Lymphomatoid granulomatosis differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Lymphamtoid granulomatosis must be differentiated from Bronchocentric granulomatosis and Churg-Strauss, Necrotizing sarcoid granulomatosis, Wegeners granulomatosis, Hodgkins disease, Non-hodgkin lymphoma, and Nasal angiocentric lymphoma

Differentiating Lymphamatoid granulomatosis from other Diseases

As Lymphamatoid granulomatosis manifests in a variety of clinical forms, differentiation must be established in accordance with the particular subtypes pulmonary being the most common. The sub types are the following:[1][2][3]

  • Pulmonary
  • CNS
  • Dermatologic

Pulmonary Lymphamatoid granulomatosis must be differentiated from other diseases that cause:[4][5][6][7]

  • Cough(non-productive, rarely hemoptysis can occur)[8]
  • Dyspnea[9]
  • Chest tightness[10]

Other Symptoms that are asscociated with the pulmonary symptoms are:[10]

  • Malaise
  • Weight loss
  • Fatigue

From the symptoms listed above; Lymphamatoid granulomatosis is usually differtiated from the following diseases Bronchocentric granulomatosis and Churg-Strauss, Necrotizing sarcoid granulomatosis, Wegeners granulomatosis, Hodgkins disease, Non-hodgkin lymphoma, and Nasal angiocentric lymphoma.

In contrast, CNS lymphamatoid granulomatosis must be differentiated from other diseases that cause:

  • Mental status changes
  • Headaches
  • Seizures
  • Hemiparesis
  • Ataxia

The differentials are the following CVA and Brain tumors or CNS lymphoma.

Finally Dermatologic Lymphamatoid granulomatosis must also be differtiated from other diseases that cause:

  • Erythematous rash
  • Macules
  • Papules
  • Plaques
  • Subcutaneous nodules
  • Larger ulcerated nodules

The differentials are the following Dermatomyositis, and Psoriasis

Differentiating Lymphamatoid Granulomatosis

On the basis of Cough, Dyspnea, and Chest tightness, Lymphamatoid granulomatosis must be differentiated from Bronchocentric granulomatosis and Churg-Strauss, Necrotizing sarcoid granulomatosis, Wegeners granulomatosis, Hodgkins disease, Non-hodgkin lymphoma, and Nasal angiocentric lymphoma.

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Imaging Histopathology
Cough Dyspnea Chest tightness Auscultation Lab findings Lab 2 Lab 3 X-ray CT scan Imaging 3
Lymphmatoid granulomatosis + + + Wheezing

Rales

Rhonchi

EBV infused B-cells on blood scan
  • Dense, large, mass like infiltrate and bilateral nodular disease.
Poorly defined nodular peribronchovascular infiltrates with air-bronchograms.
    • Nodular and diffuse lymphoid infiltrates along lymphatics and bronchovascular bundles
    • Centers of nodules have large vessels with lymphatic infiltration
    • Predisposing factor is primary or secondary immunodeficiency states
    • Patients may have fever of unknown origin, hemoptysis, history of multiple skin or other biopsies without diagnosis
Churg-Strauss syndrome + + + Wheezing

Rales

Rhonchi

  • Greater than 50% have positive ANCA, often antimyeloperoxidase
  • Eosinophilia
Pulmonary infiltrates:

Typically, these are transient patchy alveolar infiltrates.

  • Subpleural airspace consolidation
  • Enlarged hilar or mediastinal lymph nodes
  • Lung and extrapulmonary sites with eosinophilic infiltrate,
  • Granulomatous reaction near small arteries, eosinophilic vasculitis
  • May have edema, lymphocytes, sarcoid-like granulomas.
+ P-ANCA
  • Very rare
Necrotizing sarcoid granulomatosis + + - Wheezing Increased levels of ACE in the blood Hilar adenopathy bihilar lymphadenopathy and reticulonodular infiltrates
  • Inflammatory and granulomatous reactions
  • Resembling lymphoma and atypia
  • Dense, noncaseating granulomatous infiltrate
  • Also Schaumann bodies
High levels of ACE in blood
  • Affects skin, lymph nodes and organs
  • Diagnosis of exclusion
  • Patients often have anergy to delayed hypersensitivity tests
Diseases Cough Dyspnea Chest thightness Auscultation Lab findings Lab 2 Lab 3 X-ray CT scan Imaging 3 Histopathology Gold standard Additional findings
Wegeners granulomatosis + + - Wheezing C- ANCA

Urinalysis:

Hematuria

Red cell casts

Biopsy: Granulomatous inflmmation within the arterial wall or in the perivascular area

  • Nodules,
  • Infiltrates or
  • Cavities
Pulmonary nodules with or without cavitation and airspace consolidation
    • Liquefactive or coagulative necrosis in lungs with huge number of eosinophils, and granulomas; surrounded by histiocytes and giant cells with central necrosis
    • Destructive leukocytic angiitis of arteries and veins
  • p-ANCA: perinuclear staining, directed against myeloperoxidase, is usually negative, but positive in microscopic polyarteritis, inflammatory bowel disease, crescentic glomerulonephritis
Hodgkin disease - - -
  • Contains a small number of the characteristic neoplastic cells (Hodgkin and Reed-Sternberg cells or lymphocyte predominant cells) within a background rich in inflammatory cells
  • Parenchymal lung involvement occurs in 1/3 of patients with Hodgkin
  • Almost all have associated hilar or mediastinal adenopathy
Massive mediastinal soft tissue masses consistent with lymphoma
  • Neoplastic cells are Hodgkin and Reed-Sternberg (HRS) cells
  • Effaced lymph node with variable number of HRS cells in a background of inflammatory cells
Reed Sternberg cells Development of Hodgkin's disease may in some patients be preceded by enhanced activation of Epstein–Barr virus
Non-hodgkin lymphoma - - - Can be caused by;

EBV

HIV

Hep C

HTLV

Lymphomatous appearing B and T cells( condition arises from B and T cells)

Mediastinal widening due to grossly enlarged right paratracheal and left paratracheal nodes. Mediastinum can show enlarged tracheobronchial and subcarinal nodes. small bilateral pleural effusion can be seen
  • Sensitivity and specificity of diagnosis with fine needle aspiration increases with use of flow cytometry
Pop corn cells in NLPHL Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tiredness

References

  1. Roschewski M, Wilson WH (2012). "Lymphomatoid granulomatosis". Cancer J. 18 (5): 469–74. doi:10.1097/PPO.0b013e31826c5e19. PMID 23006954.
  2. Fernandez-Alvarez R, Gonzalez M, Fernandez A, Gonzalez-Rodriguez A, Sancho J, Dominguez F; et al. (2014). "Lymphomatoid granulomatosis of central nervous system and lung driven by epstein barr virus proliferation: successful treatment with rituximab-containing chemotherapy". Mediterr J Hematol Infect Dis. 6 (1): e2014017. doi:10.4084/MJHID.2014.017. PMC 3965717. PMID 24678394.
  3. Shaigany S, Weitz NA, Husain S, Geskin L, Grossman ME (2015). "A case of lymphomatoid granulomatosis presenting with cutaneous lesions". JAAD Case Rep. 1 (4): 234–7. doi:10.1016/j.jdcr.2015.05.008. PMC 4808726. PMID 27051739.
  4. Santalla-Martínez M, García-Quiroga H, Navarro-Menéndez I (2015). "Pulmonary lymphomatoid granulomatosis. A rare entity in the differential diagnosis of pulmonary nodules". Arch Bronconeumol. 51 (11): 606–7. doi:10.1016/j.arbres.2015.05.003. PMID 26078192.
  5. Miloslavsky EM, Stone JH, Unizony SH (2015). "Challenging mimickers of primary systemic vasculitis". Rheum Dis Clin North Am. 41 (1): 141–60, ix. doi:10.1016/j.rdc.2014.09.011. PMID 25399945.
  6. Tagliavini E, Rossi G, Valli R, Zanelli M, Cadioli A, Mengoli MC; et al. (2013). "Lymphomatoid granulomatosis: a practical review for pathologists dealing with this rare pulmonary lymphoproliferative process". Pathologica. 105 (4): 111–6. PMID 24466760.
  7. Fauci AS, Haynes BF, Costa J, Katz P, Wolff SM (1982). "Lymphomatoid Granulomatosis. Prospective clinical and therapeutic experience over 10 years". N Engl J Med. 306 (2): 68–74. doi:10.1056/NEJM198201143060203. PMID 7053488.
  8. Xu B, Liu H, Wang B, Zhang H, Wu H, Jin R; et al. (2015). "Fever, Dry Cough and Exertional Dyspnea: Pulmonary Lymphomatoid Granulomatosis Masquerading as Pneumonia, Granulomatosis with Polyangiitis and Infectious Mononucleosis". Intern Med. 54 (23): 3045–9. doi:10.2169/internalmedicine.54.4822. PMID 26631890.
  9. Olusina D, Ezemba N, Nzegwu MA (2011). "Pulmonary Lymphomatoid Granulomatosis: Report of A Case and Review of Literature". Niger Med J. 52 (1): 60–63. PMC 3180752. PMID 21968985.
  10. 10.0 10.1 O'Brien S, Schmidt P (2016). "Lymphomatoid Granulomatosis with Paraneoplastic Polymyositis: A Rare Malignancy with Rare Complication". Case Rep Rheumatol. 2016: 8242597. doi:10.1155/2016/8242597. PMC 4757691. PMID 26966605.

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