Secondary pulmonary alveolar proteinosis treated by lung transplant: A case report

Respiratory Medicine Case Reports
David LawiJean-Paul Janssens

Abstract

Pulmonary alveolar proteinosis (PAP) is a pulmonary disease characterized by disruption of surfactant homeostasis resulting in its accumulation in the alveoli. PAP is classically classified into three categories (Table 1): 1/primary (or autoimmune) with antibodies targeting the GM-CSF pathway, 2/secondary to another disease, typically a hematologic malignancy, and 3/genetic. A 30 year-old woman received an allogenic hematopoietic stem cell transplantation (HSCT) after treatment for acute myeloid leukemia (AML). Within the first 6 months post HSCT, she developed an ocular, oral, digestive and hepatic graft-versus-host disease associated with a mixed ventilatory defect with a very severe obstructive syndrome and a severe CO diffusion impairment. High resolution computed tomography showed a classical "crazy paving" pattern. Aspect and differential cell count of BAL were normal. All microbiological samples remained culture negative. Histo-pathological analysis of transbronchial biopsies was unremarkable. Because of the severity of the respiratory insufficiency, open-lung biopsy (OBL) could not be performed. Despite multiple immunosuppressive therapies, lung function deteriorated rapidly; the patient also developed an excavated fung...Continue Reading

References

Jul 18, 2002·American Journal of Respiratory and Critical Care Medicine·John F Seymour, Jeffrey J Presneill
Dec 26, 2003·The New England Journal of Medicine·Bruce C TrapnellKoh Nakata
Nov 7, 2009·Chest·Haruyuki IshiiUNKNOWN Japanese Center of the Rare Lung Disease Consortium
Mar 27, 2010·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Brenna Carey, Bruce C Trapnell
Feb 2, 2011·The European Respiratory Journal·H IshiiK Nakata
Jun 3, 2011·European Respiratory Review : an Official Journal of the European Respiratory Society·R BorieB Crestani
Oct 11, 2014·Hematology/oncology and Stem Cell Therapy·Chakra P ChaulagainJohn K Erban
Dec 17, 2014·Revue des maladies respiratoires·S JouneauP Delaval
Jan 27, 2015·Annual Review of Pathology·Jeffrey A WhitsettTimothy E Weaver
Aug 1, 2019·Current Opinion in Pulmonary Medicine·Cormac McCarthyFrancesco Bonella

❮ Previous
Next ❯

Methods Mentioned

BETA
lavage
biopsies
biopsy
PCR
bronchoalveolar-lavage

Related Concepts

Related Feeds

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

Aspergillosis

Aspergillosis is the name given to a wide variety of diseases caused by infection by fungi of the genus Aspergillus. Aspergillosis occurs in chronic or acute forms which are clinically very distinct. Most cases of acute aspergillosis occur in patients with severely compromised immune systems. Chronic colonization or infection can cause complications in people with underlying respiratory illnesses. Discover the latest research on aspergillosis here.

AML: Role of LSD1 by CRISPR (Keystone)

Find the latest rersearrch on the ability of CRISPR-Cas9 mutagenesis to profile the interactions between lysine-specific histone demethylase 1 (LSD1) and chemical inhibitors in the context of acute myeloid leukemia (AML) here.

Allogenic & Autologous Therapies

Allogenic therapies are generated in large batches from unrelated donor tissues such as bone marrow. In contrast, autologous therapies are manufactures as a single lot from the patient being treated. Here is the latest research on allogenic and autologous therapies.

Aspergillosis (ASM)

Aspergillosis is the name given to a wide variety of diseases caused by infection by fungi of the genus Aspergillus. Aspergillosis occurs in chronic or acute forms which are clinically very distinct. Most cases of acute aspergillosis occur in patients with severely compromised immune systems. Chronic colonization or infection can cause complications in people with underlying respiratory illnesses. Discover the latest research on aspergillosis here.

Acute Myeloid Leukemia

Acute myeloid leukemia (AML) is a clinically and genetically heterogeneous disease with approximately 20,000 cases per year in the United States. AML also accounts for 15-20% of all childhood acute leukemias, while it is responsible for more than half of the leukemic deaths in these patients. Here is the latest research on this disease.

Blood And Marrow Transplantation

The use of hematopoietic stem cell transplantation or blood and marrow transplantation (bmt) is on the increase worldwide. BMT is used to replace damaged or destroyed bone marrow with healthy bone marrow stem cells. Here is the latest research on bone and marrow transplantation.

Related Papers

Pediatric Blood & Cancer
Hiroto InabaRaul C Ribeiro
International Journal of Surgical Pathology
Tsubasa HirakiAkihide Tanimoto
Annals of Thoracic and Cardiovascular Surgery : Official Journal of the Association of Thoracic and Cardiovascular Surgeons of Asia
Hui LiuQinghua Xu
Seminars in Respiratory and Critical Care Medicine
Alan Kelly, Cormac McCarthy
© 2022 Meta ULC. All rights reserved