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      Drugs & Diseases > Hematology

      Waldenstrom Macroglobulinemia Clinical Presentation

      Updated: Feb 03, 2022
      • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD  more...
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      Presentation

      History

      Onset is insidious and nonspecific. Many patients are asymptomatic at presentation and are diagnosed incidentally from routine blood work. Weakness, anorexia and weight loss are the most common symptoms. Merlini et al reported the frequency of presenting features in 215 patients with Waldenström macroglobulinemia, as follows [22] :

      • Weakness - 66%
      • Anorexia - 25%
      • Peripheral neuropathy - 24%
      • Weight loss - 17%
      • Fever - 15%
      • Raynaud phenomenon - 11%; Raynaud phenomenon is due to cryoglobulinemia and may precede other serious symptoms by several years

      Mental status changes, including lethargy, stupor, or even coma, can occur. Infiltration of the central nervous system by the malignant clone can cause a syndrome of confusion, memory loss, disorientation, and motor abnormalities called the Bing-Neel syndrome.

      Symptoms due to hyperviscosity syndrome, which can be life threatening, include the following [23] :

      • Bleeding
      • Dizziness
      • Headache
      • Blurry vision
      • Hearing or visual problems

      Visual changes, such as blurred vision or double images, and spontaneous bleeding with minor trauma can be presenting features. Patients often present with a history of abnormal bleeding. Gastrointestinal (GI) findings may include malabsorption, GI bleeding, and diarrhea.

      Next:

      Physical Examination

      The physical findings result from tissue infiltration by the malignant clone, the hyperviscosity state caused by antigen-antibody reactions triggered by the paraprotein, and derangement of the hemostatic system by the paraprotein. Hepatosplenomegaly and lymphadenopathy are common. Merlini et al [22] reported the following occurrences of symptoms in 215 patients evaluated for Waldenström macroglobulinemia:

      • Hepatomegaly - 20%

      • Splenomegaly - 19%

      • Lymphadenopathy - 15%

      • Purpura - 9%

      • Hemorrhagic manifestations - 7%

      Papilledema, ie, sausage-shaped (distended and tortuous) retinal veins, and hemorrhages may be evident on funduscopic examination.

      Neuropathy is typically slowly progressive, distal, symmetrical, and sensorimotor. Other variants, including a chronic ataxic neuropathy known as Miller-Fisher syndrome (a variant of Guillain-Barré syndrome), have been described. POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) also may be associated with Waldenström macroglobulinemia.

      Skin manifestations include the following:

      • Purpura (see the image below)

      • Bullous skin disease

      • Papules on extremities

      • Cutaneous plaques and nodules

      • Chronic urticaria (Schnitzler syndrome)

      • Raynaud phenomenon

      • Livedo reticularis

      • Acrocyanosis

      Purpura from Waldenström macroglobulinemia is evid Purpura from Waldenström macroglobulinemia is evident in the forearm of a 65-year-old man who presented with a purpuric rash on all of his extremities. Although the patient had a history of hepatitis C, the possibility of hepatitis C cryoglobulinemia was excluded because the rash extended well beyond the hands and feet, and blood testing identified a type I cryoglobulinemia. Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD, Division of Rheumatology, University of Colorado Denver School of Medicine.

      Pulmonary involvement is rare (3-5%), with nodules, masses, parenchymal infiltrates, or pleural effusion.

      Congestive heart failure is an unusual manifestation, presenting with jugular venous distention, displaced apical impulse, S3 gallop, rales at lung auscultation, and peripheral edema.

      Periorbital masses resulting from infiltration into retro-orbital structures and the lacrimal gland have been described. This can cause proptosis and ocular nerve palsies. Osseous lesions and amyloidosis are rare.

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