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:

• Cough(non-productive, rarely hemoptysis can occur)
• Dyspnea
• Chest tightness

Other Symptoms that are asscociated with the pulmonary symptoms are:

• 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:

• Headaches
• Seizures
• Hemiparesis
• Ataxia

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

Finally Cutaneous 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