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Lymphoproliferative lung disorders: clinicopathological aspects

Venerino Poletti, Claudia Ravaglia, Sara Tomassetti, Carlo Gurioli, Gianluca Casoni, Silvia Asioli, Alessandra Dubini, Sara Piciucchi, Marco Chilosi
European Respiratory Review 2013 22: 427-436; DOI: 10.1183/09059180.00004313
Venerino Poletti
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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  • For correspondence: venerino.poletti@gmail.com
Claudia Ravaglia
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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Sara Tomassetti
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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Carlo Gurioli
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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Gianluca Casoni
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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Silvia Asioli
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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Alessandra Dubini
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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Sara Piciucchi
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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Marco Chilosi
1Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, 2Dept of Anatomic Pathology, Ospedale GB Morgagni, Forlì, 3Dept of Radiology, Ospedale GB Morgagni, Forlì, and 4Dept of Anatomic Pathology, Università di Verona, Verona, Italy.
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  • Figure 1.
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    Figure 1.

    a) 18-fluorodeoxyglucose positron emission tomography-computed tomography in a 72-year-old male revealed pulmonary nodules in the lower lobes. Surgical lung biopsy of the consolidation in the right lower lobe revealed lymphomatoid granulomatosis. b) Lymphomatoid granulomatosis histopathological specimen in the same patient showing a well-demarcated cellular nodule with a central area of necrosis (asterisk). The preserved alveolar parenchyma is shown in the lower part of the image, below the dashed line. c) The angiocentric nature of the lesion.

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    Figure 2.

    a) An axial multidetector computed tomography scan in a 43-year-old female, demonstrating consolidations with air bronchograms. b) Transbronchial biopsy of the consolidation revealed Hodgkin lymphoma. c) Immunophenotypic analysis revealed strong expression of activation markers (CD30).

Tables

  • Figures
  • Table 1. Classification system for the pulmonary lymphoproliferative disorders
    Spectrum of pulmonary lymphoproliferative disorders
        Reactive pulmonary lymphoid diseases
            Follicular bronchiolitis
            Nodular lymphoid hyperplasia (pulmonary pseudo-lymphoma)
            Lymphocytic interstitial pneumonia
        Castleman’s disease
            Localised (hyaline-vascular, plasma cell type)
            Multicentric
        Primary pulmonary lymphoma
            B-cell primary pulmonary non-Hodgkin lymphoma (MALT lymphoma)
            Primary pulmonary diffuse large B-cell lymphoma
            Lymphomatoid granulomatosis
            Follicular lymphoma
            Mantle cell lymphoma
            Extra-osseous plasmacytoma
            Intravascular large B-cell lymphoma
            Large B-cell lymphoma
            Plasmoblastic lymphoma
            T-/NK-lymphoma
            Anaplastic large cell lymphoma
            Hodgkin lymphoma
        Post-transplantation lymphoproliferative disorders
    • MALT: mucosa-associated lymphoid tissue; NK: natural killer cell.

  • Table 2. Clinical profile and laboratory syndromes: red flags for clinicians
    Asymptomatic lesionsMALT lymphoma
    Serum para-proteins and/or increased LDH
    Collagen-vascular diseaseMALT lymphoma in collagen-vascular disorders (e.g. Sjögren)
    ImmunodeficiencyLymphomatoid granulomatosis in HIV
    Lymphomatoid granulomatosis in Wiskott–Aldrich syndrome
    TransplantationPost-transplant lymphoproliferative processes
    Respiratory symptomsT-cell lymphomas
    Lymphopenia and/or eosinophiliaGranulomatous mycosis fungoides
    Hodgkin lymphoma
    ThromboembolismIntravascular lymphoma
    Acute pulmonary hypertension-like onset
    Increased LDH and/or hypercalcaemia
    Haemophagocytic syndrome#NK-/T-cell lymphoma, nasal type
    T-cell lymphoma
    Organising pneumoniaHodgkin lymphoma
    Lymphomatoid granulomatosis
    Treatment with methotrexate, natalizumab or other drugsAtypical lymphoproliferative processes
    • MALT: mucosa-associated lymphoid tissue; LDH: lactate dehydrogenase; NK: natural killer cell. #: abrupt onset, increased triglycerides, increased ferritin, coagulation abnormalities, increased transaminases, and decreased fibrinogen and cytopenia/pancytopenia.

  • Table 3. Summary of the computed tomography features in the malignant spectrum of pulmonary lymphoproliferative disorders
    Lymphoproliferative lung disordersComputed tomography features
    MALT lymphomaNodules or consolidation
    Peribronchovascular
    Multiple/bilateral (70%)
    Hilar/mediastinal nodes (30%)
    Ground-glass attenuation (halo sign)
    Rarely cysts, pleural effusion, tree-in-bud
    High-grade primary large pulmonary B-cell lymphomasNodule or mass cavitation in 50% Rarely pleural effusion
    Lymphomatoid granulomatosisBilateral nodules/masses
    Peribronchovascular
    Coalescence and/or cavitate
    Pleural effusion (30%)
    Hilar adenopathies (<25%)
    Migratory
    Uncommon: single nodules, alveolar opacities and reticulonodular diffuse lesions
    Follicular lymphomaGround-glass opacities (crazy paving)
    Nodules
    Intravascular large B-cell lymphomaBilateral reticular shadows
    Reticulonodular or nodular shadows
    Ground-glass opacities
    Wedge-shaped subpleural opacities
    Pleural effusion
    Nasal-type T-/NK-lymphomasNodules or excavated masses
    Pleural effusion
    Anaplastic large cell lymphoma, T-cell typeMasses or single nodules
    Mycosis fungoidesNodules with halo signs
    Peripheral consolidation
    Crazy paving pattern
    Primary pulmonary Hodgkin diseaseSolitary mass or multinodular disease
    Air bronchograms or cavitation
    Pleural effusion, lymphadenopathy
    • MALT: mucosa-associated lymphoid tissue; NK: natural killer cell.

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Lymphoproliferative lung disorders: clinicopathological aspects
Venerino Poletti, Claudia Ravaglia, Sara Tomassetti, Carlo Gurioli, Gianluca Casoni, Silvia Asioli, Alessandra Dubini, Sara Piciucchi, Marco Chilosi
European Respiratory Review Dec 2013, 22 (130) 427-436; DOI: 10.1183/09059180.00004313

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Lymphoproliferative lung disorders: clinicopathological aspects
Venerino Poletti, Claudia Ravaglia, Sara Tomassetti, Carlo Gurioli, Gianluca Casoni, Silvia Asioli, Alessandra Dubini, Sara Piciucchi, Marco Chilosi
European Respiratory Review Dec 2013, 22 (130) 427-436; DOI: 10.1183/09059180.00004313
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