Chest
Volume 114, Issue 4, October 1998, Pages 1205-1209
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Pulmonary and Critical Care Pearls
A 48-Year-Old Woman With Peripheral Neuropathy, Hypercalcemia, and Pulmonary Infiltrates

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Physical Examination

She was a lethargic, confused woman who was afebrile with a pulse of 100 beats/min; respirations, 20 breaths/min; and BP, 95/75 mm Hg. She had orthostatic changes in her BP and pulse from the recumbent to upright position. Crackles were heard in her mid and lower lung zones bilaterally. The abdominal examination disclosed no abnormalities. Neurologic examination was limited by the patient's compromised mental status but was grossly nonfocal. She had no peripheral clubbing, cyanosis, or edema.

Laboratory Findings

Values were as follows: sodium, 135 mmol/L; potassium, 5.0 mmol/L; chloride, 80 mmol/L; CO2, 41 mmol/L; BUN, 90 mg/dL; creatinine, 9.0 mg/dL; glucose, 247 mg/dL; and calcium, 15.6 mg/dL. The WBC was 9.5/mm3; hematocrit, 44.6%; and platelet count, 327,000/mm3. A chest x-ray film is shown in Figures 1 and 2. A 25-hydroxy vitamin D level (25-[OH]-D) was 5 ng/mL (normal, 16 to 74 ng/mL), 1,25 dihydroxy vitamin D (l,25-[OH]2-D) was 24 pg/mL (normal, 18 to 62 pg/mL), and the parathyroid hormone level

Hospital Course

The patient was admitted to the hospital and vigorously rehydrated with intravenous fluids. With rehydration, her abdominal symptoms resolved, mental status improved, and her serum calcium level and renal function normalized. Repeated levels of l,25-(OH)2-D and 25-(OH)-D (determined from blood drawn after renal failure resolved) were 31 pg/mL and <5 ng/mL, respectively.

What unifying diagnosis explains the patient's clinical presentation?

How can the diagnosis be confirmed?

Diagnosis: Angiocentric lymphoma (lymphomatoid granulomatosis)

Angiocentric lymphoma was first termed lymphomatoid granulomatosis in 1972 by Liebow, who described a triad of a polymorphic lymphoid infiltrate, angiitis, and granulomatosis. The most distinctive feature is the multicellular lymphoid infiltrate, which is composed of small lymphocytes, plasma cells, and large, atypical, mononuclear cells. The term angiitis was used to describe the transmural infiltration of lymphoid cells into the walls of arteries and veins, and the term granulomatosis was

CLINICAL PEARLS

  • 1.

    Angiocentric lymphoma (lymphomatoid granulomatosis) is a multisystem disease which often features fever, malaise, weight loss, pulmonary infiltrates, skin lesions, and renal and neurologic (both peripheral and central) involvement. It may involve any organ.

  • 2.

    Angiocentric lymphoma is an aggressive malignant neoplasm and in most cases should be treated with intensive chemotherapy.

  • 3.

    Angiocentric lymphoma, like other lymphomas, may be associated with hypercalcemia. The hypercalcemia is mediated by the

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