Clinical Aspects and Evolution under Current Treatments on Pulmonary Alveolar Phospholipoproteinosis Patients

Petru-Emil Muntean

Overview

A 34-year-old female smoker (4 packs a year), with the following symptoms: dry cough, moderate dyspnea, fatigue, night sweats and no exposure to respiratory poisoning or contact with tuberculosis. Clinical findings at admission: normal temperature, Hippocratic fingers and cyanosis, diminished bilateral breath sounds, fine bilateral rattling respiratory sounds, rhythmic cardiac noises, oxygen saturation 95%, pulse 87, blood pressure 125/80 mm Hg. Chest computed tomography describes infiltration images of intra-alveolar and intra-bronchial with matte glass pattern, confluent and stretched across all lung segments. Pulmonary biopsy: preserved pulmonary architecture, thick alveolar septs and terminal bronchioles, alveoli and macrophages loaded with lipoprotein material. Bronchial aspirate: negative BAAR, no tumor cells. Bronchoalveolar lavage: opalescent, abundant PAS + appearance. total cell numbers - 4.3 million, macrophages - 34.1%, lymphocytes - 56.9%, neutrophils - 10%, eosinophils - 0.4%, epithelial cells - 33%.

Summary

The treatment option chosen for this case was total bronchoalveolar lavage for therapeutic purposes. The patient had a rapid favourable evolution and at discharge was recommended a periodic imaging and functional control.

Author Comments

Dr. Petru-Emil Muntean, MD, Masters Degree
Dr. Petru-Emil Muntean, MD, Masters Degree
Spitalul Judetean de Urgenta Arges, Dispensarul de Pneumoftiziologie Pitesti
Medic specialist pneumolog
Competence in Bronchology and Spirometry
Pitesti, Arges | Romania
Key Messages: Bronchoalveolar lavage is the optimal diagnostic method. Moderate/severe forms, total bronchoalveolar lavage is recommended. Systemic corticotherapy or immunosuppression is not indicated. GM-CSF administered subcutaneously or by nebulization. Rituximab/plasmapheresis are under evaluation. Pulmonary transplantation is indicated in patients who do not respond to therapeutic bronchoalveolar lavage repeated 6-12 months.Dr. Petru-Emil Muntean, MD, Masters Degree

Resources

Folia Medica
DOI: 10.2478/folmed-2018-0048

Clinical Aspects and Evolution under Current Treatments on Pulmonary Alveolar Phospholipoproteinosis Patients.

Authors:
Dr. Petru-Emil Muntean, MD, Masters Degree
Dr. Petru-Emil Muntean, MD, Masters Degree
Spitalul Judetean de Urgenta Arges, Dispensarul de Pneumoftiziologie Pitesti
Medic specialist pneumolog
Competence in Bronchology and Spirometry
Pitesti, Arges | Romania

Folia Med (Plovdiv) 2019 Mar;61(1):148-151

Department of Pneumology, Cimpeni Chronic Diseases Hospital, Alba County,Romania.

A 34-year-old female smoker (4 packs a year), with the following symptoms: dry cough, moderate dyspnea, fatigue, night sweats and no exposure to respiratory poisoning or contact with tuberculosis. Clinical findings at admission: normal temperature, Hippocratic fingers and cyanosis, diminished bilateral breath sounds, fine bilateral rattling respiratory sounds, rhythmic cardiac noises, oxygen saturation 95%, pulse 87, blood pressure 125/80 mm Hg. Chest computed tomography describes infiltration images of intra-alveolar and intra-bronchial with matte glass pattern, confluent and stretched across all lung segments. Pulmonary biopsy: preserved pulmonary architecture, thick alveolar septs and terminal bronchioles, alveoli and macrophages loaded with lipoprotein material. Bronchial aspirate: negative BAAR, no tumor cells. Bronchoalveolar lavage: opalescent, abundant PAS + appearance. total cell numbers - 4.3 million, macrophages - 34.1%, lymphocytes - 56.9%, neutrophils - 10%, eosinophils - 0.4%, epithelial cells - 33%.The treatment option chosen for this case was total bronchoalveolar lavage for therapeutic purposes. The patient had a rapid favourable evolution and at discharge was recommended a periodic imaging and functional control. Key Messages: Bronchoalveolar lavage is the optimal diagnostic method. Moderate/severe forms, total bronchoalveolar lavage is recommended. Systemic corticotherapy or immunosuppression is not indicated. GM-CSF administered subcutaneously or by nebulization. Rituximab/plasmapheresis are under evaluation. Pulmonary transplantation is indicated in patients who do not respond to therapeutic bronchoalveolar lavage repeated 6-12 months.

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Source
http://dx.doi.org/10.2478/folmed-2018-0048DOI Listing
March 2019
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