What is Lymphoma?

Lymphoma is a malignant tumor of the lymphatic and hematopoietic system. It occurs when lymphocytes, due to internal or external factors, undergo certain genetic changes leading to uncontrolled growth. It can be likened to some immune cells becoming "traitors." These abnormal cells travel throughout the body via the lymphatic system and accumulate in lymph nodes, lymphatic tissues/organs, blood, or other non-lymphatic organs.

What is the Status of Lymphoma in Indonesia?

Lymphoma ranks among the top ten malignant tumors in Indonesia. In 2022, there were approximately 8,500 new cases, accounting for 45% of all hematologic malignancies. Non-Hodgkin Lymphoma (NHL) is the most common type (80%), while Hodgkin Lymphoma (HL) accounts for 20%. Among the pathological subtypes, Diffuse Large B-Cell Lymphoma (DLBCL) is the most prevalent (40%). Due to delayed diagnosis and insufficient treatment resources, the overall 5-year survival rate is approximately 55-65%, lower than the 75-85% seen in developed countries.

What are the Symptoms of Lymphoma?

1. The earliest symptom of lymphoma is often persistent, painless swelling of lymph nodes, typically occurring in the neck (60%-80% of cases), armpits (6%-20%), or groin (6%-10%).

2. Early-stage lymphoma can cause general symptoms such as intermittent low-grade fever, persistent fatigue, loss of appetite, and low mood.

3. Hodgkin Lymphoma (HL) Symptoms: HL (lymphoreticular cell sarcoma) often initially presents with an irregular low-grade fever alongside superficial lymph node swelling. Initially, these swollen nodes are often soft and movable.

4. Skin itching is also common in HL patients. It can be localized or generalized. Generalized itching may indicate that the cancer cells have spread to the mediastinum or abdomen.

What are the Conventional Diagnostic Methods for Lymphoma?

1. Blood Cell Count: Often unremarkable in early stages. Anemia may appear late or with comorbid hemolytic anemia. White blood cell count is usually normal unless bone marrow is involved; eosinophilia is more common in HL.

2. Bone Marrow Aspiration/Biopsy: Usually normal before marrow involvement. Finding Reed-Sternberg cells in HL is valuable for diagnosis.

3. Biochemical Tests: Elevated erythrocyte sedimentation rate (ESR) suggests active disease. Serum copper and ferritin levels rise with progression and fall during remission (zinc shows the opposite trend). Elevated alkaline phosphatase (ALP) may indicate liver or bone involvement. Elevated 5-nucleotidase accompanies liver involvement. Hypercalcemia suggests bone invasion.

4. Immunological Studies: HL patients may show reduced reactivity to tuberculin and other stimuli; in vitro lymphocyte transformation rates are decreased, correlating with disease progression.

5. Biopsy: Essential for definitive diagnosis. A lymph node from the lower neck or axilla is typically chosen.

6. Mediastinoscopy: Allows biopsy of mediastinal nodes via an extrapleural approach, relatively simply and safely.

7. Imaging (CT, MRI, PET-CT): Used to detect disease in thoracic/retroperitoneal/mesenteric lymph nodes and liver/spleen.

8. Laparotomy: Was used to definitively determine involvement of the spleen, liver, and abdominal lymph nodes for accurate radiotherapy planning (pathological staging). Splenectomy could also prevent radiation damage to adjacent organs during spleen irradiation.

What are the Treatment Options for Lymphoma?

Lymphoma is highly heterogeneous, with treatment outcomes varying significantly based on pathological type and stage. The main treatment modalities are listed below, but specific plans must be tailored to the individual patient.

Radiation Therapy
Indications include:

1. Stage I/II Hodgkin Lymphoma (HL) (radiotherapy alone can be very effective). Stage III typically requires combined chemoradiation.

2. For Non-Hodgkin Lymphoma (NHL): Stage I/II may be treated with radiotherapy, followed by consolidative chemotherapy. Stage III/IV is primarily treated with chemotherapy, adding local radiotherapy. This includes most early-stage low-grade/indolent lymphomas; early-stage diffuse large B-cell lymphoma; and some aggressive lymphomas like Stage IE-IIE nasal NK/T-cell lymphoma.

Chemotherapy
Lymphoma chemotherapy often uses combination regimens and may be combined with targeted therapy drugs and biological agents. Treatment protocols have improved significantly in recent years, greatly improving survival for many lymphoma types.

Bone Marrow Transplant (Stem Cell Transplant)
For patients under 60 who can tolerate high-dose chemotherapy, autologous hematopoietic stem cell transplantation may be considered for intermediate/high-risk cases. Allogeneic transplantation may be an option for some young patients with relapsed disease or bone marrow involvement.

Surgery
Role is limited to biopsy or managing complications. Splenectomy may be indicated for patients with splenomegaly causing hypersplenism (if no contraindications exist) to improve blood counts and create favorable conditions for subsequent chemotherapy.