Waldenström Macroglobulinemia Symptoms and Treatment Guidelines

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How I treat waldenström macroglobulinemia? Can waldenstrom macroglobulinemia be cured? Let’s find out about waldenström macroglobulinemia symptoms and treatment!

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Waldenström Macroglobulinemia Definition

Waldenström macroglobulinemia meaning – What is waldenström macroglobulinemia? Waldenstrom macroglobulinemia is a rare type of blood cell cancer that causes the bone marrow to have too many abnormal white blood cells.

Waldenström Macroglobulinemia Causes

What causes waldenström macroglobulinemia? What is the most common cause of waldenström macroglobulinemia?

Waldenstrom macroglobulinemia is a syndrome of IgM hypergammaglobulinemia. A low-grade non-Hodgkin lymphoma causes it with B cells that look like a mix of lymphocytes and plasma cells. The IgM paraprotein is usually made by these cells, and many of the symptoms of the disease are linked to this macroglobulin.

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Waldenström Macroglobulinemia Diagnosis

Monoclonal IgM paraprotein.
Infiltration of plasmacytic lymphocytes into the bone marrow.
Lytic bone disease is not present.

Waldenström Macroglobulinemia Symptoms

Signs and symptoms of waldenström macroglobulinemia – What are some symptoms of waldenström macroglobulinemia?

This disease usually occurs slowly when a person is in their 60s or 70s. Most of the time, patients with anemia are tired. The serum that is too thick can show up in several ways. Mucosal and GI bleeding is caused by swollen blood vessels and problems with platelets.

Other complaints include feeling sick, dizzy, and having trouble seeing. Changes in consciousness can range from mild drowsiness to coma. The IgM paraprotein can also cause symptoms of cold agglutinin disease (hemolysis) or chronic demyelinating peripheral neuropathy.

On examination, there may be enlarged liver, spleen, or swollen lymph nodes. The veins in the retina are full. It is possible to have purpura. There shouldn’t be any bone pain.

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Laboratory Findings

Nearly everyone has anemia, and rouleaux formation is common, even though red blood cells stick together when a blood smear is made at room temperature. Part of the reason for the anemia is that the paraprotein makes the plasma volume grow by 50-100%.

Most of the time, other blood counts are normal. There may be only a few abnormal plasmacytic lymphocytes on the peripheral blood smear. The plasmacytic lymphocytes usually fill up the bone marrow.

The main sign of macroglobulinemia is a monoclonal IgM spike in the beta globulin area of the serum PEP. The normal range for serum viscosity is between 1.4 and 1.8 times that of water.

Symptoms of hyperviscosity usually show up when the viscosity of the serum is more than four times that of water. When the viscosity is more than six times that of water, the symptoms are usually very noticeable. Because paraproteins have different physical and chemical properties, there isn’t a direct link between the amount of paraprotein in the blood and how thick the blood is.

The IgM paraprotein can cause a positive antiglobulin (Coombs) test for complement and has cold agglutinin or cryoglobulin properties. Suppose macroglobulinemia is thought to be the problem, but the serum PEP only shows hypogammaglobulinemia. In that case, the test should be done again, but this time special care should be taken to keep the blood at 37°C because the paraprotein may form clumps at room temperature. Radiographs of the bones are normal, and there is no sign of kidney damage.

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Differential Diagnosis

When malignant monoclonal cells are found in the bone marrow, Waldenstrom macroglobulinemia differs from MGUS. It is different from CLL because of the shape of the bone marrow, the lack of CD5 expression, and the lack of lymphocytosis. It is different from plasma cell myeloma because of the bone marrow’s shape, the IgM paraprotein’s presence, and the lack of lytic bone disease.

Waldenström Macroglobulinemia Treatment and Management Guidelines

Treatment of waldenström macroglobulinemia – What is the best treatment for waldenström macroglobulinemia?

Plasmapheresis should be used to treat patients with severe hyperviscosity syndrome (stupor, coma, pulmonary edema) as soon as possible.

Some patients with long-term conditions can be taken care of with periodic plasmapheresis alone. As with other slow-growing malignant lymphoid diseases, rituximab is very effective (375 mg/m2 intravenously once a week for 4–8 weeks).

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But be careful: after rituximab therapy, the IgM level often goes up before it goes down. So for advanced diseases, it is best to use a combination of treatments.

MYD88 is often mutated in Waldenstrom macroglobulinemia. The BTK inhibitor ibrutinib (420 mg daily) is very effective in these patients, with a 90% response rate and a 73% major response rate that can lead to long-lasting remissions.

Bortezomib, lenalidomide, and bendamustine have also been shown to help with this disease. Unfortunately, autologous hematopoietic stem cell transplantation is only done on cancer patients who have come back or are not responding to other treatments.