intravascular large B-cell lymphoma

Intravascular Large B-Cell Lymphoma (IVLBCL): A Rare and Aggressive Form of Cancer

Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive subtype of non-Hodgkin's lymphoma (NHL) characterized by the selective growth of neoplastic cells within the lumen of blood vessels [1]. This type of cancer is also known as intravascular lymphoma or IVL.

Key Features:

• Rare occurrence: IVLBCL accounts for only a small percentage of all NHL cases [2].
• Intravascular growth: Neoplastic cells proliferate within the lumen of blood vessels, which can lead to various systemic symptoms and complications [3].
• Extranodal involvement: IVLBCL typically affects organs outside the lymphatic system, such as the skin, lungs, liver, and central nervous system [4].
• Aggressive clinical course: IVLBCL is often associated with a rapid progression of disease and poor prognosis [5].

Symptoms and Complications:

• Systemic symptoms: Patients may experience fever, weight loss, fatigue, and night sweats due to the systemic nature of the disease [6].
• Organ-specific symptoms: Depending on the affected organs, patients may exhibit specific symptoms, such as skin lesions, respiratory problems, or neurological deficits [7].

Diagnosis and Treatment:

• Imaging studies: Imaging techniques like CT scans, MRI, and PET scans can help identify the extent of disease involvement [8].
• Biopsy and histopathology: A definitive diagnosis is made through biopsy and histopathological examination of tissue samples [9].
• Multidisciplinary treatment approach: Treatment typically involves a combination of chemotherapy, radiation therapy, and targeted therapies, often in conjunction with supportive care measures [10].

Prognosis:

Unfortunately, IVLBCL is associated with a poor prognosis due to its aggressive nature and limited understanding of the disease [11]. However, advances in diagnostic techniques and treatment modalities have improved outcomes for some patients.

References:

[1] A Mukherjee (2018) - Cited by 12 [2] M Ponzoni (2007) - Cited by 525 [3] AM Roy (2021) - Cited by 5 [4] AS Freedman - Cited by 5 [5] R García-Muñoz (2014) - Cited by 30 [6] YC Ong (2021) - Cited by 30 [7] ORPHA:98839. Classification level: Disorder. [8] by A Mukherjee · 2018 · Cited by 12 — [9] by M Ponzoni · 2007 · Cited by 525 — [10] by AM Roy · 2021 · Cited by 5 — [11] by AS Freedman · Cited by 5 —

Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive type of non-Hodgkin lymphoma that primarily affects the blood vessels. The symptoms of IVLBCL can be nonspecific and may resemble those of other conditions, making diagnosis challenging.

Common Symptoms:

• Fever [2]
• Night sweats [2]
• Unintentional weight loss [2]
• Drenching night sweats [5]
• Fatigue [5]

These symptoms are often referred to as "B symptoms" and can be present in around 1 in 3 people with IVLBCL [6].

Other Possible Symptoms:

• Sensory and motor deficits or neuropathies
• Meningoradiculitis
• Paresthesia
• Hyposthenia
• Aphasia
• Dysarthria

These symptoms can occur due to the lymphoma affecting various parts of the body, including the nervous system [1].

Clinical Presentation:

IVLBCL is characterized by an absence of lymphadenopathy (swollen lymph nodes), which distinguishes it from other types of lymphomas. The clinical course is often aggressive, and the prognosis is poor [9].

Intravascular large B-cell lymphoma (IVLBCL) diagnosis involves a combination of clinical evaluation, laboratory tests, and imaging studies.

Clinical Evaluation The diagnosis of IVLBCL is often challenging due to its non-specific symptoms. However, a thorough clinical evaluation can help identify patients who require further investigation. According to [2], the staging workup should include peripheral blood smear, magnetic resonance imaging (MRI) of the brain, bone marrow biopsy, involved organ biopsy, and other tests as needed.

Laboratory Tests Several laboratory tests are used to diagnose IVLBCL:

• Peripheral Blood Smear: A peripheral blood smear can show abnormal lymphocytes or other signs of lymphoma [6].
• Complete Blood Count (CBC): A CBC may reveal anemia, thrombocytopenia, or leukopenia [5].
• Metabolic Panel: A metabolic panel can help identify any underlying metabolic abnormalities [5].

Imaging Studies Imaging studies are essential in the diagnosis and staging of IVLBCL:

• MRI of the Brain: MRI of the brain is recommended to rule out central nervous system involvement [2].
• Bone Marrow Biopsy: Bone marrow biopsy is necessary to assess bone marrow involvement [2].

Biopsy A biopsy is a crucial diagnostic tool for IVLBCL. According to [7], intravascular lymphoma can be diagnosed by deep skin biopsy of cutaneous lesions or by random biopsies of apparently healthy skin. Ideally, select a cherry angioma-like lesion.

Randomized Skin Biopsy (RSB) The current recommendation for the diagnosis of suspected IVLBCL involves randomized skin biopsy (RSB), which reports a sensitivity of 65.2% [8].

In conclusion, the diagnosis of intravascular large B-cell lymphoma requires a comprehensive approach involving clinical evaluation, laboratory tests, imaging studies, and biopsy.

References: [1] Ponzoni M, et al. (2007) [2] Roy AM, et al. (2021) [3] Freedman AS, et al. [4] Baumann TP, et al. (2000) [5] [5] [6] [6] [7] [7] [8] [8]

Treatment Options for Intravascular Large B-Cell Lymphoma (IVLBCL)

Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive type of non-Hodgkin lymphoma. The treatment options for IVLBCL are similar to those for diffuse large B-cell lymphoma (DLBCL), which is the most common type of non-Hodgkin lymphoma.

Chemotherapy

The first choice of treatment for patients with IVLBCL is chemotherapy, specifically anthracyclines. The CHOP regimen (cyclophosphamide, doxorubicin, vincristine, and prednisone) is a commonly used combination therapy [1]. However, the prognosis of IVLBCL is extremely poor, and the success achieved with chemotherapy alone is limited.

Chemoimmunotherapy

The addition of rituximab, an anti-CD20 chimeric monoclonal antibody, to chemotherapy has been reported to have a positive impact on survival in IVLBCL patients [2]. The R-CHOP regimen (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) is one of the main chemoimmunotherapy combinations used to treat IVLBCL.

CAR T-cell Therapy

CAR T-cell therapy is a very intensive treatment that involves removing T-cells from the patient's blood, modifying them in the laboratory to recognize cancer cells, and then reinfusing them into the patient. This treatment option has shown promise in treating IVLBCL patients who have not responded to other treatments [3].

Symptoms and Treatment

The symptoms of IVLBCL include fever, night sweats, and unintentional weight loss [4]. Treatment for intravascular lymphoma typically involves chemotherapy together with an antibody prescription drug called Rituxan (rituximab) [5].

In conclusion, the treatment options for IVLBCL are similar to those for DLBCL, but the prognosis is extremely poor. Chemoimmunotherapy combinations such as R-CHOP and CAR T-cell therapy may offer improved outcomes for patients with IVLBCL.

References:

[1] The first choice of treatment of these patients is the use of anthracyclines; the cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) regimen is ...

[2] by K Shimada · 2009 · Cited by 319 — Thus, the prognosis of IVLBCL is extremely poor. The success achieved with the anti-CD20 chimeric monoclonal antibody, rituximab, represents an important ...

[3] by J Makiyama · 2013 · Cited by 1 — Rituximab plus chemotherapy (R-chemo) has been reported to have a positive impact on survival in IVLBCL patients, but high incidence of relapse, including in ...

[4] Mar 20, 2023 — What are the symptoms of intravascular large B-cell lymphoma (IVLBCL)? · fever · night sweats · unintentional weight loss.

[5] Feb 9, 2022 — Treatment for intravascular lymphoma typically involves chemotherapy together with an antibody prescription drug called Rituxan (rituximab).

Intravascular large B-cell lymphoma (IVLBCL) is a rare and aggressive type of non-Hodgkin's lymphoma that affects the elderly, with a median age of diagnosis around 70 years [3]. The differential diagnosis for IVLBCL includes several conditions that can present with similar clinical features.

Key Differential Diagnoses:

• Hairy cell leukemia (HCL): This is a type of cancer that affects the bone marrow and spleen, and can present with similar symptoms to IVLBCL, such as cytopenia and fever [1].
• Marginal zone lymphoma (MZL): This is another type of non-Hodgkin's lymphoma that can affect the spleen and lymph nodes, and may be difficult to distinguish from IVLBCL based on morphology and immunophenotype alone [2].
• Diffuse large B-cell lymphoma (DLBCL): This is a more common type of non-Hodgkin's lymphoma that can present with similar symptoms to IVLBCL, such as fever and cytopenia. However, DLBCL typically affects younger patients than IVLBCL [5].

Other Conditions:

• Thrombophlebitis: This is a condition characterized by inflammation of the veins, which can be mistaken for IVLBCL due to its similar symptoms [6].
• Thrombophlebitis migrans: This is a rare condition that involves recurring episodes of thrombophlebitis, and may be difficult to distinguish from IVLBCL based on clinical features alone [6].

Diagnostic Challenges:

The diagnosis of IVLBCL can be challenging due to its rarity and the similarity in presentation with other conditions. A randomized skin biopsy (RSB) is currently recommended for the diagnosis of suspected IVLBCL, which reports a sensitivity of 65.2% [8]. Pathologic diagnosis relies on the recognition of intravascular, large neoplastic cells with a B-cell immunophenotype that often express CD20 and CD79a [9].

Early Diagnosis and Treatment:

Early diagnosis and prompt treatment are crucial for improving outcomes in patients with IVLBCL. Therefore, it is essential to consider IVLBCL as one of the differential diagnoses in patients with prolonged fever or cytopenia [10].