Waldenström macroglobulinemia: Difference between revisions

Revision as of 23:36, 11 December 2024

Waldenström Macroglobulinemia

Waldenström macroglobulinemia (WM) is a rare type of non-Hodgkin lymphoma characterized by an overproduction of monoclonal IgM antibodies by lymphoplasmacytic cells. It is named after the Swedish physician Jan G. Waldenström, who first described the condition in 1944.

Pathophysiology

WM is a lymphoproliferative disorder that involves the bone marrow, lymph nodes, and spleen. The disease is characterized by the presence of lymphoplasmacytic lymphoma cells, which are a hybrid of B lymphocytes and plasma cells. These cells produce large amounts of IgM, leading to hyperviscosity syndrome, which can cause symptoms such as blurred vision, headaches, and bleeding.

The exact cause of WM is unknown, but it is associated with genetic mutations, particularly in the MYD88 gene, which is found in over 90% of cases. This mutation leads to the activation of the NF-kB signaling pathway, promoting cell survival and proliferation.

Clinical Presentation

Patients with WM may present with a variety of symptoms, including:

Fatigue
Weight loss
Night sweats
Peripheral neuropathy
Anemia
Hyperviscosity symptoms (e.g., visual disturbances, headaches)

Diagnosis

The diagnosis of WM is based on a combination of clinical, laboratory, and pathological findings. Key diagnostic criteria include:

Presence of IgM monoclonal gammopathy
Bone marrow biopsy showing infiltration by lymphoplasmacytic cells
Genetic testing for MYD88 L265P mutation

Laboratory tests often reveal elevated serum IgM levels, anemia, and sometimes thrombocytopenia.

Treatment

Treatment of WM is tailored to the individual patient and may include:

Plasmapheresis for hyperviscosity symptoms
Chemotherapy regimens such as bendamustine and rituximab
Targeted therapies like ibrutinib, a Bruton tyrosine kinase inhibitor
Stem cell transplantation in selected cases

Prognosis

The prognosis for WM varies, with a median survival of approximately 5-10 years. Factors influencing prognosis include age, performance status, and the presence of certain genetic mutations.

Also see

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-Waldenström macroglobulinemia - (VAHL-den-strum MA-kroh-GLAH-byoo-lih-NEE-mee-uh)An indolent (slow-growing) type of non-Hodgkin lymphoma marked by abnormal levels of IgM antibodies in the blood and an enlarged liver, spleen, or lymph nodes. Also called lymphoplasmacytic lymphoma.
+Waldenström Macroglobulinemia
-{{stub}}
+'''Waldenström macroglobulinemia''' (WM) is a rare type of [[non-Hodgkin lymphoma]] characterized by an overproduction of [[monoclonal]] [[IgM]] [[antibodies]] by [[lymphoplasmacytic cells]]. It is named after the Swedish physician Jan G. Waldenström, who first described the condition in 1944.
-{{dictionary-stub1}}
+==Pathophysiology==
+WM is a [[lymphoproliferative disorder]] that involves the [[bone marrow]], [[lymph nodes]], and [[spleen]]. The disease is characterized by the presence of [[lymphoplasmacytic lymphoma]] cells, which are a hybrid of [[B lymphocytes]] and [[plasma cells]]. These cells produce large amounts of IgM, leading to [[hyperviscosity syndrome]], which can cause symptoms such as [[blurred vision]], [[headaches]], and [[bleeding]].
+The exact cause of WM is unknown, but it is associated with genetic mutations, particularly in the [[MYD88]] gene, which is found in over 90% of cases. This mutation leads to the activation of the [[NF-kB]] signaling pathway, promoting cell survival and proliferation.
+==Clinical Presentation==
+Patients with WM may present with a variety of symptoms, including:
+* Fatigue
+* Weight loss
+* Night sweats
+* Peripheral neuropathy
+* Anemia
+* Hyperviscosity symptoms (e.g., visual disturbances, headaches)
+==Diagnosis==
+The diagnosis of WM is based on a combination of clinical, laboratory, and pathological findings. Key diagnostic criteria include:
+* Presence of IgM monoclonal gammopathy
+* Bone marrow biopsy showing infiltration by lymphoplasmacytic cells
+* Genetic testing for MYD88 L265P mutation
+Laboratory tests often reveal elevated serum IgM levels, anemia, and sometimes thrombocytopenia.
+==Treatment==
+Treatment of WM is tailored to the individual patient and may include:
+* [[Plasmapheresis]] for hyperviscosity symptoms
+* Chemotherapy regimens such as [[bendamustine]] and [[rituximab]]
+* Targeted therapies like [[ibrutinib]], a [[Bruton tyrosine kinase]] inhibitor
+* Stem cell transplantation in selected cases
+==Prognosis==
+The prognosis for WM varies, with a median survival of approximately 5-10 years. Factors influencing prognosis include age, performance status, and the presence of certain genetic mutations.
+==Also see==
+* [[Non-Hodgkin lymphoma]]
+* [[Multiple myeloma]]
+* [[Chronic lymphocytic leukemia]]
+* [[Monoclonal gammopathy of undetermined significance]]
+{{Lymphoma}}
+{{Hematology}}
+[[Category:Hematology]]
+[[Category:Lymphoma]]
+[[Category:Rare diseases]]