Hematology IgM Gammopathy: Review Questions Jeffrey A. Zonder, MD Dr. Zonder is an assistant professor of medicine, Division of Hematology-Oncology, Karmanos Cancer Institute, Wayne State University School of Medicine, Detroit, MI. Choose the single best answer for each question.
	Questions 1 to 3 refer to the following case. A 60-year-old man presents to his physician for a routine physical examination. Laboratory testing reveals a total serum protein level of 8.7 g/dL (normal, 6.0—8.0 g/dL). Hemoglobin, white blood cell count, platelet count, and serum calcium level are normal. Serum protein electrophoresis with immunofixation confirms the presence of IgM-κ M-protein with a titer of 1.8 g/dL. The patient denies headaches, vision changes, early satiety, weight loss, or numbness/tingling in his fingers or toes. Physical examination reveals no lymphadenopathy or hepatosplenomegaly. 1. What is the next step in this patient’s management? Bone marrow biopsy Close serial monitoring Computed tomography (CT) of the chest, abdomen, and pelvis Initiation of chemotherapy Click here to compare your answer. 2. Three years later, the patient develops normocytic anemia (hemoglobin, 9.2 g/dL), modest adenopathy, and uncomfortable splenomegaly. He has lost 14 lb over the past 5 months. The IgM level has increased to 4.8 g/dL. A bone marrow biopsy confirms 18% involvement by monoclonal lymphoplasmacytic lymphocytes. A direct Coombs’ test is mildly positive. Which of the following is the most appropriate next step in this patient’s management? Chlorambucil or another alkylating agent Combination chemotherapy Continued observation Fludarabine or another purine analogue Prednisone 1 mg/kg/day Click here to compare your answer. 3. The patient responds to initial therapy, but lymphadenopathy progresses 25 months later. The IgM level increases to 6.2 g/dL. Treatment with rituximab, an anti-CD20 antibody, is initiated. Four days after receiving therapy, the patient returns complaining of headache and blurry vision. Eye examination confirms the presence of small bilateral retinal hemorrhages. What is the most appropriate next step? Initiate purine analogue therapy Ophthalmology referral for retinal laser treatments Urgent CT scan of the brain Urgent initiation of plasmapheresis Click here to compare your answer.		4. A 77-year-old nonsmoking woman develops new-onset Raynaud’s phenomenon. A thorough evaluation reveals no palpable peripheral lymphadenopathy or hepatosplenomegaly, mild anemia (hemoglobin, 10.1 g/dL), and a normal platelet count. Antinuclear antibody (ANA), rheumatoid factor (RF), and Coombs’ tests are negative. A viral hepatitis panel is negative. Serum protein electrophoresis with immunofixation reveals a normal total serum protein level and a faint monoclonal IgM band (titer, 0.4 g/dL). What is this patient’s most likely diagnosis? Hyperviscosity syndrome Mixed connective tissue disease Thromboangiitis obliterans (Buerger’s disease) Type I cryoglobulinemia Type II (mixed) cryoglobulinemia Click here to compare your answer. 5. A 47-year-old woman presents with symmetrical numbness and tingling in her hands and feet, which has progressed over the last year. The patient works full-time as a dental hygienist, and her symptoms have led to difficulties at work. She denies associated pain or weakness and has not noticed unusual bruising or bleeding, rash, or back pain. There has been no change in her appetite or weight. Physical examination reveals subjective soft touch deficits in a stocking glove distribution, which are most severe distally. Strength and reflexes are normal. Her tongue, optic fundi, heart, and lungs are normal, and there is no peripheral adenopathy or organomegaly. Laboratory testing reveals a normal complete blood count, normal renal and liver function, a normal erythrocyte sedimentation rate, and a monoclonal IgM-κ level of 1.1 g/dL with normal levels of IgG and IgA. ANA and Coombs’ tests are negative. A hepatitis panel and HIV test are negative. Radiographs of the spine do not reveal disc space narrowing or lytic or sclerotic lesions. What is the cause of this patient’s symptoms? Anti-myelin-associated glycoprotein (anti-MAG) antibodies Guillain-Barré syndrome Myeloma-associated amyloidosis POEMS syndrome Waldenström’s macroglobulinemia (WM) Click here to compare your answer.
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