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Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy.

May A, Kane G, Yi E, Frantz R, Vassallo R - Respir Med Case Rep (2014)

Bottom Line: Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments.Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established.We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Mayo Clinic, Rochester, MN, 55905, USA.

ABSTRACT
Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH.

No MeSH data available.


Related in: MedlinePlus

A, Surgical lung biopsy demonstrating increased presence of inflammatory cells along with cavitary and cystic changes. B, Lung Biopsy with CD1a immunostaining demonstrating the presence of increased Langerhans cells.
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fig1: A, Surgical lung biopsy demonstrating increased presence of inflammatory cells along with cavitary and cystic changes. B, Lung Biopsy with CD1a immunostaining demonstrating the presence of increased Langerhans cells.

Mentions: Chest high resolution computed tomography revealed bilateral cystic changes and scattered nodularity with an upper lung predominance and sparing of the costophrenic angles. Her surgical lung biopsy slides from the outside facility were reviewed for diagnostic confirmation. Her lung specimens revealed multiple nodules composed of Langerhans cells with mixed inflammatory cells, cavitation and adjacent cystic changes (Fig. 1A). CD1a immunostaining identified the presence of increased Langerhans cells (Fig. 1B). Pulmonary function testing demonstrated mild-moderate expiratory air-flow obstruction, preserved lung volumes, and moderately reduced gas-exchange capacity. The index echocardiogram revealed normal ventricular structure and function with an elevated right ventricular systolic pressure (RVSP) of 48 mmHg.


Dramatic and sustained responsiveness of pulmonary Langerhans cell histiocytosis-associated pulmonary hypertension to vasodilator therapy.

May A, Kane G, Yi E, Frantz R, Vassallo R - Respir Med Case Rep (2014)

A, Surgical lung biopsy demonstrating increased presence of inflammatory cells along with cavitary and cystic changes. B, Lung Biopsy with CD1a immunostaining demonstrating the presence of increased Langerhans cells.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4356044&req=5

fig1: A, Surgical lung biopsy demonstrating increased presence of inflammatory cells along with cavitary and cystic changes. B, Lung Biopsy with CD1a immunostaining demonstrating the presence of increased Langerhans cells.
Mentions: Chest high resolution computed tomography revealed bilateral cystic changes and scattered nodularity with an upper lung predominance and sparing of the costophrenic angles. Her surgical lung biopsy slides from the outside facility were reviewed for diagnostic confirmation. Her lung specimens revealed multiple nodules composed of Langerhans cells with mixed inflammatory cells, cavitation and adjacent cystic changes (Fig. 1A). CD1a immunostaining identified the presence of increased Langerhans cells (Fig. 1B). Pulmonary function testing demonstrated mild-moderate expiratory air-flow obstruction, preserved lung volumes, and moderately reduced gas-exchange capacity. The index echocardiogram revealed normal ventricular structure and function with an elevated right ventricular systolic pressure (RVSP) of 48 mmHg.

Bottom Line: Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments.Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established.We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Mayo Clinic, Rochester, MN, 55905, USA.

ABSTRACT
Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon diffuse lung disease characterized by the abnormal accumulation of Langerhans' cells around small airways and other distal lung compartments. Although pulmonary hypertension (PH) is a frequent complication of PLCH, the role of advanced PH therapies for PLCH-related PH is not well-established. We describe a PLCH patient with severe, disease-related PH that responded unexpectedly well to advanced PH therapy with sustained improvement over a 10 year follow-up period. This case indicates that PLCH-associated PH may, in certain instances, be highly responsive to advanced PH therapies and emphasizes the importance of trialing these therapies among patients with PLCH-related PH.

No MeSH data available.


Related in: MedlinePlus