obsolete monomorphic B-cell PTLD

Definition and Characteristics

Monomorphic B-cell PTLD, also known as monomorphic diffuse large B cell lymphoma-like PTLD, is a rare and severe complication after solid organ or bone marrow transplantation. It is characterized by the monoclonal proliferation of malignant lymphocytes.

• Histological Features: The lymphoma cells in this subtype are similar to those found in non-Hodgkin lymphomas such as large diffuse B-cell lymphoma or Burkitt lymphoma.
• Clinical Presentation: Monomorphic B-cell PTLD often presents with a spectrum of symptoms, including fever, weight loss, and fatigue.

Comparison with Other Subtypes

Monomorphic B-cell PTLD is distinct from other subtypes of post-transplant lymphoproliferative disorder (PTLD), such as polymorphic PTLD, which features a mixture of B and T cells. It is also different from classical Hodgkin lymphoma-type PTLD, which has a more indolent course.

Incidence and Risk Factors

The risk of developing monomorphic B-cell PTLD after solid organ or bone marrow transplantation is estimated to be higher than in the general population. However, the exact incidence and risk factors are not well established.

• Risk of Lymphoma: The risk of lymphoma in SOT recipients is estimated as 3- to 21-fold higher than that in the general population.
• Risk in Children: The risk may be even higher among children who receive a solid organ transplant, with estimates suggesting a 120-fold increase.

Treatment and Prognosis

The treatment and prognosis of monomorphic B-cell PTLD are not well established. However, it is generally considered to be a more aggressive form of PTLD compared to other subtypes.

• Treatment: Treatment options for monomorphic B-cell PTLD may include reduction of immunosuppression, chemotherapy, and/or targeted therapy.
• Prognosis: The prognosis for patients with monomorphic B-cell PTLD is generally poor, with a high risk of disease progression and mortality.

Based on the provided context, here are the signs and symptoms of obsolete monomorphic B-cell PTLD:

• Fatigue [4]
• Malaise [4]
• Mononucleosis-like symptoms [4]
• B-symptoms (fever, weight loss, night sweats, and lymphadenopathy) [4]

Additionally, monomorphic PTLD can present with various clinical manifestations, including:

• Fever
• Lymph node enlargement
• Hepatomegaly
• Splenomegaly
• Pharyngitis

It's worth noting that the signs and symptoms of PTLD can vary greatly and may resemble those of other conditions, such as infections or lymphomas not associated with immunosuppression [12].

Monomorphic PTLD is typically characterized by a non-destructive form of PTLD, which often follows polyclonal B-cells [6]. However, it's essential to note that most monomorphic PTLD cases are non-Hodgkin's lymphoma of B-cell origin [13], and the majority of these cases are CD20+ diffuse large B cell lymphoma (DLBCL) [15].

References: [4] - Symptoms may include fatigue, malaise, and mononucleosis-like symptoms, or even B-symptoms (fever, weight loss, night sweats, and lymphadenopathy). It often presents with non-specific symptoms. [6] - Monomorphic or polymorphic PTLD may follow non-destructive PTLD lesions; however, most non-destructive PTLD have polyclonal B-cells. [12] - The signs and symptoms of PTLD can vary greatly and these disorders can produce symptoms that resemble those of many other conditions including various bacterial, mycobacterial and fungal infections; a life-threatening, widespread infection of the blood (sepsis); and lymphomas that are not associated with immunosuppression. [13] - The majority of monomorphic PTLD cases are non-Hodgkin's lymphoma of B-cell origin. This retrospective study was conducted to investigate the incidence, clinical manifestation, treatment, and outcomes of monomorphic PTLD among 5,817 recipients of solid organ or allogeneic hematopoietic stem cell transplantation from five institutions. [15] - Most PTLD are of B-cell origin, with CD20+ monomorphic diffuse large B cell lymphoma (DLBCL) being the most common subtype.

Diagnostic Tests for Obsolete Monomorphic B-cell Post-Transplant Lymphoproliferative Disorder (PTLD)

Monomorphic B-cell PTLD is a type of post-transplant lymphoproliferative disorder characterized by the proliferation of mature-looking B cells. The diagnosis of this condition can be established through various diagnostic tests, including:

• Histopathological examination: This is the gold standard for diagnosing PTLD, including monomorphic B-cell PTLD. A tissue specimen obtained with surgical excisional (preferably), incisional or core biopsy should be examined to confirm the presence of lymphoproliferative disorder [1][7].
• Clonality studies: These can support the diagnosis by showing clonal immunoglobulin or T-cell receptor gene rearrangements in monomorphic PTLDs [3][5].
• Immunophenotyping: This involves studying the morphology and immunophenotype of lymphocytes to confirm the presence of B cells. Fine-needle aspiration of a pulmonary nodule, for example, can show a monomorphic population of atypical lymphocytes that stain positive for B-cell markers [8].

Routine Laboratory Tests

While not specific for PTLD, routine laboratory tests may show abnormalities in patients with this condition, including:

• Leukopenia: A decrease in white blood cell count
• Thrombocytopenia: A decrease in platelet count
• Abnormal liver function: Elevated liver enzymes or other signs of liver dysfunction
• Fecal occult blood: Presence of blood in stool

These tests can be used to support the diagnosis and monitor the progression of PTLD [4].

References

[1] M Markouli, 2022 - The diagnosis of PTLD is established through histopathological examination of tissue specimen obtained with surgical excisional (preferably), incisional or core ...

[3] Nov 12, 2015 - Clonality studies can support the diagnosis because monomorphic PTLDs typically show clonal immunoglobulin or T-cell receptor gene ...

[4] Y Zhang, 2023 - Routine laboratory tests may show leukopenia, thrombocytopenia, abnormal liver function, fecal occult blood, etc.

[5] D Dierickx, 2015 - Clonality studies can support the diagnosis since monomorphic PTLDs typically show clonal immunoglobulin or T-cell receptor (TCR) gene ...

[7] M Markouli, 2022 - The diagnosis of PTLD is established through histopathological examination of tissue specimen obtained with surgical excisional (preferably), incisional or core ...

Based on the provided context, it appears that there are various treatment options for monomorphic B-cell post-transplant lymphoproliferative disorder (PTLD). Here's a summary of the relevant information:

• Rituximab: This monoclonal antibody targets the CD20 antigen and can be used to treat PTLD. According to [4] and [6], rituximab can be effective in targeting B cells, which are often involved in PTLD. Additionally, [7] suggests that use of rituximab in high-risk patients is highly effective in preventing PTLD.
• Immunosuppression reduction: Reducing immunosuppressive therapy can result in a cure rate of approximately 25% to 50% [9]. However, this approach may not be suitable for all patients, and the effectiveness of this method can vary depending on individual circumstances.
• Chemotherapy: While chemotherapy is often used as a treatment option for PTLD, it's worth noting that some studies suggest that long-term remission and survival can be achieved without chemotherapy in pediatric patients with monomorphic B-cell PTLD [14].
• Other treatments: Other treatments such as ofatumumab (a fully human MoAB) have been approved for the treatment of chronic lymphocytic leukemia, but their effectiveness in treating PTLD is not explicitly mentioned in the provided context.

It's essential to note that each patient's situation is unique, and the most effective treatment approach will depend on individual factors such as the severity of the disease, overall health, and other relevant medical conditions. Consultation with a qualified healthcare professional is necessary for personalized advice.

References:

[4] Hickmann K (2024) [6] Hickmann K (2024) [7] Markouli M (2022) [9] Beynet DP (2004) [14] Seshadri et al. (2020)

Based on the provided context, it appears that differential diagnosis for obsolete monomorphic B-cell post-transplant lymphoproliferative disorder (PTLD) may include:

• Allograft rejection
• Polymorphic PTLD
• Monomorphic PTLD (B-cell, T-cell, or natural killer–cell types)
• Classic Hodgkin’s lymphoma–like PTLD

This information is supported by search result [14], which states that differential diagnosis includes these options.

Additionally, it's worth noting that obsolete monomorphic B-cell PTLD may be distinct from EBV-positive cases and frequently contain TP53 mutations, as mentioned in search result [15].

It's also important to consider the fact that monomorphic PTLD is characterized by the monoclonal proliferation of malignant lymphocytes, while polymorphic PTLD features a mixture of B and T cells (search result [9]).

The diagnosis of PTLD is established through histopathological examination of tissue specimen obtained with surgical excisional (preferably), incisional or core biopsy (search results [2] and [7]).