Lymphomatoid Granulomatosis Differential Diagnoses

Updated: Dec 31, 2015
  • Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Zab Mosenifar, MD, FACP, FCCP  more...
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Diagnostic Considerations

The differential diagnosis of the clinical and radiological manifestations of lymphomatoid granulomatosis (LYG) is extensive and beyond the scope of this article. When tissue is available for histology, the following 2 groups of diseases need to be differentiated from lymphomatoid granulomatosis:

  • Other types of pulmonary granulomatosis

    • Bronchocentric granulomatosis and Churg-Strauss (allergic angiitis and granulomatosis) are characterized by asthma and eosinophilia, which are not features of lymphomatoid granulomatosis.

    • Necrotizing sarcoid granulomatosis has nodular pulmonary sarcoid lesions that mimic lymphomatoid granulomatosis. Unlike lymphomatoid granulomatosis, in necrotizing sarcoid, mediastinal adenopathy often occurs; extrapulmonary disease rarely exists; and histology demonstrating well-formed granulomas with central necrosis also exists.

    • WG, unlike angiitis seen in lymphomatoid granulomatosis, is a true vasculitis with acute and chronic inflammatory cells and vessel destruction. Sinus, upper airway, and renal involvement with necrotizing glomerulonephritis are common in WG but rare in lymphomatoid granulomatosis.

  • Other types of malignant lymphoma

    • Hodgkin disease is different because pulmonary involvement without mediastinal adenopathy is rare. The diagnosis requires demonstration of Reed-Sternberg cells.

    • Nasal angiocentric lymphoma (NAL), also known as polymorphic reticulosis or lethal midline granuloma, and lymphomatoid granulomatosis initially were believed to be the same disease, with the former predominantly affecting the upper airway. Recent work has shown that NAL is an EBV-related, natural killer (NK) cell lymphoma and a separate disease entity. Lymphomatoid granulomatosis does not affect the upper airway and nasal passages.

    • Non-Hodgkin lymphoma has well-described pulmonary and extranodal involvement. In particular, peripheral T-cell lymphomas are characterized by vascular infiltration and a degree of morphological heterogeneity. Careful histological diagnosis and studies to determine clonal expansion of T cells are required to rule out lymphomatoid granulomatosis.

Differential Diagnoses