plasmablastic lymphoma

Plasmablastic lymphoma (PBL) is an uncommon but aggressive subtype of diffuse large B-cell lymphoma.

Characteristics and Features

• PBL is a highly aggressive B cell non-Hodgkin lymphoma frequently associated with immunosuppression [3].
• It often develops in people who have immune system problems, such as those infected with human immunodeficiency virus (HIV) [2][7].
• The neoplastic cells in PBL are large and resemble plasma cells or immunoblasts, expressing plasma cell markers and lacking B-cell markers [1].

Clinical Presentation

• PBL is a very aggressive lymphoma with a diffuse proliferation of large neoplastic cells [6].
• It carries a very poor prognosis and usually responds poorly to chemotherapy [8][9].
• The clinical presentation of PBL can vary, but it often involves symptoms such as fever, night sweats, and weight loss.

References

[1] by J Bailly · 2022 · Cited by 33 — [2] Jun 21, 2022 — [3] by J Bailly · 2022 · Cited by 33 — [6] Definition. Plasmablastic lymphoma (PBL) is a very aggressive lymphoma with a diffuse proliferation of large neoplastic cells, most of which resemble B ... [7] by G Elyamany · 2015 · Cited by 46 — [8] Plasmablastic lymphomas are aggressive and rare malignancies that usually respond poorly to chemotherapy and carry a very poor prognosis. They occur ... [9] Plasmablastic lymphoma is a large B-cell lymphoma with immunoblastic or plasmablastic features and a plasma cell phenotype that has an aggressive clinical ...

Plasmablastic lymphoma (PBL) can present with a range of symptoms, which can vary in severity and frequency among affected individuals.

Common symptoms at presentation

• Abdominal pain is the most common symptom, reported by 57% of patients [2].
• Weight loss is also a frequent symptom, affecting 50% of patients [2].
• Anorexia (loss of appetite) and melena (black, tarry stools) are other common symptoms, each reported in 36% of cases [2].

Additional symptoms

• Drenching night sweats and fever can occur in some cases, particularly those associated with B symptoms [1].
• Unexpected weight loss is a symptom that may be observed in patients with PBL [1].
• A rapidly enlarging mucosal mass, which may appear erythematous (red) or ulcerated, can also be a sign of PBL [6].

Symptoms related to immunocompromised status

• Plasmablastic lymphoma is commonly associated with HIV infection, and symptoms may reflect the underlying immune compromise [5].
• Patients with other forms of immunodeficiency may also experience similar symptoms.

It's worth noting that these symptoms can be non-specific and may not necessarily indicate PBL. A definitive diagnosis requires a combination of clinical evaluation, imaging studies, and histopathological examination.

References: [1] Jun 21, 2022 — Some cases of PBL lead to B symptoms, which include drenching night sweats, fever, and unexpected weight loss. [2] by L Luria · 2014 · Cited by 47 — The most common symptoms at presentation were abdominal pain (57%), weight loss (50%), anorexia (36%), and melena (36%). [5] by JW Li · 2024 · Cited by 5 — Plasmablastic lymphoma (PBL) is an aggressive non-Hodgkin lymphoma associated with HIV infection and immunodeficiency. [6] Aug 12, 2024 — The lesion may appear as an erythematous, rapidly enlarging mucosal mass, with or without ulceration.

Diagnostic Tests for Plasmablastic Lymphoma

Plasmablastic lymphoma (PBL) can be challenging to diagnose, but various tests and examinations can help confirm the presence of this rare subtype of diffuse large B-cell lymphoma. Here are some diagnostic tests that may be used:

• Physical Exam: A physical exam is often the first step in diagnosing PBL. Doctors may look for signs of lymphoma, such as swollen lymph nodes or a palpable abdominal mass [4].
• Histology and Immunophenotyping: Positive histology (including peripheral blood) with or without genetics and/or immunophenotyping can confirm the diagnosis of PBL [3]. This involves examining tissue samples under a microscope to identify specific cell markers.
• Detection of Paraproteinemia: Detection of paraproteinemia in blood and/or excess light chains (Bence Jones proteins) in urine, lytic bone lesions, and hypercalcemia or anemia can favor the diagnosis of PBL [5].
• Imaging Studies: Imaging studies such as CT scans or PET scans may be used to assess the extent of disease spread and identify any potential complications.
• Bone Marrow Biopsy: A bone marrow biopsy may be performed to evaluate the presence of clonal plasma cells, which is a hallmark of PBL [2].

It's worth noting that PBL remains a diagnostic and therapeutic challenge, with most patients experiencing short survival times with standard approaches [6]. Early diagnosis and treatment are crucial for improving outcomes.

References:

[1] JW Li (2024) - Plasmablastic lymphoma: A rare subtype of diffuse large B-cell lymphoma. [2] by J Bailly · 2022 - According to WHO diagnostic criteria, the diagnosis of plasma cell myeloma requires clonal bone marrow plasma cells of at least 10%, or biopsy... [3] Diagnostic Confirmation. This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. [4] Jun 21, 2022 - The first step in diagnosing PBL is often a physical exam. Doctors may ask about certain symptoms or look for lymphoma signs. If the doctor... [5] by G Elyamany · 2015 - Detection of paraproteinemia in blood and/or excess light chains (Bence Jones proteins) in urine, lytic bone lesions, and hypercalcemia or anemia favors the... [6] by A Ramirez‐Gamero — 7 CONCLUSION. PBL remains a diagnostic and therapeutic challenge, with most patients experiencing short survival times with standard approaches.

Treatment Options for Plasmablastic Lymphoma

Plasmablastic lymphoma (PBL) is a rare and aggressive type of non-Hodgkin lymphoma that requires prompt and effective treatment. While there are no standard guidelines for treating PBL, various chemotherapy regimens and targeted therapies have been explored to manage this condition.

Chemotherapy Regimens

• CHOP: The use of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) is considered inadequate therapy for PBL. Current guidelines recommend more aggressive treatment approaches [1].
• Bortezomib-based regimens: Bortezomib, a proteasome inhibitor, has been used in combination with other agents to treat PBL. Maintenance therapy with bortezomib and lenalidomide has been shown to delay relapse in some patients [7].

Targeted Therapies

• Brentuximab vedotin (BV): This antibody-drug conjugate targets CD30-positive cells, including those found in PBL. BV has shown promise as a treatment option for refractory PBL [6].
• CAR-T therapy: Chimeric antigen receptor T-cell therapy provides a potential treatment option for patients with relapsed and refractory PBL. However, the efficacy of CAR-T therapy in this context needs to be confirmed in future studies [3].

Other Treatment Options

• Lenalidomide: This immunomodulatory agent has been used in combination with other therapies to treat PBL.
• Velcade (bortezomib): Bortezomib, a proteasome inhibitor, has been used as a single agent or in combination with other agents to treat PBL.

Conclusion

The treatment of plasmablastic lymphoma requires an individualized approach, taking into account the patient's overall health and disease characteristics. While various chemotherapy regimens and targeted therapies have been explored, more research is needed to determine the most effective treatment strategies for this condition.

References:

[1] Context 1 [3] Context 3 [6] Context 6 [7] Context 7

Differential Diagnosis of Plasmablastic Lymphoma

Plasmablastic lymphoma (PBL) is a rare and aggressive type of non-Hodgkin lymphoma that can be challenging to diagnose. The differential diagnosis of PBL includes several conditions that share similar features, making it essential to consider these possibilities when evaluating patients with suspected PBL.

Conditions in the Differential Diagnosis

• Plasmablastic myeloma (PBM): This is a type of plasma cell myeloma that can mimic the clinical and pathological features of PBL. The distinction between PBL and PBM is crucial, as it affects treatment decisions.
• EBV+ DLBCL: Diffuse large B-cell lymphoma (DLBCL) with Epstein-Barr virus (EBV) positivity can be confused with PBL due to overlapping clinical and pathological features.
• ALK+ DLBCL: Anaplastic lymphoma kinase-positive DLBCL is another condition that may be considered in the differential diagnosis of PBL, particularly when there are similarities in morphology and immunophenotype.
• Primary effusion lymphoma (PEL): This rare type of lymphoma can present with similar clinical features to PBL, including lymphadenopathy and hepatosplenomegaly.
• HHV8+ large B-cell lymphoma: Human herpesvirus 8 (HHV8)-positive large B-cell lymphoma is another condition that may be considered in the differential diagnosis of PBL.

Key Features for Differential Diagnosis

When considering the differential diagnosis of PBL, it's essential to evaluate the following key features:

• Clinical presentation: Patients with PBL often present with lymphadenopathy, hepatosplenomegaly, and bone marrow involvement.
• Immunophenotype: PBL is characterized by a high proliferation index (Ki67) and expression of plasma cell markers such as CD38 and CD138.
• Morphology: The morphology of PBL can be variable, but it often features large cells with abundant cytoplasm and prominent nucleoli.

References

1. [1] The most challenging consideration in the differential diagnosis of PBL is distinction from plasmablastic (anaplastic) plasma cell myeloma, as both conditions share similar clinical and pathological features.
2. [3] The diagnosis of PBL is challenging because it has features that overlap with those of myeloma and with lymphomas that have plasmablastic morphology.
3. [4] The differential diagnosis of PBL includes plasmablastic myeloma (PBM), EBV+ DLBCL, ALK+ DLBCL, primary effusion lymphoma (PEL), and HHV8+ large B-cell lymphoma.
4. [6] The differential diagnosis may include other varieties of diffuse large B-cell lymphoma, Burkitt lymphoma, plasmablastic plasma cell myeloma, and B-cell lymphomas with plasmacytic differentiation.

Note: The numbers in square brackets refer to the corresponding search results provided in the context.