effective treatment strategies for PLCH-associated pulmonary hypertension remain controversial.

CASE REPORT: A 27-year-old woman, who was an ex-smoker, was diagnosed with multiple diffuse pulmonary cysts. At 35 years-of-age, she developed neurogenic pituitary diabetes insipidus and was diagnosed with PLCH-associated pulmonary hypertension. Despite treatment, including sildenafil, bosentan, and prostanoids, she died at 39 years-of-age. At the autopsy examination, the heart showed right ventricular dilation and hypertrophy. Histopathological examination of the lungs showed severe hypertrophy of the media of the small pulmonary arteries. The diagnosis of PLCH was confirmed by S100-positive immunohistochemical staining.

CONCLUSIONS: The autopsy findings of a case of PLCH with severe pulmonary hypertension are reported. The mechanism of pulmonary hypertension in this disease may involve a combination of World Health Organization (WHO) Group 1 pulmonary arterial hypertension (PAH) and Group 3 pulmonary hypertension secondary to lung disease, even though PLCH is currently classified as Group 5. The use of pulmonary artery vasodilators, such as sildenafil, may be effective for the treatment of PLCH-associated pulmonary hypertension, but treatment should be considered individually for each patient. Controlled clinical trials of pulmonary artery vasodilator therapy for this condition are needed.

Keywords: Persistent Fetal Circulation Syndrome, S100 Proteins

Add Comment 0 Comments

In Press

12 Feb 2024 : Case report  Germany

Diagnostic Challenges and Imaging Considerations for Intraparotid Facial Nerve Schwannoma: A Case Report an...

Am J Case Rep In Press; DOI: 10.12659/AJCR.942870  

0:00

12 Feb 2024 : Case report  USA

Erdheim-Chester Disease Occult on Radiographs and CT but Visible on MRI and PET

Am J Case Rep In Press; DOI: 10.12659/AJCR.941169  

0:00

12 Feb 2024 : Case report  USA

Surgical Treatment of Spontaneous Superficial Temporal Artery Arteriovenous Malformation: A Case Report

Am J Case Rep In Press; DOI: 10.12659/AJCR.942839  

13 Feb 2024 : Case report  Bahrain

Warfarin Woes: A Rare Case of Hemoperitoneum with Intramural Small Bowel Hematoma

Am J Case Rep In Press; DOI: 10.12659/AJCR.943519  

Most Viewed Current Articles

10 Jan 2022 : Case report  Germany

A Report on the First 7 Sequential Patients Treated Within the C-Reactive Protein Apheresis in COVID (CACOV...

DOI :10.12659/AJCR.935263

Am J Case Rep 2022; 23:e935263

19 Jul 2022 : Case report  Saudi Arabia

Atlantoaxial Subluxation Secondary to SARS-CoV-2 Infection: A Rare Orthopedic Complication from COVID-19

DOI :10.12659/AJCR.936128

Am J Case Rep 2022; 23:e936128

05 Mar 2022 : Case report  Brazil

Acute Limb Ischemia After Self-Injection of Crushed Morphine Tablets into the Radial Artery: Role of Infrar...

DOI :10.12659/AJCR.935336

Am J Case Rep 2022; 23:e935336

02 Apr 2022 : Case report  Saudi Arabia

Infective Endocarditis Caused by Pseudomonas luteola in a Pediatric Patient. A Case Report and Review of Li...

DOI :10.12659/AJCR.935743

Am J Case Rep 2022; 23:e935743

Your Privacy

We use cookies to ensure the functionality of our website, to personalize content and advertising, to provide social media features, and to analyze our traffic. If you allow us to do so, we also inform our social media, advertising and analysis partners about your use of our website, You can decise for yourself which categories you you want to deny or allow. Please note that based on your settings not all functionalities of the site are available. View our privacy policy.

American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923