Secondary Pulmonary Alveolar Proteinosis Associated with Myelodysplastic Syndrome.

Ohnishi, Tetsuro; Yamada, Gen; Shijubo, Noriharu; Takagi-Takahashi, Yoko; Itoh, Takayuki; Takahashi, Hiroki; Satoh, Masaaki; Nakata, Koh; Abe, Shosaku

doi:10.2169/internalmedicine.42.187

Cited by 31 publications

(19 citation statements)

References 15 publications

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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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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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“…While the acquired form of PAP is associated with an autoimmune process directed at the granulocyte-macrophage colony-stimulating factor (GM-CSF), resulting in suppression of proper alveolar function, the congenital type is associated with a rare mutation in the GM-CSF receptor [3, 16, 17]. Along those same lines, secondary PAP in association with hematological disorders such as CDA II could result from defective GM-CSF receptors on pulmonary macrophages and/or in a disrupted signal transduction pathway after GM-CSF and receptor interaction, which has been proposed elsewhere [8]. It is possible, as well, that our patient had a functional impairment of alveolar macrophages resulting from the environmental disruption of normal robust monocyte production caused by pathogenic erythrocyte production inherent to CDA II.…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary alveolar proteinosis (PAP) is also a rare disorder in which lipoproteinaceous material congregates within alveolar spaces, leading to a host with impaired pulmonary immunity and susceptibility to opportunistic infection [2]. Although historically reported in patients with various hematological disorders [3–8], to our knowledge, this is the first case report of PAP associated with CDA.…”

Section: Introductionmentioning

confidence: 99%

Pulmonary Alveolar Proteinosis in Association with Congenital Dyserythropoietic Anemia: A Case Report

Carden

Barman

Massey

2012

Case Reports in Pediatrics

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A two-year-old girl with congenital dyserythropoietic anemia (CDA) acutely developed fever, tachypnea, and increased oxygen requirement. Chest X-ray revealed bilateral interstitial infiltrates and mild cardiomegaly. Blood cultures grew no infectious agents, while pulmonary specimens grew cytomegalovirus (CMV). Treatment with intravenous ganciclovir was initiated but without response. Final cytologic preparations of bronchoalveolar lavage (BAL) fluid revealed eosinophilic amorphous material consistent with pulmonary alveolar proteinosis (PAP). CDA and PAP are extremely rare disorders in pediatrics. PAP should be considered in patients with hematological disorders who present with acute interstitial pneumonia, after infectious causes are ruled out.

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“…IPAP is likely autoimmune, and autoantibodies to GM-CSF may be detected, especially in adults [2], but autoantibodies are not found in SPAP [2, 11]. In SPAP arising in hematological malignancy, the alveolar macrophage may be defective because it derives from the malignant clone and has a defective GM-CSF transduction pathway [6, 12]. Treatment of IPAP with aerosolized and subcutaneous GM-CSF has produced mixed results [13, 14].…”

Section: Discussionmentioning

confidence: 99%

Secondary Pulmonary Alveolar Proteinosis

Chung

Pipavath

Myerson

et al. 2009

Journal of Thoracic Imaging

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Summary Objective We analyzed the CT and clinical findings of pulmonary alveolar proteinosis secondary to hematological malignancy. Methods Seven patients with hematological malignancy and pathologically proven secondary pulmonary alveolar proteinosis were identified from 2000−2007. Six had chest CT scans, which were analyzed retrospectively; medical records were also reviewed. Results Patient age ranged from 30−54 years. Four had chronic myelogenous leukemia, 1 had myelodysplastic syndrome, and 1 had cutaneous T cell lymphoma. As in idiopathic pulmonary alveolar proteinosis, geographic ground-glass opacities with or without septal thickening were most common (5/6). No axial or zonal predominance was present. Two patients died from respiratory failure. Conclusion It is important to consider secondary pulmonary alveolar proteinosis as a cause of geographic ground-glass opacities and septal thickening in a patient with hematological malignancy. Whereas idiopathic pulmonary alveolar proteinosis has a low mortality rate, the death of 2 of our 6 patients implies that secondary pulmonary alveolar proteinosis may have a worse prognosis. Our case of secondary pulmonary alveolar proteinosis associated with cutaneous T-cell lymphoma is the first described in the literature.

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Secondary Pulmonary Alveolar Proteinosis Associated with Myelodysplastic Syndrome.

Cited by 31 publications

References 15 publications

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

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

Pulmonary Alveolar Proteinosis in Association with Congenital Dyserythropoietic Anemia: A Case Report

Secondary Pulmonary Alveolar Proteinosis

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