Waldenstrom macroglobulinemia
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Waldenstrom macroglobulinemia
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Waldenstrom macroglobulinemia
Hematological system pathology review
Microcytic anemia: Pathology review
Non-hemolytic normocytic anemia: Pathology review
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Waldenstrom macroglobulinemia
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Robyn Hughes, MScBMC
Alex Aranda
Tanner Marshall, MS
Waldenstrom Macroglobulinemia, or Waldenstrom syndrome, is a rare type of malignant lymphoma.
It is characterized by the overproduction of an antibody called immunoglobulin M, or IgM, in the blood.
Like all antibodies, IgM is produced by a subpopulation of white blood cells called B lymphocytes, or B-cells for short. B-cells start off as stem cells within the bone marrow, where they undergo a series of developmental phases.
When they’re mature enough, they leave the bone bone marrow and enter the blood, and from there, they go into secondary lymph organs such as the spleen and lymph nodes.
Within the secondary lymph organs,these B-cells can then further differentiate into plasma cells which ultimately give rise to the immunoglobulins.
Thus, Waldenstrom’s macroglobulinemia is more specifically referred to as a lymphoplasmacytic lymphoma.
Now, IgM is actually composed of 5 separate antibodies bound together, thus creating a pentamer, which is a very large, or MACRO-globulin.
These macroglobulins increase the thickness of blood and aggregate with one another causing a condition known as hyperviscosity syndrome.
Hyperviscosity causes blood vessels to become engorged, causes hypercoagulability, and reduces the overall rate of blood flow.
Like with many cancers, the exact cause of this overproduction is not well understood but it is hypothesized to be due to mutations in the MYD88 and CXCR4 genes.
When mutated, these genes allow lymphoplasmacytic cells to avoid programmed cell death, so they end up proliferating excessively.
The overproduction of lymphoplasmacytic cells in the bone marrow can also crowd out and interfere with the production of normal red blood cells and platelets.
Although commonly asymptomatic, the symptoms that do occur are mainly attributed to hyperviscosity syndrome, which leads to mucosal bleeding from the nose and gums.
Summary
Waldenstrom Macroglobulinemia, or Waldenstrom syndrome, is a rare type of malignant lymphoma that's characterized by the overproduction of an antibody called immunoglobulin M, or IgM, in the blood, which can lead to hyperviscosity syndrome and a collection of symptoms including bleeding, visual changes, headaches, and weakness.
Diagnosis is typically made through serum electrophoresis and immunofixation, which can identify elevated levels of immunoglobulin M. Treatment is mainly done through the removal of these large immunoglobulin M proteins via plasmapheresis, but chemotherapeutic agents may also be used. Stem cell transplantation may also be considered in certain cases.
Sources
- "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
- "CURRENT Medical Diagnosis and Treatment 2020" McGraw-Hill Education / Medical (2019)
- "Yen & Jaffe's Reproductive Endocrinology" Saunders W.B. (2018)
- "Bates' Guide to Physical Examination and History Taking" LWW (2016)
- "Robbins Basic Pathology" Elsevier (2017)
- "CXCR4 mutations affect presentation and outcomes in patients with Waldenström macroglobulinemia: A systematic review" Expert Review of Hematology (2019)
- "Epigenetic modifications as key regulators of Waldenstrom's Macroglobulinemia biology" Journal of Hematology & Oncology (2010)
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