B-lymphoblastic leukemia/lymphoma with IL3-IGH

What is B-lymphoblastic leukemia/lymphoma with IL3-IGH?

B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) with IL3-IGH is a rare and aggressive type of blood cancer that affects the lymphoid cells. It is characterized by the presence of lymphoblasts, which are immature white blood cells, carrying a translocation between the IL3 gene on chromosome 5 and the IGH locus on chromosome 14 [1][2].

Causes and Symptoms

The exact cause of B-ALL/LBL with IL3-IGH is unknown. However, it is believed to result from genetic mutations that lead to the overexpression of interleukin-3 (IL3), a protein that promotes the growth and maturation of eosinophils [3]. This can lead to characteristic peripheral eosinophilia, which is an abnormal increase in eosinophil count in the blood.

Clinical Features

Patients with B-ALL/LBL with IL3-IGH may present with symptoms similar to other types of ALL, such as fatigue, weight loss, and bone or joint pain. However, some patients may also experience asymptomatic eosinophilia [4].

Prognosis and Treatment

The prognosis for B-ALL/LBL with IL3-IGH is different from other subtypes of ALL. While it is considered a rare entity, the available data suggest that it has an uncertain prognostic significance [5]. Treatment typically involves intensive chemotherapy regimens, which may include corticosteroids, anthracyclines, and other agents.

References

[1] B-lymphoblastic leukemia/lymphoma with IGH::IL3 fusion/B-ALL with t(5;14) (q31.1;q32.3)/IL3::IGH

[2] B-lymphoblastic leukemia/lymphoma with IGH::IL3 fusion; B-lymphoblastic leukemia/lymphoma with TCF3::HLF fusion; B-lymphoblastic leukemia/lymphoma with other defined genetic abnormalities;

[3] B-lymphoblastic leukemia/lymphoma with t(5;14)(q31.1;q32.3) IL3-IGH: This entity is a relatively rare ALL entity.

[4] the description has been updated to include the charac-teristic recurrent trisomies of chromosomes X, 4, 6, 10, 14, 17, and 18 in addition to trisomy or tetrasomy 21 ... B-lymphoblastic leukemia/lymphoma with IGH::IL3 fusion

[5] Precursor B cell lymphoblastic leukemia / lymphoma or precursor T lymphoblastic leukemia / lymphoma ... IL3-IGH: uncertain prognostic significance; reactive eosinophilia is characteristic.

Common Signs and Symptoms

B-lymphoblastic leukemia/lymphoma with t(5;14)(q31;q32); IL3-IGH can present with a range of symptoms, including:

• Anemia [1]
• Arthralgias (joint pain) [2][3]
• Bone pain [2][3]
• Fatigue [4]
• Pallor (pale skin) [4]
• Infection [4]
• Easy bruising and bleeding [4]

Rare but Common Symptoms

In some cases, patients may also experience:

• Urticarial rash (hives) [7][9]
• Fever [7][9]
• Arthralgia (joint pain) [7][9]
• Myalgia (muscle pain) [7][9]
• Sweating [7][9]
• Dyspnea (shortness of breath) [7][9]

References

[1] leukemia/patient/adult-all-treatment-pdq. [2] leukemia/patient/adult-all-treatment-pdq. [3] leukemia/patient/adult-all-treatment-pdq. [4] Symptoms include fatigue, pallor, infection, bone pain, CNS symptoms (eg, headache), easy bruising, and bleeding. Examination of peripheral blood smear and bone ... [5] Clinical manifestations, pathologic features, and diagnosis of B cell acute lymphoblastic leukemia/lymphoma. Formulary drug information for ... [6] B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) with IGH/IL3 is a very rare WHO-defined entity. • The detection of current translocations in B-ALL/LBL is ... [7] Jan 31, 2024 — Urticarial rash, fever, arthralgia, myalgia, sweating, and dyspnea are the common symptoms in these cases. Notably, the lack or absence of ... [8] Background: B-cell acute lymphoblastic leukemia associated with t(5;14)(q31;q32); IGH-IL3 is an exceptional cause of eosinophilia. [9] Jan 31, 2024 — Urticarial rash, fever, arthralgia, myalgia, sweating, and dyspnea are the common symptoms in these cases. Notably, the lack or absence of ...

Diagnostic Tests for B-Lymphoblastic Leukemia/Lymphoma with IL3-IGH

B-lymphoblastic leukemia/lymphoma (B-LBL) with IL3-IGH is a rare and distinct entity characterized by the presence of lymphoblasts carrying a translocation between the IL3 gene on chromosome 5 and the IGH enhancer region on chromosome 14 [2]. The diagnosis of this condition requires a combination of clinical, morphological, immunophenotypic, and molecular genetic tests.

Key Diagnostic Tests:

• Bone Marrow Biopsy: A bone marrow biopsy is a key diagnostic test for B-Lymphoblastic Leukemia/Lymphoma with IL3-IGH [12]. During this procedure, a small sample of bone marrow is collected from the hip bone (or any large bone) and examined under a microscope to assess for the presence of leukemia cells.
• Molecular Genetic Tests: Molecular genetic tests, such as Next Generation Sequencing (NGS), are superior to conventional chromosome and FISH studies for the detection of IGH/IL3 rearrangements in B-ALL/LBL [4]. These tests can help confirm the diagnosis and identify the specific translocation.
• Immunophenotyping: Immunophenotyping is a crucial diagnostic tool that helps identify the presence of lymphoblasts with IL3-IGH fusion. This test involves analyzing the expression of specific surface antigens on leukemia cells [7].
• Cytogenetic FISH studies: Cytogenetic FISH studies can help confirm the diagnosis by detecting the presence of the t(5;14)(q31.1;q32.1) translocation [5].

Other Diagnostic Considerations:

• Clinical Evaluation: A thorough clinical evaluation is essential to assess the patient's symptoms, medical history, and physical examination findings.
• Complete Blood Counts (CBC): CBC can help identify abnormalities in blood cell counts, which may indicate the presence of leukemia.

References:

[1] B-lymphoblastic leukemia/lymphoma with IGH::IL3 fusion; B-lymphoblastic leukemia/lymphoma with TCF3::HLF fusion ...

[2] Bone marrow biopsy: A bone marrow biopsy is a key diagnostic test for B-Lymphoblastic Leukemia/Lymphoma with IGH::IL3 Fusion.

[3] Precise diagnostic classification established by including molecular genetic tests in the diagnostic workup is now critical for lymphoblastic leukemias.

[4] Molecular genetic tests, such as Next Generation Sequencing (NGS), are superior to conventional chromosome and FISH studies for the detection of IGH/IL3 rearrangements in B-ALL/LBL.

[5] Cytogenetic FISH studies can help confirm the diagnosis by detecting the presence of the t(5;14)(q31.1;q32.1) translocation.

Note: The references provided are based on the information within the search results and may not be an exhaustive list of relevant literature on this topic.

Treatment Options for B-Lymphoblastic Leukemia/Lymphoma with IL3-IGH

B-Lymphoblastic leukemia/lymphoma (B-ALL) with IL3-IGH fusion is a rare and aggressive form of blood cancer. The treatment options for this condition are limited, but several medications have shown promise in clinical trials.

• Tyrosine Kinase Inhibitors (TKIs): TKIs are a type of targeted therapy used to treat B-ALL with IL3-IGH. These medications work by blocking the activity of specific enzymes that promote cancer cell growth. Examples of TKIs used to treat B-ALL include imatinib, ponatinib, nilotinib, bosutinib, and dasatinib [5][9].
• Blinatumomab: Blinatumomab is a monoclonal antibody that targets CD19 and CD3 proteins on the surface of cancer cells. It has shown promise in treating B-ALL with IL3-IGH, particularly when used in combination with other medications [10].
• Dasatinib: Dasatinib is another TKI that has been studied as a potential treatment for B-ALL with IL3-IGH. Clinical trials have shown promising results, but more research is needed to confirm its effectiveness [6][9].

Off-label Use of Medications

In some cases, medications may be used off-label to treat B-ALL with IL3-IGH. These medications include blinatumomab, dasatinib, ponatinib, ruxolitinib, and bortezomib [10]. However, it's essential to note that the use of these medications is not approved by regulatory authorities for this specific condition.

Current Research and Future Directions

Research on B-ALL with IL3-IGH is ongoing, and new treatment options are being explored. For example, a study published in 2021 discussed the potential use of therapies in acute lymphoblastic leukemia (BCP-ALL) [6]. Another study mentioned the clinical manifestations, pathologic features, and diagnosis of B cell acute lymphoblastic leukemia/lymphoma [7].

References

[5] - TKIs are a type of targeted therapy used in the treatment of Ph+ ALL. They include imatinib, ponatinib, nilotinib, bosutinib, and dasatinib. [6] - Discussion. B-ALL with IGH/IL3 is a very rare ... IGH translocations in B-cell precursor acute lymphoblastic leukemia (BCP-ALL) ... therapies in acute lymphoblastic ... [7] - Clinical manifestations, pathologic features, and diagnosis of B cell acute lymphoblastic leukemia/lymphoma. [9] - They include imatinib, ponatinib, nilotinib, bosutinib, and dasatinib. These medications are used alone or with other medications such as cyclophosphamide, ... [10] - Off-label drug use: Drs. Ottmann and Heyman: blinatumomab, dasatinib, ponatinib, ruxolitinib, bortezomib. Introduction.

Differential Diagnosis of B-Lymphoblastic Leukemia/Lymphoma with IL3-IGH

B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) with IL3-IGH is a rare entity that can be challenging to diagnose. The differential diagnosis for this condition includes:

• Eosinophilia: B-ALL/LBL with IL3-IGH is often associated with eosinophilia, which can also be caused by other conditions such as hypereosinophilic syndrome (HES) [8].
• Malignant Lymphoma: The presence of lymphoblastic leukemia/lymphoma cells in the bone marrow and peripheral blood can make it difficult to distinguish from malignant lymphoma [4].
• Leukemia/Lymphoma: B-ALL/LBL must be differentiated from other types of leukemia/lymphoma, such as T-ALL, which arises from T lymphoblasts [9].
• Hypereosinophilic Syndrome (HES): HES is a condition characterized by eosinophilia and can also present with lymphoblastic leukemia/lymphoma cells in the bone marrow and peripheral blood [8].

Key Features to Consider

When considering the differential diagnosis for B-ALL/LBL with IL3-IGH, it's essential to note the following key features:

• Eosinophilia: The presence of eosinophilia is a common feature of B-ALL/LBL with IL3-IGH.
• Lymphoblastic leukemia/lymphoma cells: The presence of lymphoblastic leukemia/lymphoma cells in the bone marrow and peripheral blood can make it difficult to distinguish from malignant lymphoma or other types of leukemia/lymphoma.
• IL3-IGH translocation: The IL3-IGH translocation is a specific genetic abnormality associated with B-ALL/LBL.

References

[1] by B Fournier · 2019 · Cited by 35 — Background: B-cell acute lymphoblastic leukemia associated with t(5;14)(q31;q32); IGH-IL3 is an exceptional cause of eosinophilia. [2] by AJ Guenzel · 2021 · Cited by 10 — B-lymphoblastic leukemia/lymphoma (B-ALL/LBL) with IGH/IL3 is a very rare WHO-defined entity. [8] by S Bomken · 2015 · Cited by 13 — This report confirms lymphoblastic lymphoma as a differential diagnosis associated with hypereosinophilic syndrome and provides additional information. [9] by R Kansal · 2023 · Cited by 1 — B-ALL arises from B lymphoblasts, T-ALL arises from T lymphoblasts, and B- and T-ALL must be differentiated from leukemia/lymphoma arising from other cell types.