Table 1 Clinical presentation of Lymphomatoid Granulomatosis-a review of literature

1. Pulmonary (lung and mediastinal lymph nodes)

-Dyspnea, Cough, Chest pain, Fatigue, Non-productive cough

-Constitutional symptoms

-rarely, asymptomatic

-Underlying Immunodeficiency e.g. AIDS

- may clinically mimic pneumonia or interstitial lung disease [5]

-Chest Radiograph-non specific Differential Diagnosis: Pseudolymphoma, Interstitial Pneumonia, Wegener's Granulomatosis, Sarcoidosis, Metastasis [8]

-High Resolution CT chest-peribronchovascular distribution of nodules and coarse irregular opacities, small thin walled cysts, and conglomerating small nodules [8]

- Gold standard-Histopathology and Immuno-histochemical staining with EBV RNA in situ hybridization.

Progresses to malignant lymphoma in 13-47% cases [3, 8]

Mortality ranges from 53-63.5% [3, 8]

Treatment modalities-combination chemotherapy, Rituximab, Interferon-α2b, Autologous stem cell transplantation [10–13]

2. Central Nervous System

(in 20% cases)

[14, 15]

-Spastic Paraparesis

-Gait disturbances

-Neurogenic Bladder

-Central Diabetes Insipidus

-Peripheral neuropathy

-Concomitant Pulmonary involvement

-Elevated soluble IL-2 receptor level (normal 167-497 U/ml)

-CSF-elevated protein, lymphocytic pleocytosis

-MRI-spotty high intensity lesions on T2 imaging and enhancement with gadolinium contrast

-PET scan-increased uptake of FDG

-Gold standard-Biopsy and immuno-histochemical staining studies, EBV RNA in situ hybridization.

No well established treatment.

CNS involvement is a marker of poor prognosis.

Whole brain irradiation, chemotherapy, stem cell transplantation tried without much efficacy.

Rituximab monotherapy demonstrating efficacy [14].

3. Others-skin, liver, kidney, spleen, mesenteric lymph nodes, etc

-Rash, subcutaneous nodules, ulceration. Usually non tender but occasionally pruritic

-Usually associated with pulmonary or CNS LG

Work up as above

Treatment is along lines of systemic LG.