A 60-year-old woman comes to the emergency department due to 2 weeks of worsening bilateral headaches, blurry vision, dizziness, and tingling sensation in her feet. She has had no fevers, night sweats, unintentional weight loss, or jaw pain but has had several episodes of epistaxis. The patient has a history of hypertension and takes chlorthalidone. She does not use tobacco, alcohol, or illicit drugs.
Temperature is 37.2 C (99.0 F), blood pressure is 166/98 mm Hg, pulse is 85/min, and respirations are 14/min. Physical examination shows moist mucous membranes, normal tympanic membranes, and no sinus tenderness. Bilateral pupils are equal and reactive. Visual acuity is decreased in both eyes, and funduscopic examination reveals dilated, segmented, and tortuous retinal veins. Facial sensation and strength are normal. Bilateral upper- and lower-extremity muscle strength is normal. There is decreased pinprick sensation and loss of ankle reflex bilaterally.
Laboratory results are as follows:
Which of the following is the most appropriate next step in management of this patient?

Question

A 60-year-old woman comes to the emergency department due to 2 weeks of worsening bilateral headaches, blurry vision, dizziness, and tingling sensation in her feet.  She has had no fevers, night sweats, unintentional weight loss, or jaw pain but has had several episodes of epistaxis.  The patient has a history of hypertension and takes chlorthalidone.  She does not use tobacco, alcohol, or illicit drugs.
Temperature is 37.2 C (99.0 F), blood pressure is 166/98 mm Hg, pulse is 85/min, and respirations are 14/min.  Physical examination shows moist mucous membranes, normal tympanic membranes, and no sinus tenderness.  Bilateral pupils are equal and reactive.  Visual acuity is decreased in both eyes, and funduscopic examination reveals dilated, segmented, and tortuous retinal veins.  Facial sensation and strength are normal.  Bilateral upper- and lower-extremity muscle strength is normal.  There is decreased pinprick sensation and loss of ankle reflex bilaterally.
Laboratory results are as follows:
 Which of the following is the most appropriate next step in management of this patient?

Quizplus · Accepted Answer

This patient with an elevated gamma gap (total protein − albumin > 4 g/dL) has several clinical features concerning for Waldenström macroglobulinemia (WM), a rare lymphoplasmacytic lymphoma marked by excessive circulating monoclonal IgM. Patients often present with constitutional symptoms, oozing from mucosal sites, and manifestations related to: The infiltration of hematopoietic tissue with neoplastic cells, leading to hepatosplenomegaly, lymphadenopathy, and cytopenias (particularly anemia)Excessive IgM in the blood, leading to cryoglobulinemia, sensorimotor peripheral neuropathy, and hyperviscosity syndrome (blurred vision, headache, vertigo) The infiltration of hematopoietic tissue with neoplastic cells, leading to hepatosplenomegaly, lymphadenopathy, and cytopenias (particularly anemia) The infiltration of hematopoietic tissue with neoplastic cells, leading to hepatosplenomegaly, lymphadenopathy, and cytopenias (particularly anemia) Excessive IgM in the blood, leading to cryoglobulinemia, sensorimotor peripheral neuropathy, and hyperviscosity syndrome (blurred vision, headache, vertigo) Excessive IgM in the blood, leading to cryoglobulinemia, sensorimotor peripheral neuropathy, and hyperviscosity syndrome (blurred vision, headache, vertigo) Other common findings include "sausage link" retinal veins, markedly elevated erythrocyte sedimentation rate, and rouleaux formation on peripheral blood smear. Patients with suspected WM should undergo screening with serum protein electrophoresis. The presence of monoclonal IgM gammopathy supports the diagnosis; confirmation requires bone marrow aspiration and biopsy (≥10% plasmacytoid or plasma cell infiltration with characteristic immunophenotype patterns). plasma cell infiltration WM is not curable with current therapies, so treatment is geared toward controlling symptoms and limiting end-organ involvement. Those with hyperviscosity syndrome are treated with emergent plasmapheresis to reduce circulating IgM. Median survival is approximately 10 years. (Choice A) Carbonic anhydrase inhibitors and loop diuretics are used in the treatment of idiopathic intracranial hypertension, which often presents with headache and transient visual symptoms. However, funduscopic examination almost always reveals papilledema. In addition, a gamma gap and signs of peripheral neuropathy are not features of this disease. (Choices B and E) High-dose glucocorticoids are used to treat giant cell arteritis, which can present with constitutional symptoms, headache, transient vision loss, and elevated erythrocyte sedimentation rate. However, vision loss is usually monocular, and funduscopic examination is typically normal (or shows signs of ischemia). Temporal artery biopsy aids in diagnosis. (Choice C) Patients with hypertensive crisis can develop headache and encephalopathy, but most cases occur when blood pressure is severely elevated (>180/120 mm Hg), not moderately elevated. In addition, this patient's gamma gap and "sausage link" retinal veins make WM far more likely than hypertensive crisis. Educational objective: Waldenström macroglobulinemia often presents with constitutional symptoms, oozing from mucosal sites, and manifestations related to neoplastic infiltration of hematopoietic tissue (eg, hepatosplenomegaly, lymphadenopathy, cytopenias) or high levels of IgM (eg, cryoglobulinemia, peripheral neuropathy, hyperviscosity syndrome). Diagnosis is made with serum protein electrophoresis and bone marrow biopsy. __________

A 60-Year-Old Woman Comes to the Emergency Department Due to 2