Waldenström's macroglobulinemia overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]
Overview
Waldenström macroglobulinemia is a cancer involving subtype of white blood cells called lymphocytes. The main attributing antibody is IgM. It is a type of lymphoproliferative disease, and shares clinical characteristics with the indolent non-Hodgkin lymphomas.[1]
Historical Perspective
Waldenström's macroglobulinemia was first discovered by Jan G. Waldenström, a Swedish physician in 1944.[2]
Classification
Waldenström's macroglobulinemia is classified as a subtypes of Non-Hodgkin Lymphoma according to the new World Health Organization and the Revised European American Lymphoma (REAL) classifications.[2][3]
Pathophysiology
Waldenström Macroglobulinemia is uncontrolled clonal proliferation of terminally differentiated B lymphocytes, which are normally involved in humoral immunity.[2] Genes involved in pathogenesis of Waldenström Macroglobulinemia include MYD88-L265P, CXCR4 and chromosomes 6q, 13q, 3q, 6p and 18q.[4] The progression to Waldenström Macroglobulinemia usually involves the MYD88/IRAK, PI3K/Akt/mTOR molecular pathway. [5]
Causes
Common causes of Waldenström's macroglobulinemia include genetic, environmental, and autoimmune factors.[6][7]
Differentiating Waldenström's macroglobulinemia from other B cell lymphoid neoplasms
Waldenström's macroglobulinemia must be differentiated from Multiple myeloma, Chronic lymphocytic leukemia/small lymphocytic lymphoma, B-cell prolymphocytic leukemia, Follicular lymphoma, Mantle cell lymphoma, and Marginal zone lymphoma. [4]
Epidemiology and Demographics
The prevalence of Waldenström's macroglobulinemia is estimated to be 1500 cases in United States annually. Waldenström's macroglobulinemia represents 1% of all hematological cancers.[6]
Risk factors
Common risk factors in the development of Waldenström's macroglobulinemia are Monoclonal gammopathy of undetermined significance, age >50 year old, white ethnicity, hereditary, hepatitis C, and immune disorders.[8]
Screening
According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for Waldenström's macroglobulinemia.[9]
Natural History, Complications and Prognosis
If left untreated, patients with asymptomatic Waldenström's macroglobulinemia may progress to develop symptomatic disease. Common complications of Waldenström's macroglobulinemia include hyperviscosity syndrome, cold haemagglutinin disease, cryoglobulinemia, peripheral neuropathy, venous thromboembolism, primary amyloidosis, heart failure, malabsorbtive diarrhea, and bleeding manifestations. Prognosis varies depending on the prognostic factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease. [10]
History and symptoms
The hallmark of Waldenström's macroglobulinemia is hyperviscosity syndrome. A positive history of oronasal bleeding and peripheral neuropathy is suggestive of Waldenström's macroglobulinemia.[11]
Physical Examination
Patients with Waldenström's macroglobulinemia usually appear oriented to time, place, and person. Physical examination of patients with Waldenström's macroglobulinemia is usually remarkable for various findings depending on degree of tissue infiltration by malignant tumor cells, hyperviscosity syndrome, and accumulation of paraprotein.[12]
Laboratory Findings
Elevated Beta-2-microglobulin and abnormal coagulation studies are diagnostic of Waldenström's macroglobulinemia. [13]
Bone marrow aspiration and biopsy
Bone marrow aspirate is frequently hypocellular in Waldenström's macroglobulinemia. Biopsy specimen is usually hypercellular and infiltrated with lymphoid and plasmacytoid cells. Dutcher bodies (PAS positive Intranuclear vacuoles containing IgM monoclonal protein) are characteristic feature of Waldenström's macroglobulinemia.[14]
Electrophoresis and Immunofixation
In Waldenström's macroglobulinemia, Serum and urine protein electrophoresis is used as the screening test, while Serum and urine immunofixation is used as the confirmatory test.[14]
Chest x-ray
On chest x-ray, Waldenström's macroglobulinemia may be characterized by pulmonary infiltrates, nodules, effusion, and congestive heart failure.[13]
CT scan
CT scan imaging of chest, abdomen, and pelvis may show evidence of adenopathy, and hepatomegaly.[13]
MRI
MRI of the spine may show bone marrow involvement. [13]
Other Diagnostic Studies
Patients with Waldenström's macroglobulinemia should have nerve conduction study and antimyelin associated glycoprotein serology done.[14]
Medical Therapy
Risk stratification determines the protocol of management used for Waldenström's macroglobulinemia patients. Watchful waiting is recommended for asymptomatic Waldenström's macroglobulinemia. Symptomatic Waldenström's macroglobulinemia is treated with Rituximab +/- Chemotherapy.[15]
References
- ↑ Cheson BD (2006). "Chronic Lymphoid Leukemias and Plasma Cell Disorders". In Dale DD, Federman DD. ACP Medicine. New York, NY: WebMD Professional Publishing. ISBN 0974832715.
- ↑ 2.0 2.1 2.2 Waldenström's macroglobulinemia. Wikipedia (2015)https://en.wikipedia.org/wiki/Waldenström%27s_macroglobulinemia#Pathophysiology Accessed on November 6, 2015
- ↑ Waldenström's macroglobulinemia. International Waldenström's macroglobulinemia Foundation (2015)http://www.iwmf.com/sites/default/files/docs/WM_Review_Ghobrial_Jan2014.pdf Accessed on November 9, 2015
- ↑ 4.0 4.1 Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=see_link Accessed on November 9, 2015
- ↑ Waldenström macroglobulinemia. International Waldenström Macroglobulinemia foundation (2015)http://www.iwmf.com/sites/default/files/docs/WM_Review_Ghobrial_Jan2014.pdf Accessed on November 12, 2015
- ↑ 6.0 6.1 Waldenström's macroglobulinemia. Wikipedia (2015). https://en.wikipedia.org/wiki/Waldenström%27s_macroglobulinemia#cite_note-19 Accessed on November 6, 2015
- ↑ Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia. UpToDate (2015). http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia#H3 Accessed on November 6, 2015
- ↑ Waldenström's macroglobulinemia. American Cancer Society (2015)http://www.cancer.org/cancer/waldenstrommacroglobulinemia/detailedguide/waldenstrom-macroglobulinemia-risk-factors Accessed on November 6, 2015
- ↑ Recommendations. US preventive services task force(2015) http://www.uspreventiveservicestaskforce.org/BrowseRec/Search?s=medulloblastoma Accessed on November 10, 2015
- ↑ Waldenström's macroglobulinemia. Patient (2015)http://patient.info/doctor/waldenstroms-macroglobulinaemia-pro Accessed on November 10, 2015
- ↑ Waldenström's macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=machineLearning&search=Waldenström%27s+macroglobulinemia&selectedTitle=1%7E80§ionRank=3&anchor=H29#H6 Accessed on November 10, 2015
- ↑ Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-overview#a3 Accessed on November 10, 2015
- ↑ 13.0 13.1 13.2 13.3 Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/207097-workup#c7 Accessed on November 10, 2015
- ↑ 14.0 14.1 14.2 Waldenström's macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=machineLearning&search=Waldenström%27s+macroglobulinemia&selectedTitle=1%7E80§ionRank=3&anchor=H29#H18 Accessed on November 10, 2015
- ↑ Waldenström's macroglobulinemia. Medscape (2015)http://emedicine.medscape.com/article/2057687-overview Accessed on November 11, 2015