obsolete lymphoplasmacytic lymphoma

Obsolete Lymphoplasmacytic Lymphoma Description

Lymphoplasmacytic lymphoma, also known as Waldenström's macroglobulinemia in some contexts [1], is a rare type of cancer that affects the immune system. It is characterized by an abnormal increase of B-cells in the bone marrow and peripheral lymphoid tissues [4].

The disease is marked by the accumulation of monoclonal cells, which are cancerous cells that have multiplied uncontrollably [8]. This leads to various symptoms, including fatigue, weight loss, and bleeding or bruising easily.

In terms of its classification, lymphoplasmacytic lymphoma is a type of non-Hodgkin lymphoma (NHL), which is a group of cancers that affect the immune system [6]. It is considered an obsolete term in some contexts, with Waldenström's macroglobulinemia being used as a synonym in others.

Key Features:

• A rare type of cancer affecting the immune system
• Characterized by abnormal increase of B-cells in bone marrow and peripheral lymphoid tissues
• Accumulation of monoclonal cells leading to various symptoms
• Classified as a non-Hodgkin lymphoma (NHL)

References:

[1] - Definition: A B-cell lymphocytic neoplasm characterized by an uncontrolled increase of B-cells. [4] - Disease Ontology Definition:A B-cell lymphoma characterized by the accumulation of monoclonal cells in the bone marrow and peripheral lymphoid tissues, ... [6] - Any cancer of lymphocytes other than Hodgkin lymphoma. There are two main groups – high grade which are aggressive and fast growing and low grade which are slow ... [8] - A B-cell lymphoma characterized by the accumulation of monoclonal cells in the bone marrow and peripheral lymphoid tissues, and associated with the ...

Based on the provided context, here are the signs and symptoms of Waldenström macroglobulinemia (WM), a type of indolent B-cell lymphoma:

Common Early Symptoms:

• Fever
• Night sweats
• Weight loss (also known as "B-symptoms")
• Enlargement of the lymph nodes, spleen, and liver

These symptoms are often referred to as "B-symptoms" and can be a sign of an underlying malignancy. [1][2]

Physical Manifestations:

• Hepatomegaly (enlarged liver) in 20% of cases
• Splenomegaly (enlarged spleen) in 15% of cases
• Lymphadenopathy (enlarged lymph nodes) in 15% of cases

These physical manifestations can be a sign of the disease's progression. [3][4]

Other Symptoms:

• Anemia and thrombocytopenia (low platelet count)
• Hepatosplenomegaly (enlargement of both liver and spleen)
• Lymphadenopathy
• Rarely, hyperviscosity syndrome (a condition characterized by thickened blood)

These symptoms can occur in various combinations and may not be present in all patients. [5][6]

Progression of the Disease:

• If left untreated, patients with asymptomatic disease may progress to develop fatigue, weight loss, peripheral neuropathy, shortness of breath, and other symptoms.

Early detection and treatment are crucial to prevent the progression of the disease. [7]

It's essential to note that these symptoms can also be present in other conditions, and a definitive diagnosis requires medical evaluation and testing.

Based on the provided context, it appears that you are looking for diagnostic tests related to Waldenström macroglobulinemia (WM), a type of lymphoplasmacytic lymphoma.

To confirm the diagnosis of WM, several diagnostic tests can be performed:

• Bone Marrow Biopsy: A bone marrow biopsy is essential for diagnosing WM. It involves taking a sample of bone marrow from the hipbone and examining it under a microscope to look for clonal lymphoplasmacytic cells (1). The presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis (5, 7).
• Blood Tests: Blood tests can help identify the presence of an IgM M protein, which is a characteristic feature of WM (9).
• Imaging Studies: Imaging studies such as CT scans and PET/CT scans may be used to assess the extent of disease and monitor response to treatment (2, 8).
• Funduscopic Eye Exam: A funduscopic eye exam by an experienced ophthalmologist should also be conducted to look for signs of hyp

Treatment Options for Lymphoplasmacytic Lymphoma (LPL)

Lymphoplasmacytic lymphoma, also known as Waldenstrom macroglobulinemia, is a rare type of cancer that affects the immune system. While there are no specific "obsolete" treatments for LPL, the treatment landscape has evolved over time with the introduction of new therapies.

Historical Treatment Options

In the past, treatment options for LPL were limited and often focused on managing symptoms rather than targeting the underlying disease. However, with advancements in medical research, several treatment options have been developed to target the specific characteristics of LPL.

• Rituximab-based combinations: Rituximab is a monoclonal antibody that targets CD20, a protein found on B cells. When combined with other therapies, such as alkylating agents or proteasome inhibitors, rituximab has been shown to be moderately active in treating LPL (Search Result 3).
• Proteasome inhibitors: Proteasome inhibitors, such as bortezomib, have been used to treat LPL by targeting the protein degradation pathway. However, their use is often limited due to side effects and resistance (Search Result 5).

Current Treatment Options

In recent years, several new treatment options have emerged for LPL.

• BTK inhibitors: BTK inhibitors, such as ibrutinib, have been shown to be highly effective in treating LPL. Ibrutinib is an orally administered covalent BTK inhibitor that has been FDA-approved for the treatment of WM (Search Result 6).
• Rituximab-based chemo-immunotherapy: Rituximab-based chemo-immunotherapy remains a standard first-line treatment for Waldenstrom macroglobulinaemia, while ibrutinib has emerged as an effective alternative (Search Result 8).

Emerging Treatment Options

Research is ongoing to develop new and more effective treatments for LPL.

• Targeted therapies: Targeted therapies, such as monoclonal antibodies and kinase inhibitors, are being explored as potential treatment options for LPL.
• Immunotherapies: Immunotherapies, which harness the power of the immune system to fight cancer, may also hold promise in treating LPL.

In summary, while there are no specific "obsolete" treatments for lymphoplasmacytic lymphoma, the treatment landscape has evolved over time with the introduction of new therapies. Current treatment options include BTK inhibitors, rituximab-based chemo-immunotherapy, and emerging targeted therapies and immunotherapies.

The differential diagnosis of lymphoplasmacytic lymphoma (LPL), also known as Waldenström's Macroglobulinemia (WM), is broad and can be challenging.

Key considerations:

• Atypical LPL/WM vs. MZL: The distinction between atypical LPL/WM and marginal zone lymphoma (MZL) can be problematic, but MYD88 mutations can help clarify the diagnosis [2].
• LPL vs. CLL/SLL: Lymphoplasmacytic lymphoma can be distinguished from chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) by the presence of IgM monoclonal gammopathy and morphologic evidence of lymphoplasmacytic cells.
• WM vs. other lymphomas: The differential diagnosis of WM includes other types of non-Hodgkin lymphomas, such as follicular lymphoma, mantle cell lymphoma, and diffuse large B-cell lymphoma.

Diagnostic features:

• Bone marrow biopsy: A bone marrow biopsy showing infiltration by clonal lymphoplasmacytic cells/LPL is a key diagnostic feature of WM [9].
• IgM monoclonal gammopathy: The presence of IgM monoclonal gammopathy is a characteristic feature of WM.
• Lymphadenopathy and hepatosplenomegaly: Clinical features such as lymphadenopathy, hepatosplenomegaly, anemia, thrombocytopenia, and rarely hyperviscosity can be present in patients with WM [8].

References:

[1] Cingam S. (2022). Waldenström's Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM.

[2] Kazmierski D. (2017). The distinction between certain lymphomas can be problematic and in this case MYD88 was helpful in clarifying a diagnosis of atypical LPL/WM from MZL and in ...

[3] Cingam S. (2022). WM is a rare, indolent, incurable, non-Hodgkin's B cell lymphoma.

[4] Thomas R. (2019). Currently, WM is believed to be a manifestation of a distinct type of non-Hodgkin lymphoma called “lymphoplasmacytic lymphoma” (LPL).

[5] Cingam S. (2022). The differential diagnosis of WM is broad, and workup including a bone marrow biopsy, ...

[6] Cingam S. (2022). Abstract: Waldenström's Macroglobulinemia (WM) is a clonal B-lymphocyte neoplasm characterized by the presence of IgM.

[7] García-Sanz R. (2023). 1. Waldenström's macroglobulinemia (WM) is a lymphoplasmacytic lymphoma (LPL) with immunoglobulin M (IgM) monoclonal gammopathy and morphologic evidence of ...

[8] Gertz MA. (2021). Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.

[9] Dec 5, 2019. The diagnosis of WM requires a bone marrow (BM) biopsy showing infiltration by clonal lymphoplasmacytic cells/lymphoplasmacytic lymphoma (LPL).