eMedicine Specialties > Hematology > Plasma Cell Disorders

Waldenstrom Hypergammaglobulinemia: Follow-up

Author: Doris Ponce, MD, Fellow, Department of Hematology/Oncology, New York Medical College
Coauthor(s): Karen Seiter, MD, Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College; Vijay Ramu, MBBS, Staff Physician, Department of Internal Medicine, East Tennessee State University; Harsha Vyas, MD, Fellow, Section of Hematology and Oncology, Wake Forest University School of Medicine; Koyamangalath Krishnan, MD, FRCP, FACP, Paul Dishner Endowed Chair of Excellence in Medicine, Professor of Medicine and Chief of Hematology-Oncology, Program Director, Hematology-Oncology Fellowship, James H Quillen College of Medicine at East Tennessee State University
Contributor Information and Disclosures

Updated: Aug 29, 2009

Follow-up

Further Inpatient Care

  • Except for patients requiring emergency treatment of hyperviscosity syndrome, most patients can be treated as outpatients.

Further Outpatient Care

  • Periodic physical examinations for organomegaly, routine chemistry evaluations, serum paraprotein level, serum viscosity, and coagulation tests should be performed to monitor for progression and to aid in treatment decisions.

Transfer

  • Patients requiring emergency plasmapheresis should be transferred to a center that offers this therapy.

Complications

  • Hyperviscosity syndrome
  • Visual disturbances secondary to hyperviscosity syndrome
  • Diarrhea and malabsorption secondary to GI involvement
  • Renal disease (less common)
  • Amyloidosis of the heart, kidney, liver, lungs, and joints
  • Bleeding manifestations secondary to platelet dysfunction and coagulation factor and fibrinogen abnormalities due to interaction with plasma IgM
  • Raynaud phenomenon secondary to cryoglobulinemia
  • Increased predisposition to infection due to B-cell dysfunction (disease-related) or T-cell dysfunction (therapy-related, particularly after nucleoside analogues)
  • Cardiac failure
  • Increased incidence of lymphomas, myelodysplasia, and leukemias

Prognosis

  • Waldenström macroglobulinemia is an indolent disorder, and patients survive for a median of approximately 78 months. Different studies have been performed to assess prognosis, as follows:
    • A median survival of 7.7 years was noted in patients when their macroglobulin level was reduced by at least 75%.
    • Patients with a nodular type of bone marrow involvement tend to do better than those with diffuse involvement.
  • Poor prognostic factors include (1) age older than 65 years, (2) hemoglobin value of less than 10 g/dL, (3) albumin level less than 4.0 g/dL, and (4) elevated beta-2-microglobulin level.

Miscellaneous

Medicolegal Pitfalls

  • The manifestations of Waldenström macroglobulinemia are protean. Considering the diagnosis of Waldenström macroglobulinemia in patients who present with unexplained fatigue and weakness, neurological symptoms, unexplained bleeding, visual blurring, and neuropathies is important. This is especially important because hyperviscosity symptoms can be life threatening. Therefore, considering the diagnosis and performing protein electrophoresis, immunoglobulin quantitation, and hyperviscosity measurements are critical.
 


More on Waldenstrom Hypergammaglobulinemia

Overview: Waldenstrom Hypergammaglobulinemia
Differential Diagnoses & Workup: Waldenstrom Hypergammaglobulinemia
Treatment & Medication: Waldenstrom Hypergammaglobulinemia
Follow-up: Waldenstrom Hypergammaglobulinemia
References
Further Reading
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Keywords

Waldenström hypergammaglobulinemia, macroglobulinemia, Waldenström's hypergammaglobulinemia, malignant lymphoproliferative disease, monoclonal gammopathy, malignant monoclonal gammopathies, Waldenström macroglobulinemia, Waldenström's macroglobulinemia, Waldenstrom macroglobulinemia, WM,

lymphoproliferative disorder, clonal disorder, B-lymphocyte disorder, blood malignancy, hematologic malignancy, blood cell cancer, plasmacytoid lymphocytic lymphoma, lymphoplasmacytoid lymphoma, primary macroglobulinemia, plasma cell neoplasms hemostatic disorders, paraproteinemias

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Contributor Information and Disclosures

Author

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.

Coauthor(s)

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; Schering Honoraria Speaking and teaching; Cephalon Honoraria Speaking and teaching

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.

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
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, Program Director, Hematology-Oncology Fellowship, 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.

Medical Editor

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

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: eMedicine Salary Employment

Managing Editor

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.

CME Editor

Rajalaxmi McKenna, MD, FACP, Consulting Staff, Department of Medicine, Southwest Medical Consultants, SC, Good Samaritan Hospital, Advocate Health Systems
Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis
Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD, Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, 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 Hematology, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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