Acute respiratory failure caused by secondary alveolar proteinosis in a patient with acute myeloid leukemia: a case report

Gacouin, Arnaud; Tulzo, Yves Le; Suprin, E.; Briens, E.; Bernard, Marc; Camus, Christophe; Thomas, R.

doi:10.1007/s001340050563

Cited by 19 publications

(5 citation statements)

References 9 publications

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“…Hematologic malignancies, predominantly myelogenous leukemias are well established causes. [59][60][61][62] This observation is supported by the resolution of pulmonary process after the restoration of normal hematopoietic function. 63 Immunodeficiency disorders such as thymic alymphoplasia, 64 and IgA deficiency, 65 or acquired states such as solid organ transplantation, 66 treatment with imatinib mesylate 67 and acquired immune deficiency syndrome 68,69 have been associated with PAP.…”

Section: Secondary Papmentioning

confidence: 73%

Pulmonary alveolar proteinosis

2006

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Pulmonary alveolar proteinosis is a rare syndrome characterized by intra-alveolar accumulation of surfactant components and cellular debris, with minimal interstitial inflammation or fibrosis. The condition has a variable clinical course, from spontaneous resolution to respiratory failure and death due to disease progression or superimposed infections. The standard of care for alveolor proteinosis therapy is represented by whole lung lavage. Important discoveries have been made in the last decade with respect to disease pathogenesis and therapy of both congenital and acquired forms of the disease. Granulocyte-macrophage colony-stimulating factor (GM-CSF) pathway has been shown to be involved in the disease pathogenesis of both acquired and congenital disease. Furthermore, anti-GM-CSF blocking autoantibodies have been found in the serum and bronchoalveolar lavage fluid and seem to interfere with the surfactant clearance by alveolar macrophages in many acquired cases. In the congenital form, the most common defects identified to date are several mutations of the genes encoding GM-CSF receptor subunits or surfactant proteins. Using GM-CSF as a therapeutic tool has also been shown to be effective in at least half of the acquired cases treated, while the importance of quantitative determination of anti-GM-CSF antibodies before and during the course of the therapy, as well as the autoantibody titer-GM-CSF dose relationship are to be elucidated. The congenital form of the disease does not respond to therapy with GM-CSF, consistent with the known primary defects and differences in disease pathogenesis.

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Section: Secondary Papmentioning

confidence: 73%

Pulmonary alveolar proteinosis

2006

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“…Although neutralizing antibodies to GM-CSF are present in patients with primary PAP, 14,30,31 and secondary PAP has been linked to AML, 32,33 CML 34 and MDS, 35,36 the prevalence of these antibodies among leukemia patients and their biological link to myeloid leukemia is unknown. Kitamura et al 14 showed that anti-GM-CSF IgG, but not IgM or IgA, are present in patients with PAP and may be pathogenic in this disease.…”

Section: Discussionmentioning

confidence: 99%

High titer autoantibodies to GM-CSF in patients with AML, CML and MDS are associated with active disease

et al. 2008

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“…89 Approximately 10 -30% of patients are asymptomatic at presentation, 9,14,90 whereas others present acutely with rapid progression to respiratory failure. [91][92][93][94][95] Acute and rapid progression can occur because of infection. 12 Pneumothorax and cor pulmonale occur rarely.…”

Section: Clinical Featuresmentioning

confidence: 99%