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Iron deficiency anemia
Anemia of chronic disease
Hemolytic disease of the newborn
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Autoimmune hemolytic anemia
Pyruvate kinase deficiency
Paroxysmal nocturnal hemoglobinuria
Sickle cell disease (NORD)
Anemia of chronic disease
Folate (Vitamin B9) deficiency
Vitamin B12 deficiency
Acute intermittent porphyria
Porphyria cutanea tarda
Vitamin K deficiency
Immune thrombocytopenic purpura
Thrombotic thrombocytopenic purpura
Von Willebrand disease
Disseminated intravascular coagulation
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
Protein S deficiency
Polycythemia vera (NORD)
Essential thrombocythemia (NORD)
Langerhans cell histiocytosis
Monoclonal gammopathy of undetermined significance
Microcytic anemia: Pathology review
Non-hemolytic normocytic anemia: Pathology review
Intrinsic hemolytic normocytic anemia: Pathology review
Extrinsic hemolytic normocytic anemia: Pathology review
Macrocytic anemia: Pathology review
Heme synthesis disorders: Pathology review
Coagulation disorders: Pathology review
Platelet disorders: Pathology review
Mixed platelet and coagulation disorders: Pathology review
Thrombosis syndromes (hypercoagulability): Pathology review
Lymphomas: Pathology review
Leukemias: Pathology review
Plasma cell disorders: Pathology review
Myeloproliferative disorders: Pathology review
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Robyn Hughes, MScBMC
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.
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.
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