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B-Lineage Acute Lymphoblastic Leukemia of Childhood - An Overview

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B-cell acute lymphoblastic lymphoma (B-ALL) is a fast-growing cancer mainly seen in children and young adults, involving immature B-cells in the bone marrow.

Written by

Dr. Vennela. T

Medically reviewed by

Dr. Rajesh Gulati

Published At March 4, 2024
Reviewed AtApril 1, 2024

Introduction

Acute lymphoblastic leukemia (ALL) is a frequent childhood malignancy that affects B and T cells, two types of immune system cells. It usually affects B lymphocytes in youngsters. Children are more susceptible to infections if they have B-cell ALL because these cells' defenses against infections are compromised.

The spongy bone marrow, where new blood cells are produced, is where the cancer begins. The leukemia cells proliferate rapidly and occupy bone marrow space, impeding the body's ability to generate sufficient healthy cells. Chemotherapy will be given to the child to treat the illness. Some might also require a stem cell transplant. Keeping children away from sick persons while undergoing treatment is crucial to prevent infections. The child may be cleared of ALL following treatment but will still need to see their doctor regularly to ensure the condition does not recur.

What Is Leukemia?

Leukemia is a cancerous condition mostly affecting white blood cells, which aid the immune system in combating infections. The spongy substance inside the bones is called bone marrow. This process produces platelets, red blood cells, and white blood cells. The bone marrow produces malformed white blood cells that cannot carry out their intended function in leukemia. These aberrant cells cannot protect the body from pathogens. They spread quickly, taking over the bone marrow and potentially reaching other body parts, such as the brain, liver, and lymph nodes.

The most frequent malignancy among children is leukemia. It may grow quickly (acutely) or slowly (chronically). Fortunately, most kids and teenagers with leukemia can recover with proper care.

What Is the Pathogenesis of B-Lineage Acute Lymphoblastic Leukemia of Childhood?

When specific blood cells, known as lymphoid cells, grow improperly in the bone marrow, leukemia begins. Before becoming completely functional, these cells, known as B cells, usually undergo maturation. This regular mechanism interferes with in B-cell acute lymphoblastic leukemia (B-ALL) patients. As a result of their fast multiplication, the cells fail to mature normally. As a result, malignant cells begin to replace healthy ones, which might spread to other body organs.

The development of B-ALL is linked to multiple genetic alterations. AML1 and TEL gene fusion are two of these mutations that can happen even before an individual is born. The BCR-ABL fusion gene is another frequently occurring mutation. It is associated with a more aggressive type of B-ALL and initiates a number of signaling pathways that support cell division and survival.

In B-ALL, the PAX5 gene, which controls B cell development, can also develop abnormalities that cause B cells to grow into leukemia cells early on. Furthermore, B-ALL patients frequently have mutations in genes linked to the PI3K and RAS pathways, which can lead to unchecked cell proliferation and treatment resistance.

Moreover, B-ALL development is influenced by cell cycle abnormalities, namely those involving proteins such as c-MYC and nucleophosmin (NPM). These anomalies can potentially accelerate cell proliferation and circumvent typical regulatory systems.

Knowing these genetic and molecular elements aids researchers in creating focused treatments and enhances B-ALL patient outcomes.

What Are the Causes of B-Lineage Acute Lymphoblastic Leukemia in Childhood?

Doctors often are unsure of the precise cause of B-cell ALL in youngsters. On the other hand, a few factors may make this illness more likely to strike. These include receiving chemotherapy as a cancer treatment or being subjected to large amounts of X-rays or other radiation. Children with specific genetic abnormalities, such as Down syndrome, are also more likely to develop ALL.

What Are the Symptoms of B-Lineage Acute Lymphoblastic Leukemia in Childhood?

ALL symptoms begin when leukemia cells overtake bone marrow and displace healthy blood cells. The symptoms may differ depending on the ratio of healthy blood cells to cancerous blood cells. Kids who have B-cell ALL may:

  • Feel extremely exhausted.

  • Bleed or bruise more easily.

  • Get infections more frequently.

  • Lose their appetite.

Additional signs and symptoms may consist of:

  • Pain in the bones or joints.

  • Limping.

  • High temperature.

  • Pale complexion.

  • Petechiae, which are red or purple blotches on the skin.

  • Inflammatory lymph nodes in the groin, underarms, or neck.

The liver and spleen may enlarge and cause the abdomen to bulge if the cancer cells spread there. Boys occasionally may also detect an increase in the size of their testicles.

How Is B-Lineage Acute Lymphoblastic Leukemia of Childhood Diagnosed?

To diagnose B-cell ALL in a child, the physician may ask:

  • The child's changes are initially noticed.

  • Signs and symptoms the youngster is exhibiting.

  • If any other family members have ALL.

  • If the child suffers from Down syndrome or any other hereditary illness.

In addition, by counting blood cells and looking for abnormal white blood cells, the doctor may perform blood tests to identify the kind of ALL.

A bone marrow test could also be conducted in which the physician obtains samples from the spine or leg. The young patient will be given a local anesthetic while lying down on a table. Subsequently, the physician takes a small sample of liquid bone marrow for microscopic analysis using a very fine needle. White blood cells are evaluated for size and form; undeveloped cells may indicate B-cell ALL.

How Is B-Lineage Acute Lymphoblastic Leukemia of Childhood Treated?

Since this kind of ALL grows rapidly, treatment must begin right away. The ideal place to go is a hospital that treats pediatric malignancies. There are phases to the treatment:

  • Induction: The young patient will visit the hospital to get chemotherapy medication, which destroys leukemia cells. Stopping the cancer from spreading is the aim of putting it into remission. This helps the bone marrow make healthy blood cells again, but it is not a cure. After about a month of treatment, the majority of children enter remission.

  • Consolidation or Intensification: To eradicate any cancer cells that may still be present, further chemotherapy will be administered. This stage lasts for four to six months.

  • Maintenance: Ongoing treatment eradicates all cancer from the body and stops it from returning. Chemotherapy at lower doses is administered for two to three years.

The doctor will continuously monitor the child's progress during treatment. Early on, a bone marrow examination will assess how well leukemia responds to therapy. Blood tests will be performed to confirm the complete eradication of the malignancy. Additional treatment can be required if cancer cells are discovered.

In certain instances:

  • Stem Cell Transplant: A stem cell transplant may be required if the cancer recurs or if the first course of treatment is unsuccessful. Replacing the bone marrow entails receiving healthy cells from a donor, either a matched donor or a close family. After the transplant, the child might need to stay in the hospital until their immune system fully heals. The fresh cells are administered through an IV.

  • CAR T-Cell Therapy: This gene therapy may be an alternative in situations where previous treatments have failed. It is known as CAR T-cell therapy. This entails utilizing the child's own T cells immune cells that have been altered to combat cancer more effectively. After being altered, these cells are reintroduced into the body to destroy cancer cells.

Conclusion

The most prevalent cancer in children is B-ALL. Even with increased cure rates, some patients continue to recur. For this reason, knowledge of the molecular mechanisms underlying B-ALL and its relapse is essential. Current treatments only work for some patients. Improved outcomes and increased efficacy of chemotherapy may be possible with new immune-targeting treatments. Biomarkers can be used to guide dose and medication adjustments and track the efficacy of a treatment. Planning is crucial for clinical trials to create more effective immune-targeting medications and regimens.

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Dr. Rajesh Gulati
Dr. Rajesh Gulati

Family Physician

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