SavedAdd your journal

Journals / The Anatolian Journal of Cardiology / 2021 / Cilt: 25 - Sayı: 9

Sustained response to targeted therapies in a patient with pulmonary hypertension owing to Langerhans cell histiocytosis

Aykun HAKGÖR
Hacer Ceren TOKGÖZ
Özgür Yaşar AKBAL
Seda TANYERİ
Berhan KESKİN
Barkın KÜLTÜRSAYAR
Ali KARAGÖZ
Cihangir KAYMAZ
Pages
665–668
DOI
—
🏛️Bibliographic metadata and full text access for this article are provided via DergiPark.
DergiPark Record ↗
✨

AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

Generally, pulmonary arterial hypertension (PAH) targeted therapies are not considered to be beneficial in this subgroup of PH.

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Pulmonary hypertension (PH) in association with Pulmonary Langerhans Cell Histiocytosis (PLCH) is an uncommon pulmonary vascular disease classified in group 5 PH according to currently available guidelines for the diagnosis and treatment of PH.
📊
Key Findings & Data:Oxygen support, diuretic treatment and lung transplantation are standard therapies for this disease.
💡
Conclusion & Impact:Generally, pulmonary arterial hypertension (PAH) targeted therapies are not considered to be beneficial in this subgroup of PH.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Özet

Pulmonary hypertension (PH) in association with Pulmonary Langerhans Cell Histiocytosis (PLCH) is an uncommon pulmonary vascular disease classified in group 5 PH according to currently available guidelines for the diagnosis and treatment of PH. Oxygen support, diuretic treatment and lung transplantation are standard therapies for this disease. Generally, pulmonary arterial hypertension (PAH) targeted therapies are not considered to be beneficial in this subgroup of PH.

🔖

Cite This Article

HAKGÖR, A., TOKGÖZ, H. C., AKBAL, Ö. Y., TANYERİ, S., KESKİN, B., KÜLTÜRSAYAR, B., KARAGÖZ, A., KAYMAZ, C. (2021). Sustained response to targeted therapies in a patient with pulmonary hypertension owing to Langerhans cell histiocytosis. The Anatolian Journal of Cardiology, Cilt: 25 - Sayı: 9, 665–668. https://ajindex.com/articles/365165

📄 Full Text PDF Viewer

📄

PDF görüntüleyici hazırlanıyor...

References (1)

  1. Roden AC, Yi ES. Pulmonary Langerhans Cell Histiocytosis: An Update From the Pathologists' Perspective. Arch Pathol Lab Med 2016; 140: 230-40. [Crossref] 2. Kinoshita Y, Watanabe K, Sakamoto A, Hidaka K. Pulmonary Langerhans Cell Histiocytosis-associated Pulmonary Hypertension Showing a Drastic Improvement Following Smoking Cessation. Intern Med 2016; 55: 491-5. [Crossref] 3. Epaud R, Ducou Le Pointe H, Fasola S, Ploussard S, Delestrain C, Sileo C, et al. Cladribine improves lung cysts and pulmonary function in a child with histiocytosis. Eur Respir J 2015; 45: 831-3. [Crossref] 4. Nemoto K, Oh-Ishi S, Inui T, Nakazawa M, Hyodo K, Nakajima M, et al. Long-term improvement during tadalafil therapy in a patient with pulmonary hypertension secondary to pulmonary Langerhans cell histiocytosis. Respir Med Case Rep 2016; 18: 54-7. [Crossref] 5. May A, Kane G, Yi E, Frantz R, Vassallo R. Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy. Respir Med Case Rep 2014; 14: 13-5. [Crossref] 6. Le Pavec J, Lorillon G, Jaïs X, Tcherakian C, Feuillet S, Dorfmüller P, et al. Pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension: clinical characteristics and impact of pulmonary arterial hypertension therapies. Chest 2012; 142: 1150-7. [Crossref] 7. Fartoukh M, Humbert M, Capron F, Maître S, Parent F, Le Gall C, et al. Severe pulmonary hypertension in histiocytosis X. Am J Respir Crit Care Med 2000; 161: 216-23. [Crossref] 8. Yoshida T, Konno S, Tsujino I, Sato T, Ohira H, Chen F, et al. Severe pulmonary hypertension in adult pulmonary Langerhans cell histiocytosis: the effect of sildenafil as a bridge to lung transplantation. Intern Med 2014; 53: 1985-90. [Crossref] 9. Weatherald J, Savale L, Humbert M. Medical Management of Pulmonary Hypertension with Unclear and/or Multifactorial Mechanisms (Group 5): Is There a Role for Pulmonary Arterial Hypertension Medications? Curr Hypertens Rep 2017; 19: 86. [Crossref]