Small Lymphocytic Lymphoma - Etiology, Clinical Features, and Treatment

What Is Small Lymphocytic Lymphoma?

Small lymphocytic lymphoma (SLL) is a cancer affecting the lymph nodes and minor organs. It specifically affects B-cell lymphocytes, specialized white blood cells that assist the body to fight against infection. Small lymphocytic lymphoma causes the body to develop immature, malignant B-cells in the lymph nodes. There are usually no symptoms in the early stages, although the disease can cause lymph node growth over time. Small lymphocytic lymphoma is diagnosed using a combination of blood testing, imaging scans, and tissue biopsy.

What Is the Classification of Small Lymphocytic Lymphoma?

Small lymphocytic lymphoma and chronic lymphocytic lymphoma represent the various forms of the same disease. Although blood testing can assist in distinguishing the disorders, the therapy for each is essentially the same. Small lymphocytic lymphoma is a type of non-Hodgkin lymphoma. It is closely related to another kind of blood cancer known as chronic lymphocytic leukemia (CLL), which also affects B-cells. Their location is the primary distinction between small and chronic lymphocytic lymphoma. Small lymphocytic lymphoma causes the proliferation of cancer cells in the lymph nodes and bone marrow. Conversely, with chronic lymphocytic lymphoma, cancer cells are primarily seen in the bloodstream and bone marrow.

What Is the Etiology of Small Lymphocytic Lymphoma?

The exact cause of small lymphocytic lymphoma is unknown, but it is thought to be caused by a combination of genetic mutations, some of which may be inherited. According to current research, up to 80 percent of people with small lymphocytic lymphoma have some form of chromosome abnormality. The deletion of chromosome 13q14, which results in a missing copy of genetic material on chromosome 13, is another prevalent cause of small lymphocytic lymphoma. All congenital abnormalities are frequently handed down via families.

How Does Small Lymphocytic Lymphoma Affect the Body?

The quantity of healthy blood cells in the blood that circulates throughout the body can be reduced by SLL/CLL that builds up in the spleen and bone marrow. Fatigue, shortness of breath, and a propensity to bleed or bruise can result from low platelet counts in the bloodstream (thrombocytopenia) and reduced red blood cells (anemia). Red blood cell or platelet transfusions may be necessary to treat these symptoms. Bone marrow failure is the name given to this condition, which is brought on by malignant B cells taking over healthy bone marrow.

What Are the Clinical Features of Small Lymphocytic Lymphoma?

The clinical features of small lymphocytic lymphoma are as follows:

• Swollen lymph nodes (lymphadenopathy) are usually felt in the armpits, neck, and groin.

• Bruising.

• Swollen and painful abdomen due to an enlarged spleen or liver.

• Fatigue.

• Night sweats.

• Fever.

• Weight loss.

What Is the Difference Between Leukemia and Lymphoma?

Although both lymphoma and leukemia are types of blood cancer, they have distinct effects on the body. Cancer of the bone marrow cells that produce blood is known as leukemia. Although it occasionally affects other blood cells, it primarily affects white ones. Lymphoma originates from the immune system and impacts lymph nodes and lymphocytes, a type of white blood cell. B cells and T cells constitute the two primary subtypes of lymphocytes. Hodgkin lymphoma and non-Hodgkin lymphoma are the two primary forms of lymphoma. They have an impact on several kinds of white blood cells.

How Is Small Lymphocytic Lymphoma Diagnosed?

The commonly used methods to diagnose small lymphocytic lymphoma are as follows:

1. Physical Examination

• The healthcare professional records the medical history, and symptoms such as weight loss, night sweats, or fever are reviewed.

• Palpation around the area around the neck is done to look for enlarged cervical lymph nodes, axillary lymph nodes, and inguinal lymph nodes.

• The lack of discomfort in the afflicted lymph nodes is a significant indicator of lymphoma.

• A key sign of lymphoma is the absence of pain in the affected lymph nodes, and the nodes will be mobile when touched.

• Liver and spleen enlargement is also checked; if present, it indicates the severity of the disease.

2. Biopsy and Bone Marrow Tests:

• The doctor may also recommend a lymph node biopsy. The primary test for SLL diagnosis is this one.

• After removing the lymph node, the physician examines it through a microscope for cancerous growths.

• There are a lot of lymph nodes close to the skin's surface. In that scenario, patients will receive a skin-numbing injection from the physician. Subsequently, a cut will be made, and a lymph node will be removed.

• To determine the extent of the cancer, the physician might also do two bone marrow tests: a bone marrow aspiration and a biopsy. These are typically done with a single surgery to remove hip bone marrow.

3. Blood Examinations

• In small lymphocytic lymphoma, lymphadenopathy might appear and disappear in the early stages or affect one portion of the body more than another. As a result, blood tests are required to diagnose small lymphocytic lymphoma.

• A complete blood count, a panel of blood tests that examines the number and comparative ratios of various cells in a blood sample, is critical.

• The initial indication of small lymphocytic lymphoma is lymphocytosis (excessively high lymphocyte count).

• The difficulty in identifying small lymphocytic lymphoma is that many healthy persons who do not have the condition have lymphocyte counts in the same range.

• As a result, healthcare providers need to examine the red blood cells and look for signs of anemia (lack of hemoglobin in the blood), thrombocytopenia (decrease in platelet count), or bone marrow suppression.

4. Computed Tomography (CT)

• These scans provide three-dimensional images of inside structures by combining numerous coordinated X-ray pictures.

• The CT scan uses a contrast dye, injected into a vein or taken orally, to help visualize enlarged lymph nodes.

• Swollen lymph nodes are found using these scans in the chest (mediastinal lymphadenopathy) and abdomen (mesenteric lymphadenopathy).

5. Immunophenotyping

• This test can identify and categorize proteins on the surface of cells.

• These categories can assist pathologists in identifying small lymphocytic lymphoma and distinguishing it from other lymphomas.

What Is the Treatment for Small Lymphocytic Lymphoma?

It is advised to undergo genetic testing for SLL/CLL before beginning treatment. This is crucial in assisting the treatment team in selecting the best course of action because it shows any chromosomal abnormalities or particular gene alterations before therapy. The standard approach to treating people who are symptom-free and in good health is to "watch and wait" (active observation alone). This entails scheduling frequent meetings with the medical staff, who will monitor the patient's progress. Treatment must start if the patient develops symptoms, becomes ill, has big tumors, or experiences bone marrow failure.

1. Chemotherapy - Chemotherapy is successful early in treatment and after relapse. Most chemotherapeutic drugs used in the treatment are:

• Fludarabine.

• Cyclophosphamide.

• Rituximab.

2. Targeted Therapy - Medication designed specifically to identify and eliminate cancer cells is used in targeted therapy. They target cancer cell surface proteins that affect cell division.

3. Monoclonal Antibody Treatment - These medications precisely target cancer cells and function similarly to artificial antibodies. They aid in their destruction by the immune system. These medicines are administered through an intravenous (IV) route. Among the options are:

• Obinutuzumab.

• Polatuzumab vedotin.

• Ofatumumab.

• Rituximab.

• Tafasitamab.

4. Inhibitors of Bruton Tyrosine Kinase (BTK) - Bruton Tyrosine Kinase (BTK) inhibitors are a class of novel medications that work by blocking the activity of the BTK enzyme. By obstructing the B-cell receptor transmission, which leukemias and lymphomas require to proliferate and endure, they contribute to the induction of cell death. As B-cell receptor signaling is essential to the growth and survival of SLL and CLL, inhibiting this route prevents blood cancer from increasing.

5. Steroids - An essential component of SLL/CLL treatment is steroids. Among its various uses are enhancing healthy cell counts in the blood and managing autoimmune disorders.

6. Stem Cell Transplantation - Stem cell transplants may also be explored for the elderly, usually in a milder form known as reduced-intensity conditioning allogeneic stem cell transplantation, in which radiation pretreatment is minimized. However, this process extends life, reducing symptoms rather than curing the disease.

7. Assistive Care - Symptoms and adverse effects are prevented and treated with supportive treatment. Social and emotional support are also possible components. The intention is to alleviate your SLL/CLL symptoms; the illness is not being treated. Transfusions of blood or platelets may be part of this.

What Is the Differential Diagnosis of Small Lymphocytic Lymphoma?

The characteristics of SLL are similar to other kinds of lymphoma and diseases, such as:

• Acute promyelocytic leukemia.

• Mantle cell lymphoma.

• Follicular lymphoma.

• Nodal marginal zone lymphoma.

• Burkitt lymphoma.

• Lymphocyte predominant Hodgkin lymphoma.

• Diffuse large B cell lymphoma.

• Lymphocyte predominant Hodgkin lymphoma.

What Are the Risk Factors for Small Lymphocytic Lymphoma?

Aside from genetics and family history, there are several other risk factors for small lymphocytic lymphoma:

• Age: Most cases are diagnosed between the ages of 65 and 75.

• Gender: Men are approximately twice as likely as women to get SLL.

• Race: Whites are more likely than other racial or ethnic groupings to be impacted by small lymphocytic lymphoma. Around ten out of every 100,000 white people, seven out of every 100,000 Blacks, and five out of every 100,000 Hispanics are affected. Asians are seldom impacted.

Other significant risk factors are prior hepatitis C infection and having an atopic illness such as eczema (dry and itchy skin), a food allergy, or asthma (lung disorder).

Conclusion

Small lymphocytic lymphoma is a subtype of non-Hodgkin's lymphoma that affects a type of white blood cell (lymphocyte). Small lymphocytic lymphoma causes the lymphocytes to multiply in the neck, groin, and armpit lymph nodes. It is asymptomatic and tends to grow slowly. Therefore, the doctor will watch the patient's health and suggest therapy only when needed. The three components required for a successful recovery are building support systems, remaining in continuous medical treatment, and building coping skills.