A 48‐year‐old man with a history of hypertension, diabetes, and hepatitis C presented to the ED after being found disorientated. He remembered driving to the grocery store, at which point he became confused, and on his return incurred a minor traffic accident. He “woke up” sitting on the front porch. Recently, he had become frequently forgetful. The patient denied the use of illicit substances. He complained of frequent maroon stools but denied headache, change in vision, chest pain, shortness of breath, nausea, vomiting, and diarrhea. Except for a positive hemoccult, the physical exam was normal. CT scan of the head was negative. Laboratory data revealed an anion‐gap acidosis and protein gap. Ammonia, ethanol, lactic acid, osmolality, and liver function tests were within normal limits. Urine toxicology panel was negative. Protein analysis with serum protein electrophoresis reveled a monoclonal spike in the gamma region measuring 0.7 g/dL. Serum IgM was measured at 900 mg/dL (normal, 40–230 mg/dL). Bone marrow biopsy revealed a lymphoplasmacytic lymphoma. Hematology–oncology was consulted for emergent plasmapheresis. After 2 rounds of plasmapheresis, the patient clinically improved and was discharged to follow up in the hematology–oncology clinic.
Hyperviscosity syndrome occurs in 30% of patients with Waldenstrom's macroglobulinemia. Waldenstrom's macroglobulinemia is a lymphoid neoplasm characterized by a monoclonal lymphoplasmacytic expansion with a serum monoclonal M protein (IgM). The increasing amounts of IgM pentameters bind electrostatically to red blood cells, causing aggregation and rouleaux formation, thereby increasing the serum viscosity. The syndrome usually occurs at IgM concentrations > 3000 mg/dL but can occur at lower concentrations. It is characterized by oronasal bleeding, neurologic symptoms, and mucosal hemorrhage. Neurologic symptoms include headache, dizziness, vertigo, nystagmus, ataxia, vision changes, and stupor. Ophthalmology exam shows retinal vein engorgement, flame hemorrhages, or papilledema. Hyperviscosity syndrome should be suspected in a patient who presents with the triad of neurologic, vision, and bleeding abnormalities. Their serum viscosity and serum immunoglobulin should be measured. If an abnormal monoclonal spike is present, further immuno‐fixation is required.
Emergent therapy includes hydration with diuresis, plasmapheresis, and control of the underlying malignancy. Plasmapheresis dramatically halts the symptoms occurring in hyperviscosity syndrome, as the elevated protein component is removed. Waldenstrom's macroglobulinemia is an incurable but indolent disease. Individuals are closely monitored but once symptomatic require aggressive therapy with chemotherapeutic agents. Death is a result of progressive disease, transformation to lymphoma/leukemia, infection, or the development of myelodysplasia.
M. Shams ‐ none
To cite this abstract:Shams M. Thicker Than Blood. Abstract published at Hospital Medicine 2011, May 10-13, Dallas, Texas. Abstract 391. Journal of Hospital Medicine. 2011; 6 (suppl 2). https://www.shmabstracts.com/abstract/thicker-than-blood/. Accessed January 26, 2020.