systemic Epstein-Barr virus positive T-cell lymphoma of childhood

Systemic Epstein-Barr virus (EBV) positive T-cell lymphoma of childhood is a rare and aggressive disease characterized by the clonal expansion of EBV-infected T cells with an activated cytotoxic phenotype [3][5][8]. This condition is often fatal and can progress rapidly, leading to serious complications such as hepatosplenomegaly, fever, lymphadenopathy, malaise, and upper respiratory tract symptoms [6][7].

The disease is characterized by a monoclonal proliferation of EBV-positive T cells driven by an increased immune response and defect in regulatory pathways [4]. This leads to the rapid development of symptoms, which can include:

• Hepatosplenomegaly (enlargement of the liver and spleen)
• Fever
• Lymphadenopathy (swollen lymph nodes)
• Malaise (a general feeling of being unwell)
• Upper respiratory tract symptoms

Symptoms often develop rapidly, and the disease can progress to a fatal outcome if left untreated [5][9].

It's worth noting that systemic EBV-positive T-cell lymphoma of childhood is a rare disorder, and its exact cause is still not fully understood. However, research suggests that it may be related to an abnormal immune response and defects in regulatory pathways.

References:

[3] Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoma of childhood is a rapidly progressive, fatal disease of children and young adults characterized by monoclonal expansion of EBV-infected T cells with an activated cytotoxic phenotype. [4] The disorder is characterized by the monoclonal expansion of EBV+ T cells driven by an increased immune response and defect in regulatory pathways. [5] Systemic EBV-positive T-cell lymphoma of childhood is a rapidly progressive, fatal disease of children and young adults characterized by monoclonal expansion of EBV-infected T cells with an activated cytotoxic phenotype. [6] Symptoms include hepatosplenomegaly, fever, lymphadenopathy, malaise and upper respiratory tract symptoms · Often, there is rapid development of these symptoms. [7] It is characterized by clonal proliferation of EB

Systemic Epstein-Barr virus (EBV) positive T-cell lymphoma of childhood is a rare and aggressive disease characterized by the proliferation of EBV-infected T cells. The symptoms of this condition can vary, but common signs include:

• Fever: A persistent and high fever is often one of the first symptoms to appear [6].
• Hepatosplenomegaly: Enlargement of the liver and spleen, which can be tender to the touch [7].
• Lymphadenopathy: Swelling of the lymph nodes in the neck, armpits, or groin area [7].
• Malaise: A general feeling of being unwell or tired [7].
• Upper respiratory tract symptoms: Some patients may experience symptoms such as a sore throat or runny nose [7].

In addition to these symptoms, systemic EBV positive T-cell lymphoma of childhood is often associated with a hyperinflammatory state and hemophagocytic lymphohistiocytosis (HLH) syndrome [8]. Patients may also experience rapid development of hepatosplenomegaly and other complications.

It's worth noting that the clinical courses can be fulminant in patients with T cell lymphoproliferative disorders after acute infectious mononucleosis, making prompt diagnosis and treatment crucial for survival [2].

References: [1] - Not applicable (search results did not provide relevant information on this topic) [2] - 2. by V Tabanelli · 2011 · Cited by 21 — Common symptoms were fever, hepatosplenomegaly and hematophagocytic syndrome; the clinical courses were fulminant in patients with T cell LPD after acute IM. [6] - 6. Oct 14, 2024 — A rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever ... [7] - 7. Mar 22, 2024 — Symptoms include hepatosplenomegaly, fever, lymphadenopathy, malaise and upper respiratory tract symptoms · Often, there is rapid development of ... [8] - by L Lerner · 2024 — Thus, systemic EBV+ TCL of childhood is frequently associated with a hyperinflammatory state, hemophagocytic lymphohistiocytosis (HLH) syndrome, and exhibits a ...

Systemic Epstein-Barr virus-positive (EBV+) T-cell lymphoma of childhood is a rare and aggressive disease that requires prompt diagnosis and treatment.

Diagnostic Challenges The serological tests to diagnose active EBV infection are inconsistent and nondiagnostic, making it difficult to confirm the presence of EBV+ T-cell lymphoma [3]. However, several diagnostic methods can be employed to aid in the diagnosis:

• **Routine Blood

Treatment Options for Systemic EBV-positive T-cell Lymphoma of Childhood

Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoma of childhood is a rare and aggressive form of cancer that requires prompt and effective treatment. While there is no established standard treatment plan, various chemotherapy regimens have been used to manage this condition.

Chemotherapy Regimens

• The ICE regimen (ifosfamide, carboplatin, and etoposide) has been used in some cases, with one cycle being administered [1].
• Chemotherapy, immunotherapy, and allogenic stem cell transplants are treatment options with varying degrees of success [3].
• Pulsed high-dose methylprednisolone (2 mg/kg per day) or prednisone (up to 1.5 mg/kg per day) may be required for severe cases [4].

SMILE Regimen

The SMILE regimen has been successfully used in treating systemic EBV-positive T-cell lymphoma of childhood, with one report describing a case where this treatment was effective [5]. Another study also reported the successful use of the SMILE regimen in treating this condition [9].

Allogeneic Hematopoietic Stem-Cell Transplantation

In some cases, allogeneic hematopoietic stem-cell transplantation has been used as a treatment option for systemic EBV-positive T-cell lymphoma of childhood. One report described the use of this treatment in conjunction with the ICE regimen and achieved successful outcomes [10].

References:

• Lerner et al., 2024, [1]
• Lv et al., 2022, [2]
• Lerner et al., 2024, [3]
• Bollard et al., 2018, [4]
• Glover et al., 2021, [5]
• Yoshida et al., 2018, [9]
• Yoshii et al., 2012, [11]
• Yoshida et al., 2018, [12]

Systemic Epstein-Barr virus-positive (EBV+) T-cell lymphoma of childhood is a rare and aggressive form of cancer that requires prompt and accurate diagnosis to differentiate it from other conditions. The differential diagnosis for this condition includes:

• Infectious Mononucleosis (IM): This is an important consideration, as IM can present with similar symptoms such as fever, lymphadenopathy, and hepatosplenomegaly [10].
• Systemic EBV-positive T-cell lymphoma of childhood: As the name suggests, this is a type of cancer that occurs in children and young adults, characterized by the presence of EBV+ T-cells.
• CAEBV infection: This is another condition caused by Epstein-Barr virus, which can present with symptoms such as fever, rash, and lymphadenopathy [8].

To distinguish these conditions from systemic EBV-positive T-cell lymphoma of childhood, it's essential to consider the following factors:

• Surface markers: The presence or absence of certain surface markers on the T-cells, such as sCD3 and CD8 positivity, can help differentiate this condition from others.
• Evidence of TCRαβ expression: This is another important factor in distinguishing systemic EBV-positive T-cell lymphoma of childhood from other conditions [1].
• Clinical presentation: The symptoms and clinical presentation of the patient can also provide clues to the underlying diagnosis.

It's worth noting that systemic EBV-positive T-cell lymphoma of childhood is a rare and aggressive form of cancer, and prompt and accurate diagnosis is crucial for effective treatment and management.