WALDENSTROM MACROGLOBULINEMIA HOW CURE ????
Waldenstrom macroglobulinemia is a syndrome of IgM hypergammaglobulinemia that occurs in the setting of a low grade non-Hodgkin lymphoma characterized by B cells that are morphologically a hybrid of lymphocytes and plasma cells. These cells secrete the IgM paraprotein, and many clinical manifestations of the disease are related to this macroglobulin. Patients usually present with fatigue related to anemia. Hyperviscosity of serum may be manifested in a number of ways.
Gastrointestinal or mucosal bleeding is related to engorged blood vessel or dysfunction of platelets. Other complaints like vertigo, nausea and visual disturbances. Patients may observed consciousness that are vary from mild lethargy to stupor and coma. The IgM paraprotein may also cause symptoms of cold agglutinin disease (hemolysis) or chronic demyelinating peripheral neuropathy. By diagnosing there may be lymphadenopathy and hepatosplenomegaly. Retinal veins are engorged and purpura may be present. There will be bone tenderness not seen.
Anemia is nearly universal, and rouleaux formation is common, although the red blood cells are agglutinated when the blood smear is prepared and done at room temperature. The anemia is related in part to expansion of the plasma volume by 50% to100% due to the presence of the paraprotein.
Other blood counts are usually normal in value. The abnormal plasmacytic lymphocytes may appear in small numbers on the blood smear. The bone marrow is characteristically infiltrated by the plasmacytic lymphocytes. The mark of macroglobulinemia is the presence of a monoclonal IgM spike seen on serum PEP in the beta- globulin region. The serum viscosity is above the viscosity of water. Symptoms of hyperviscosity usually develop when the serum viscosity is over four times that of water, and marked symptoms usually arise when the viscosity is over six times that of water. Because paraproteins vary in their physicochemical properties, there is no correlation between the concentration of paraprotein and serum viscosity.
The IgM paraprotein may cause a positive antiglobulin (Coombs) test for complement and have cold agglutinin or cryoglobulin properties. If macroglobulinemia is suspected but the serum PEP shows only hypogammaglobulinemia, the test should be done again while taking special measures to maintain the blood at 37°C, since the paraprotein may precipitate out at room temperature. Differential Diagnosis Waldenstrom macroglobulinemia is differentiated from MGUS by the finding of bone marrow infiltration with monoclonal malignant cells. It is distinguished from CLL by morphology of bone marrow, the absence of lymphocytosis and absence of CD5 expressions.
TREATMENT
Patients with syndrome of hyperviscosity like pulmonary edema and stupor must take a treatment as early as possible with plasmapheresis.
Some chronic ill patients take plasmapheresis therapy on a periodical basis. As with other malignant lymphoid disease rituximab shows a significant activity. In patients with relapsed or refractory disease the BTK inhibitor ibrutinib is shown a significant activity. Other drugs that are used in this disease are lenlidomide, bortezomib,and bendamustine.
For Informational purpose only. Consult your Physician for advice.
