Lymphomatoid granulomatosis differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Kamal Akbar, M.D.[2]

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		X-ray	CT scan
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 Centers of nodules have large vessels		Predisposing factor immunodeficient 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 Elevated ESR	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 May have edema, lymphocytes, sarcoid-like granulomas.	+ P-ANCA in cells on lung biposy	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 granulomas 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	X-ray	CT scan	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 Airspace consolidation	Liquefactive or coagulative necrosis in lungs Destructive leukocytic angiitis of arteries and veins	Biopsy of the lung	p-ANCA: perinuclear staining, directed against myeloperoxidase, which is usually negative,
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	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 BandC HTLV Lymphomatous appearing B and T cells( condition arises from B and T cells) Abnormal LFT's CBC count: Can be normal in early-stage disease; in more advanced stages, can show: anemia thrombocytopenia leukopenia pancytopenia lymphocytosis thrombocytosis	Mediastinal widening	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 Occasionally associated with monoclonal gammopathy;	Pop corn cells in NLPHL	Symptoms include: enlarged lymph nodes fever Night sweats, weight loss Tiredness