Hyperviscosity Syndrome Provoking Heart Failure in a Patient with Waldenström macroglobinemia

Abstract

We present a 75-year-old woman with no medical follow up for several years who developed progressively worsening shortness of breath with associated orthopnea and weight loss over the past year. Additionally, she reported leg heaviness, abdominal distention, subjective fevers, dry cough and a poor appetite. She denied any drenching night sweats. Her past medical history was notable solely for chronic anemia and alcohol dependence. She had not been taking any medications prior to admission. Her family history was significant for a mother with anemia. The patient was an active smoker, with a 35-40 pack-year smoking history.On arrival to the emergency department, patient was hypoxic, prompting administration of supplemental oxygen via nasal cannula. She was afebrile and hemodynamicallystable. On exam, she was cachectic and fatigued with hepatomegaly and without palpable lymphadenopathy. Labs were drawn, which showed a thrombocytopenia of 58 th/mm 3 and hemoglobin of 5g/dL. Chest x-ray showed a moderate right-sided pleural effusion with a possible pneumonia or atelectasis. An echocardiogram showed an ejection fraction of 55% with pulmonary artery pressure of 38mmHg suggestive of mild pulmonary hypertension. Brain natriuretic peptide (BNP) was elevated to 1840pg/mL. The patient was initially treated for community-acquired pneumonia and given intravenous (IV) furosemide. Computed tomography (CT) of the chest revealed a moderately-sized, partially loculated right pleural effusion with resulting complete middle lobe and multisegment right lower lobe atelectasis. Given these findings, the patient underwent a thoracentesis with removal of 1.2 liters. InvestigationFluid analysis revealed a pH 7.52, albumin 1.3 g/dL, glucose 112 mg/dL, amylase 52 IU/L, protein 3.7 g/dL, lactate dehydrogenase (LDH) 107 U/L. Serum LDH 187 U/L, serum protein 8.8 g/dL. Given these findings, the patient's effusion was characterized as transudative in nature with negative cytology. The patient underwent a CT of the abdomen and pelvis due to her worsening abdominal distension which revealed small-volume ascites, hepatomegaly, measuring 19 cm in length, splenomegaly, measuring 15 cm in length with a large confluent retroperitoneal mass at the root of the mesentery extending from the celiac access to at least the level of the aortic bifurcation with extension along the common iliac chains and external iliac chains. Much of the mass had enveloped the mesenteric arteries and solitary renal arteries bilaterally as well as the ureters. Her serum protein electrophoresis (SPEP) was notable for an M-spike of 4.3 g/dL, an elevated kappa/lamda light chain ratio of 17.7 and serum viscosity was elevated at 3.9 cP.The degree of lymphadenopathy was concerning for a lymphomatous process. Fine needle aspiration (FNA) of the axillary lymph node was consistent with B-cell lymphoproliferative disorder, showing a monotonous population of small lymphocytes with round, ovoid nuclei, condensed chromatin and scant cytoplasm. Occasional plasmacytoid cells were identified. ...