primary mediastinal B-cell lymphoma

Primary Mediastinal B-Cell Lymphoma (PMBCL): An Overview

Primary mediastinal B-cell lymphoma (PMBCL) is a rare and aggressive type of non-Hodgkin lymphoma that affects the mediastinum, the area behind the breastbone and between the lungs. This cancerous condition primarily affects young adults, with a slight female predominance [6].

Characteristics

• PMBCL is a subtype of diffuse large B-cell lymphoma (DLBCL) that arises in the thymus [7].
• It is characterized by the expression of B cell surface molecules such as CD19, CD20, CD22, and CD79a [4].
• The cancer develops from B cells in the mediastinum and may spread to organs and tissues such as the lungs, heart, and other structures in the chest [12].

Symptoms and Diagnosis

While symptoms of PMBCL can vary, common signs include:

• Chest pain or discomfort
• Shortness of breath
• Coughing
• Fatigue
• Weight loss

Diagnosis is typically made through a combination of imaging tests (such as CT scans), biopsy, and laboratory tests to confirm the presence of cancer cells.

Treatment Options

Treatment for PMBCL includes:

• Chemotherapy: A combination of drugs used to kill cancer cells.
• Radiation therapy: High-energy rays used to target and destroy cancer cells.
• Immunotherapy: Treatments that stimulate the immune system to attack cancer cells.
• Stem cell transplant: A procedure where healthy stem cells are infused into the body to replace damaged ones.

The choice of treatment depends on various factors, including the stage of the disease, overall health, and specific characteristics of the cancer [5].

Prognosis

While PMBCL is an aggressive form of lymphoma, advances in treatment have improved outcomes for patients. The prognosis varies depending on individual circumstances, but with timely and effective treatment, many people can achieve remission or long-term survival.

References:

[1] - Primary mediastinal large B-cell lymphoma is a type of Non-Hodgkin lymphoma that affects the area behind the breastbone and between the lungs. [2] by A Dabrowska-Iwanicka · 2014 · Cited by 60 — Primary mediastinal B-cell lymphoma (PMBCL) is a relatively rare lymphoma subtype affecting mainly young adults. [3] July 21, 2021 - Primary mediastinal large B-cell lymphoma (PMBCL) — sometimes called primary thymic mediastinal lymphoma — is a cancer that forms from cells of the immune [4] Primary mediastinal large B cell lymphoma (PMBCL) is an aggressive large B cell lymphoma originating in the mediastinum, that mainly expresses B cell surface molecules, such as CD19, CD20, CD22, andCD79a. [5] Primary mediastinal B-cell lymphoma (PMBCL) is a type of non-Hodgkin lymphoma (NHL). Treatments for PMBCL include chemotherapy, radiation therapy, immunotherapy and stem cell transplant. [6] Primary mediastinal B-cell lymphoma develops from B cells in the mediastinum (the area behind the breastbone). It may spread to organs and tissues such as ... [7] December 30, 2021 - Recently, Lisa Giulino-Roth, MD, from Weill Cornell Medical College in New York, discussed the management of primary mediastinal B-cell lymphoma, a rare subtype of non-Hodgkin lymphoma that occurs most often in adolescents and young adults. [8] Primary mediastinal large B-cell lymphoma is a type of non-Hodgkin lymphoma. Learn more about its signs, symptoms, diagnosis, types, and treatment. [9] 1 week ago - Primary mediastinal B-cell lymphoma, abbreviated PMBL or PMBCL, is a rare type of lymphoma that forms in the mediastinum (the space in between the lungs) and predominantly affects young adults.

Common Signs and Symptoms of Primary Mediastinal B-Cell Lymphoma

Primary mediastinal B-cell lymphoma (PMBCL) is a type of non-Hodgkin lymphoma that affects the area behind the breastbone and between the lungs. The disease often presents with symptoms such as:

• Cough or shortness of breath: This is one of the most common symptoms, caused by the tumor pressing on the windpipe and the large veins above the heart [1][2].
• Chest pain or discomfort: The mass effect of the tumor can cause chest pain or discomfort, which may worsen with deep breathing or coughing [3][4].
• Swelling in the neck or face: As the lymph nodes around the lungs and behind the breastbone become enlarged, they can cause symptoms such as a cough or an ache or pain in the chest [5].
• Superior vena cava syndrome: This is a rare but serious symptom caused by compression of the superior vena cava vein, which can lead to swelling in the face, neck, and arms [6][7].

These symptoms are often related to tumor mass infiltration or compression, and diagnosis is made by imaging studies such as CT scans, PET scans, and biopsies [8]. It's essential to seek medical attention if you experience any of these symptoms, as prompt treatment can improve outcomes.

References:

[1] July 21, 2021 - Primary mediastinal large B-cell lymphoma (PMBCL) is an aggressive large B cell lymphoma originating in the mediastinum...

[2] Primary mediastinal B-cell lymphoma often presents with symptoms of cough, shortness of breath, or swelling of the head and neck, due to the tumor pressing on the windpipe and the large veins above the heart.

[3] May 4, 2022 - PMBCL affects young adults in their ... compression by a rapidly growing mass in the anterior mediastinum; these include superior vena cava syndrome, cough, dyspnea, hoarseness, and dysphagia....

[4] It may also be called mediastinal ... nodes around the lungs and behind the breastbone become enlarged, they can cause symptoms such as a cough or an ache or pain in the chest.

[5] The Most Common Signs and Symptoms of Primary Mediastinal B-Cell Lymphoma include: · Cough or shortness of breath: · Chest pain or discomfort: · Swelling in the ...

[6] Primary mediastinal large B-cell lymphoma is a type of non-Hodgkin lymphoma. Learn more about its signs, symptoms, diagnosis, types, and treatment.

[7] The disease often presents with symptoms such as chest pain, cough, shortness of breath, and superior vena cava syndrome due to the mass effect of the tumor in ...

[8] by C Weidenbaum · 2022 — 7 Common signs/symptoms are cough, chest pain, dysphagia, and tachypnea, which are related to tumor mass infiltration or compression. Diagnosis is made by ...

Diagnostic Tests for Primary Mediastinal B-Cell Lymphoma (PMBCL)

Diagnosing PMBCL involves a combination of blood tests, biopsy tests, and imaging tests to confirm the presence of this type of cancer. Here are some of the diagnostic tests used:

• Blood Tests: Blood tests can reveal abnormal levels of cells and substances in the body, such as anemia or high levels of lactate dehydrogenase (LDH) [1].
• Imaging Tests: Imaging methods like computed tomography (CT), magnetic resonance imaging (MRI), and PET/CT are used to visualize the tumor and its spread [2]. CT is often the first-line imaging method for PMBCL diagnosis.
• Biopsy: A biopsy, where a sample of tissue is taken from the affected area, is the most common test for diagnosing lymphoma, including PMBCL [6].
• Bone Marrow Aspirate and Biopsy: This test involves taking a sample of bone marrow to check for cancer cells [5].

Additional Tests

Other tests may be performed to assess kidney and liver function, measure serum glucose, calcium, albumin, LDH, and beta2-microglobulin levels, among other things [8]. However, these tests are not diagnostic on their own.

It's essential to note that a tissue biopsy should be evaluated by a pathologist who is an expert in the diagnosis of lymphoma for accurate diagnosis [3].

Molecular Diagnosis

The molecular diagnosis of PMBL using a gene expression signature may offer a more precise diagnosis than currently available methods [7].

Treatment Options for Primary Mediastinal B-Cell Lymphoma

Primary mediastinal B-cell lymphoma (PMBCL) is a type of non-Hodgkin lymphoma that affects the mediastinum, which is the central part of the chest cavity. The treatment options for PMBCL typically involve a combination of chemotherapy and immunotherapy.

Chemotherapy

Chemotherapy is the standard initial treatment approach for PMBCL [9]. A commonly used regimen consists of four drugs: cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) [3]. This combination has been shown to be effective in treating PMBCL.

Chemoimmunotherapy

In addition to chemotherapy, a chemoimmunotherapy regimen consisting of rituximab and an anthracycline is often used for upfront treatment [1]. Rituximab is an immunotherapy drug that targets the CD20 protein on B cells, while anthracyclines are a type of chemotherapy drug.

Immunotherapy

Immunotherapy has emerged as a promising treatment option for PMBCL. Axicabtagene ciloleucel (Yescarta) is a CAR T-cell therapy that may be offered for PMBCL [5]. Bispecific antibodies, such as tafasitamab and mosunetuzumab, are also being investigated as potential treatments for PMBCL.

Targeted Therapy

Pembrolizumab (Keytruda) is an immunotherapy drug that has been approved by the FDA for people whose PMBCL doesn't respond to chemo or has come back [8]. This targeted therapy works by blocking a protein called PD-1, which can help cancer cells evade the immune system.

Treatment of Relapsed/Refractory Disease

For patients with relapsed or refractory PMBCL, high-dose chemotherapy is typically used, followed by radiation therapy (RT) if necessary [4]. CAR T-cell therapies, such as axicabtagene ciloleucel and lisocabtagene maraleucel, are also being investigated for the treatment of relapsed/refractory PMBCL.

References:

[1] Dec 30, 2021 — There is no standard of care for upfront treatment, although most groups use a chemoimmunotherapy regimen consisting of rituximab and an anthracycline. [3] May 31, 2024 — Most often, the treatment is chemotherapy (chemo), usually with 4 drugs known as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), plus the ... [4] by L Giulino-Roth · 2018 · Cited by 79 — For all other patients, the treatment of relapsed/refractory disease is typically high-dose chemotherapy, with or without RT, followed by ... [5] by B Fakhri · 2021 · Cited by 26 — Axicabtagene ciloleucel and lisocabtagene maraleucel are two products used in rrPMBCL. Keywords: CAR T therapy; Primary mediastinal B cell ... [6] PMBCL is often treated with a combination of the immunotherapy drug rituximab and chemotherapy. This is called chemoimmunotherapy. [7] Axicabtagene ciloleucel (Yescarta) is a CAR T-cell therapy that may be offered for PMBCL. Bispecific antibodies are a new type of immunotherapy. They may be ... [8] Mar 5, 2024 — The FDA has approved an immunotherapy drug called pembrolizumab (Keytruda) for people whose PMBCL doesn't respond to chemo or has come back. [9] Jul 21, 2021 — Chemotherapy is the standard initial treatment approach for PMBCL. Combinations of chemotherapy drugs are often used for certain treatment ...

Differential Diagnosis of Primary Mediastinal B-Cell Lymphoma (PMBCL)

Primary mediastinal B-cell lymphoma (PMBCL) is a type of non-Hodgkin lymphoma that affects the mediastinum, which is the central part of the chest cavity. The differential diagnosis of PMBCL includes other types of lymphomas and conditions that can present with similar symptoms.

Types of Lymphomas to Consider:

• "Gray zone" lymphoma: This type of lymphoma is a subtype of B-cell lymphoma that can be challenging to diagnose due to its overlapping features with PMBCL [1].
• Diffuse large B cell lymphoma (DLBCL): DLBCL is a type of non-Hodgkin lymphoma that can present in the mediastinum and may require molecular diagnosis for accurate identification [9].
• Nodal sclerosis Hodgkin lymphoma (NSCHL): NSCHL is a subtype of Hodgkin lymphoma that can mimic PMBCL in its presentation [6].

Other Conditions to Consider:

• Acute Lymphoblastic Leukemia (ALL): ALL is a type of blood cancer that can present with mediastinal masses and may require differential diagnosis from PMBCL [3].
• Thyroid or lung cancers: These types of cancers can also present as anterior mediastinal masses, making them part of the differential diagnosis for PMBCL [8].

Key Points to Consider:

• The diagnosis of PMBCL mainly depends on pathological features, imaging examination, and clinical features [2].
• Molecular diagnosis may be required to accurately identify DLBCL or other types of lymphomas that can mimic PMBCL [9].
• A thorough differential diagnosis is essential to ensure accurate identification and treatment of PMBCL.

References:

[1] - "gray zone" lymphoma - B-cell lymphoma, [1] [2] - The diagnosis of PMBCL mainly depends on pathological features, imaging examination and clinical features. [2] [3] - Acute Lymphoblastic Leukemia (ALL), [3] [6] - Nodal sclerosis Hodgkin lymphoma (NSCHL), [6] [8] - Thyroid or lung cancers, [8] [9] - Diffuse large B cell lymphoma (DLBCL), [9]