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mantle cell lymphoma
ICD-10 Codes
Description
Mantle cell lymphoma (MCL) is a rare type of B-cell non-Hodgkin lymphoma (NHL). It develops when white blood cells called lymphocytes grow out of control.
Characteristics:
- MCL is a cancer that starts in the mantle zone of the lymph node, which is the outer edge of the lymph node follicle.
- The abnormal B cells usually found in this area are malignant and multiply uncontrollably.
- This type of lymphoma is often slow-growing before symptoms appear.
Symptoms:
- Fever
- Involuntary weight loss of more than 5% of body weight in a short amount of time
- Night sweats
Definition:
MCL is a rare subtype of B-cell non-Hodgkin lymphomas (NHLs) usually identified by a translocation of the CCND1 gene.
Cancer cells:
The cancer cells associated with MCL are small- to medium-size and may be found in various parts of the body, including: * Lymph nodes * Spleen * Bone marrow * Blood * Gastrointestinal system
Note: The information provided is based on the search results and citations from the following sources:
[2] - Mantle cell lymphoma is a rare blood cancer that starts in white blood cells in your lymph nodes. [3] - Mantle cell lymphoma is a rare type of B cell non-Hodgkin lymphoma (NHL). [5] - Mantle cell lymphoma (MCL) is a rare subtype of B-cell non-Hodgkin lymphomas (NHLs) usually identified by a translocation of the CCND1 gene. [8] - Mantle cell lymphoma is a type of non-Hodgkin lymphoma that develops from B cells. [9] - It is marked by small- to medium-size cancer cells that may be in the lymph nodes, spleen, bone marrow, blood, and gastrointestinal system.
Additional Characteristics
- Night sweats
- Fever
- MCL is a rare type of B-cell non-Hodgkin lymphoma (NHL)
- it develops when white blood cells called lymphocytes grow out of control
- MCL is a cancer that starts in the mantle zone of the lymph node, which is the outer edge of the lymph node follicle
- The abnormal B cells usually found in this area are malignant and multiply uncontrollably
- This type of lymphoma is often slow-growing before symptoms appear
- Involuntary weight loss of more than 5% of body weight in a short amount of time
- MCL is a rare subtype of B-cell non-Hodgkin lymphomas (NHLs) usually identified by a translocation of the CCND1 gene
- The cancer cells associated with MCL are small- to medium-size and may be found in various parts of the body, including: Lymph nodes, Spleen, Bone marrow, Blood, Gastrointestinal system
Signs and Symptoms
Common Signs and Symptoms of Mantle Cell Lymphoma
Mantle cell lymphoma (MCL) is a rare type of non-Hodgkin lymphoma that can cause various symptoms. Here are some common signs and symptoms associated with MCL:
- Swollen lymph nodes: The most obvious symptom of MCL is the presence of swollen lymph nodes, particularly in the neck, armpit, or groin [7]. These swellings are enlarged lymph nodes that can be painless [5].
- Painless swellings: In addition to swollen lymph nodes, people with MCL may experience painless swellings in other parts of their body [4].
- Fever and night sweats: Some individuals with MCL may experience fever and night sweats as a symptom [1].
- Weight loss and nausea: Weight loss and nausea or vomiting are also common symptoms associated with MCL [2, 8].
- Heartburn and bloating: Heartburn, belly pain, or bloating can also be experienced by people with MCL [3].
It's essential to note that these symptoms can vary from person to person, and not everyone will experience all of them. If you're experiencing any of these symptoms, it's crucial to consult a healthcare professional for proper diagnosis and treatment.
References: [1] Sep 28, 2020 — Mantle cell lymphoma symptoms include fever, involuntary weight loss of more than 5% of your body weight in a short amount of time, and night sweats. [2] Aug
Additional Symptoms
- Swollen lymph nodes
- Painless swellings
- Fever and night sweats
- Weight loss and nausea
- Heartburn and bloating
Diagnostic Tests
Mantle cell lymphoma (MCL) is a rare type of blood cancer that affects the white blood cells in the lymph nodes. Diagnostic tests for MCL are crucial to confirm the diagnosis and rule out other conditions.
Common diagnostic tests for MCL:
- Lymph node biopsy: A small sample of tissue from an affected lymph node is removed and examined under a microscope. This test is considered definitive for diagnosing MCL [12][13].
- Flow cytometry: This test measures the number of lymphocytes present in the blood or bone marrow. It can help identify abnormal cells that may indicate MCL [15].
- Bone marrow biopsy: A sample of bone marrow tissue is removed and examined to check for any abnormalities, including cancerous cells.
- Blood tests: Blood samples are taken to check for various biomarkers, such as lactate dehydrogenase (LDH) levels, which can indicate the presence of MCL [10].
Other diagnostic methods:
- Imaging studies: Imaging techniques like CT scans or PET scans may be used to visualize the affected lymph nodes and monitor the progression of the disease.
- Molecular testing: Genetic tests can identify specific mutations associated with MCL, such as overexpression of cyclin D1 [14].
Early detection is key:
While these diagnostic tests are essential for confirming a diagnosis of MCL, early detection is critical to improve treatment outcomes. If you're experiencing symptoms or have concerns about your health, consult a healthcare professional for guidance.
References:
[10] - Blood tests. Your doctor takes some of your blood and sends it to a lab to get analyzed. [12] - Diagnosis of MCL should not be based on the examination of bone marrow alone; obtaining a lymph node biopsy is required. [13] - Mantle cell lymphoma is typically diagnosed by a biopsy. This is a small procedure to remove a sample of tissue, such as a swollen lymph node. [14] - Identification of excess cyclin D1 from a biopsy is considered a very sensitive tool for diagnosing MCL. [15] - In that case, your doctor may perform a test called flow cytometry to detect how many lymphocytes are present.
Additional Diagnostic Tests
- Blood tests
- Imaging studies
- Bone marrow biopsy
- Molecular testing
- Flow cytometry
- Lymph node biopsy
Treatment
Treatment Options for Mantle Cell Lymphoma
Mantle cell lymphoma (MCL) is a type of non-Hodgkin lymphoma that requires prompt and effective treatment to manage its progression. The most common drug combinations used to treat MCL are:
- R-CHOP: A combination of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone. This regimen is often used as a first-line treatment for MCL [1].
- BR (Bendamustine + Rituximab): A combination of bendamustine and rituximab, which has shown efficacy in treating MCL patients [7].
Targeted Therapies
In addition to chemotherapy regimens, targeted therapies have emerged as a promising treatment option for MCL. These include:
- BTK inhibitors: Small molecule drugs that target the Bruton's tyrosine kinase (BTK) protein, which plays a crucial role in B-cell signaling. Examples of BTK inhibitors used to treat MCL include zanubrutinib and acalabrutinib [5].
- Covalent BTK inhibitors: A newer class of BTK inhibitors that have shown significant efficacy in treating relapsed or refractory MCL patients [10].
Other Treatment Options
Other treatment options for MCL may include:
- Stem cell transplantation: A procedure where the patient's stem cells are collected, treated with chemotherapy and/or radiation therapy, and then reinfused into the body to replace damaged cells [8].
- Maintenance rituximab: The use of rituximab as a maintenance therapy to prevent disease recurrence in patients who have completed initial treatment [6].
References
[1] Feb 1, 2024 — The most common regimens are rituximab plus CHOP (R-CHOP), rituximab plus bendamustine or a variation of R-CHOP called VR-CAP, which includes a ...
[5] Drugs used to treat Mantle Cell Lymphoma ; Generic name: zanubrutinib systemic; Brand name: Brukinsa; Drug class: BTK inhibitors; For consumers: dosage, ...
[7] The most common drug combinations used to treat mantle cell lymphoma are: BR – bendamustine (Treanda, Benvyon, Esamuze) and rituximab; R-CHOP – rituximab ...
[10] by A Kumar · 2022 · Cited by 37 — Covalent BTK (cBTK) inhibitors are a cornerstone of treatment for patients with relapsed/refractory mantle cell lymphoma.
Recommended Medications
- R-CHOP
- Covalent BTK inhibitors
- Stem cell transplantation
- Maintenance rituximab
- bendamustine
- acalabrutinib
💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.
Differential Diagnosis
Differential Diagnosis of Mantle Cell Lymphoma
Mantle cell lymphoma (MCL) can be challenging to diagnose due to its overlapping features with other types of lymphomas. The differential diagnosis for MCL includes:
- Small lymphocytic lymphoma (SLL)/Chronic lymphocytic leukemia (CLL): SLL and CLL share similar cytologic features with MCL, making it essential to obtain a lymph node biopsy for accurate diagnosis [2].
- Follicular lymphoma (FL): FL can be distinguished from MCL by the presence of follicles in the lymph node biopsy specimen [4].
- Marginal zone-related B-cell lymphomas: These lymphomas can also be considered in the differential diagnosis, as they share some overlapping features with MCL [3].
- Hodgkin's disease and non-Hodgkin's lymphoma (NHL): While not typically part of the differential diagnosis for MCL, it is essential to rule out these conditions when evaluating a patient with suspected MCL [3].
Key Diagnostic Features
To accurately diagnose MCL, it is crucial to obtain a lymph node biopsy and examine the tissue specimen. The presence of cyclin D1 overexpression, which results from the t(11;14) chromosomal translocation, is a key diagnostic feature of MCL [9]. Additionally, CD200 expression may help in differential diagnosis between MCL and B-cell chronic lymphocytic leukemia [8].
References
[1] eMedicine Logo. (2024, Jun 7). Diagnosis of MCL should not be based on the examination of bone marrow alone; obtaining a lymph node biopsy is required.
[2] Vose JM. (2015). The differential diagnosis of MCL includes small lymphocytic lymphoma, marginal zone lymphoma, and follicular lymphoma.
[3] ... mantle cell lymphoma (MCL). Comparisons may be useful for a differential diagnosis: Hodgkin's disease and non-Hodgkin's lymphoma (NHL) are the two major ...
[4] The differential diagnosis includes CLL/SLL, FL, PLL, and marginal zone-related B-cell lymphomas. There are overlapping cytologic features between CLL/SLL and MCL.
[5] Differential diagnoses include follicular lymphoma (see this term) and other forms of lymphoma. Treatment of mantel cell lymphoma includes intensive ...
[6] Feb 1, 2024 — Diagnosis and staging. Mantle cell lymphoma is typically diagnosed by a biopsy. This is a small procedure to remove a sample of tissue, such as ...
[7] Jan 5, 2024 — DIAGNOSIS AND CLASSIFICATION · Diagnosis · Classification · DIFFERENTIAL DIAGNOSIS · Chronic lymphocytic leukemia · Follicular lymphoma ...
[8] Palumbo GA. (2009). CD200 expression may help in differential diagnosis between mantle cell lymphoma and B-cell chronic lymphocytic leukemia.
[9] Quintanilla-Martinez L. (2009). Mantle cell lymphoma is characterized by the t(11;14) chromosomal translocation, resulting in the overexpression of cyclin D1 (CycD1).
Additional Differential Diagnoses
- diffuse large B-cell lymphoma
- obsolete lymphoplasmacytic lymphoma
- splenic marginal zone lymphoma
- B-cell adult acute lymphocytic leukemia
- lymphoproliferative syndrome 2
- lymphoplasmacytic lymphoma
- lymphoblastic lymphoma
- B-lymphoblastic leukemia/lymphoma
- B-lymphoblastic leukemia/lymphoma MLL rearranged
- B-lymphoblastic leukemia/lymphoma with hyperdiploidy
- B-lymphoblastic leukemia/lymphoma with iAMP21
- diffuse large B-cell lymphoma germinal center B-cell type
- B-cell prolymphocytic leukemia
- EZB diffuse large B-cell lymphoma
- ST2 diffuse large B-cell lymphoma
- EZB-MYC+ diffuse large B-cell lymphoma
- prolymphocytic leukemia
- obsolete lymphoid leukemia
- Chronic lymphocytic leukemia (CLL)
- Marginal zone-related B-cell lymphomas
- follicular lymphoma
- Hodgkin's lymphoma
Additional Information
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- A B-cell lymphocytic neoplasm due to CD5 positive antigen-naive pregerminal center B-cell within the mantle zone that surrounds normal germinal center follicles.
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