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:

• Pulmonary
• CNS
• Dermatologic

Pulmonary Lymphamatoid granulomatosis must be differentiated from other diseases that cause:

• 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:

• 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 and Eosinophilia			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 Some are EBV+, occasionally due to clonal IgH
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; widespread consolidation is discerned occasionally. Enlarged hilar or mediastinal lymph nodes on pulmonary thin-section CT scan		Lung and extrapulmonary sites with eosinophilic infiltrate, Granulomatous reaction and pallisading giant cells near small arteries or arterioles, eosinophilic vasculitis May have fibrin rich 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			chest X-ray shows: nodules, infiltrates or cavities			Liquefactive or coagulative necrosis in lungs with huge number of eosinophils, multinucleated giant cells, as part of poorly formed granulomas, surrounded by pallisading histiocytes and giant cells with central necrosis Destructive leukocytic angiitis of arteries and veins outside of the necrotic granuloma by neutrophils, plasma cells and eosinophils		p-ANCA: perinuclear staining, directed against myeloperoxidase, is usually negative, but positive in microscopic polyarteritis, inflammatory bowel disease, crescentic glomerulonephritis; ELISA test for anti-myeloperoxidase now available
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			mediastinal widening due to grossly enlarged right paratracheal and left paratracheal nodes.	CT scan through the 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		Symptoms include enlarged lymph nodes, fever, night sweats, weight loss, and tiredness