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Washington University Experience | NEOPLASMS (HEMATOLYMPHOID) | Lymphomatoid Granulomatosis | 2A0 Case 2 History
Case 2 History (Bilbao & Schmidt Biopsy Diagnosis of Peripheral Neuropathy, 2015, page 266) ---- A 30 year old nickel worker experienced painful paresthesias involving the forearms, progressing over 3-4 months to affect almost the entire body multifocally. A multifocal macular rash affected his forearms most severely. Physical examination did not show pulmonary disease, organomegaly or lymphadenopathy. Marked atrophy and weakness were seen in the ulnar nerve distribution, right>left. There was stocking-glove distribution of light touch and pinprick deficit. ---- Electrophysiological testing showed a multifocal axonal neuropathy superimposed on a mild diffuse axonal sensorimotor neuropathy. CSF examination revealed a normal cell count. ESR, ANA, RF, C3, C4, ANCA, immunoelectrophoresis, cryoglobulins, bone marrow biopsy, CXR and abdominal ultrasound were also normal. Sural nerve biopsy was suggestive of angiocentric lymphoma or lymphomatoid granulomatosis which was favored as the latter by the presence of angiodestruction despite the absence of lung findings. Cyclophosphamide and prednisone resulted in dramatic improvement of sensory symptoms within a month and significant recovery of clinical and electrophysiologic evidence in his ulnar nerves over several years. An additional nerve biopsy performed one year after the first showed only nonspecific chronic degenerative changes without cellular infiltration.
