Waldenstrom Macroglobulinemia Clinical Presentation

  • Author: Karen Seiter, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 31, 2012
 

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:

  • 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 CNS 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[8] :

  • 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. GI system findings may include malabsorption, GI bleeding, and diarrhea.

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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[9] 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
  • Bullous skin disease
  • Papules on extremities
  • Cutaneous plaques and nodules
  • Chronic urticaria (Schnitzler syndrome)
  • Raynaud phenomenon
  • Livedo reticularis
  • Acrocyanosis

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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Contributor Information and Disclosures
Author

Karen Seiter, MD  Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology

Disclosure: Novartis Honoraria Speaking and teaching; Novartis Consulting fee Speaking and teaching; Eisai Honoraria Speaking and teaching; Celgene Honoraria Speaking and teaching

Coauthor(s)

Doris Ponce, MD  Fellow, Department of Hematology/Oncology, New York Medical College

Doris Ponce, MD is a member of the following medical societies: American College of Physicians, American Medical Association, American Society of Clinical Oncology, and American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Wendy Hu, MD Consulting Staff, Department of Hematology/Oncology and Bone Marrow Transplantation, Huntington Memorial Medical Center

Wendy Hu, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Blood and Marrow Transplantation, American Society of Hematology, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Koyamangalath Krishnan, MD, FRCP, FACP Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, James H Quillen College of Medicine at East Tennessee State University

Koyamangalath Krishnan, MD, FRCP, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, and Royal College of Physicians

Disclosure: Nothing to disclose.

Vijay Ramu, MBBS Staff Physician, Department of Internal Medicine, East Tennessee State University

Vijay Ramu, MBBS is a member of the following medical societies: American College of Physicians

Disclosure: Nothing to disclose.

Paul Schick, MD Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Harsha Vyas, MD Fellow, Section of Hematology and Oncology, Wake Forest University School of Medicine

Harsha Vyas, MD is a member of the following medical societies: American College of Physicians, American Society of Clinical Oncology, and American Society of Hematology

Disclosure: Nothing to disclose.

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