Management of Hyperviscosity Syndromes
The hyperviscosity syndromes are a group of hematologic disorders characterized by (among other symptoms) the classic triad of visual changes, mucosal bleeding, and neurologic deficits. They include disorders of WBCs (hyperleukocytosis), platelets (thromb
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Management of Hyperviscosity Syndromes Brian P. O’Connor and Indhu M. Subramanian
Case Presentation A 74 year old man with a history of hypertension, hyperlipidemia, and diabetes mellitus presented with worsening fatigue, blurry vision, headache and shortness of breath over the previous 3 months. In addition, he noted gum bleeding when he brushed his teeth, despite proper oral care. Vitals were remarkable for tachypnea and an oxygen saturation of 82 % which improved to 87 % on 15 L O2 via nonrebreather. Physical exam was notable for 4/5 weakness in his right arm and leg, an enlarged spleen, and Roth spots, cotton wool spots, and tortuous veins (as seen in Fig. 76.1) on fundoscopic exam. His initial set of labs revealed a WBC of 145 thou/ mcL, hemoglobin of 7.4 g/dL (decreased from a baseline of 13 g/dL 2 years prior), a creatinine of 1.3 mg/dL, a total protein of 8.4 g/dL and an albumin of 4.3 g/dL. CXR showed bilateral infiltrates in the lower lung bases (Fig. 76.2). CT of the chest showed no pulmonary emboli, but did
B.P. O’Connor (*) Department of Internal Medicine, Highland Hospital, Alameda Health System, Oakland, CA, USA e-mail: [email protected] I.M. Subramanian Pulmonary and Critical Care Faculty, Department of Internal Medicine, Highland Hospital, Alameda Health System, Oakland, CA, USA
confirm the presence of bilateral lower lobe infiltrates. Noncontrast CT of the brain was notable for age appropriate atrophy without signs of ischemia or hemorrhage. Peripheral blood smear was remarkable for marked blasts and a normocytic anemia. Question What is the most likely diagnosis? Answer Hyperviscosity Hyperleukocytosis
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This patient exhibited the classic triad of mucosal bleeding, visual changes, and focal neurologic deficits seen in hyperviscosity syndromes. The elevated WBC with blasts on the differential suggested the possibility of an underlying hematologic malignancy as the etiology of the patient’s symptoms. In this case, emergent intubation was necessary given the patient’s impending hypoxic respiratory failure. Flow cytometry was performed and the patient was preliminarily diagnosed with acute myelogenous leukemia (AML). Oncology was consulted, who recommended emergent leukapheresis. After the leukapheresis, the patient markedly improved and was subsequently extubated. He later received two more sessions of leukapheresis resulting in complete resolution of his symptoms and a reduction in his WBC count to 33 thou/mcL. As seen in Fig. 76.3, the patient’s CXR also cleared completely after treatment. Bone marrow biopsy
© Springer International Publishing Switzerland 2017 R.C. Hyzy (ed.), Evidence-Based Critical Care, DOI 10.1007/978-3-319-43341-7_76
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Fig. 76.1 Fundoscopic photograph showing intraretinal hemorrhage (blue arrow), tortuous blood vessel (black arrow), cotton wool spots (yellow arrow), and Roth spots (green arrow) (From Shirley and McNicholl [1]. Reprinted with permission from BMJ Publishing Group Ltd.)
B.P. O’Connor and I.M. Subramanian
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