Non Hodgkin Lymphoma is a malignant disease characterized by the uncontrolled proliferation of lymphocyte series cells. Although it is most commonly seen in organs such as lymph nodes, Peyer's patches and spleen, bone marrow involvement is common in children. Rarely, bone and central nervous system lymphoma have also been reported. NHL accounts for 6-7% of childhood cancers. It ranks 3rd in frequency among cancers and is seen with a frequency of 10.5 per million.
It occurs more frequently in Middle Eastern and African countries due to endemic Burkitt lymphoma.
All people under the age of twenty. NHL constitutes 45% of lymphoma cases. While there has been no change in the incidence in children under the age of 15 in the last twenty years, a 50% increase in the incidence has been detected between the ages of 15-19.
The male/female ratio is 2.5/1. While it is seen at a constant rate at all ages, an increase in its incidence is detected between the ages of 15-19.
Some risk factors related to the disease have been identified: The incidence of NHL increases in immunodeficiency diseases. Immunosuppression during bone marrow or solid organ transplantation increases the risk of NHL. NHL is more common in patients taking epilepsy medications such as diphenylhydantoin. The risk of occurrence increases in patients receiving chemotherapy and radiotherapy. Epstein-Barr virus (EBV) and HIV virus infections increase susceptibility to NHL.
Pathological classification
The histological classification of childhood NHL is as follows:
- Lymphoblastic lymphoma
- Notched cell
- Non-notched cell
- T precursor cell (90%)
- B precursor cell (10%)
- Small round cell lymphoma (Diffuse and undifferentiated)
- Burkitt lymphoma
- Burkitt-like lymphoma
- Large cell lymphoma
- Diffuse large cell lymphoma, B and T
- Anaplastic large cell lymphoma, usually T
Lymphoblastic lymphoma usually occurs in the anterior mediastinum at a rate of 50-70%. All N 30-35% of HLs fall into this group. Conditions such as fluid accumulation in the lungs, enlargement of the lymph nodes in the neck, armpit and groin, and sometimes in the abdomen may occur. Chromosomal anomalies such as t(10;14), t(11;14) and t(1;14), t(1;19) and t(8;14) are seen. Small round cell lymphoma is usually B cell and 90% It probably occurs in the abdomen. 40-50% of all NHLers fall into this group. The most common disease that causes intestinal obstruction in children over the age of 6 is NHL. Chromosome anomalies such as t(8;14), t(8;22), and t(2;8) are usually observed.
Large cell lymphomas, which occur at a rate of 15-20 percent, are usually in the abdomen and sometimes They are also seen in uncommon areas. Anaplastic large cell lymphoma often arises from areas such as skin, central nervous system, lymph nodes, lung, testicle, muscle, gastrointestinal tract and bone.
Clinical findings
Clinical findings It varies depending on where the tumor occurs. When it occurs in the abdomen, symptoms such as abdominal pain, nausea and vomiting, constipation or diarrhea, abdominal swelling, palpable mass, intestinal obstruction, peritoneal infection, fluid accumulation in the abdomen, bleeding, jaundice, liver and spleen enlargement.
When it occurs in the head and neck region, it shows symptoms such as enlargement of the lymph nodes and parotid gland in this region, swelling in the chin and unilateral tonsil enlargement. Nasal congestion or discharge, inability to smell, and cranial nerve paralysis may also occur. When it occurs in the chest cavity, patients present to the clinic with superior vena cava syndrome. This syndrome is characterized by findings such as fullness in the neck veins, edema in the neck and face, difficulty breathing, headache and feeling dizzy, and loss of consciousness when bending over. It occurs as a result of the lymphoma mass compressing the superior vena cava. Tumors originating from the chest cavity tend to involve the bone marrow, turn into leukemia, and involve the central nervous system and reproductive organs. Most of them are of T lymphocyte origin. Fluid accumulating between the lung and heart membranes may cause breathing and circulation problems.
If the tumor also compresses the trachea, patients are placed in a lying position or receive general anesthesia. They may face the risk of death when they are diagnosed.
Fever and weight loss are rare findings other than anaplastic large cell lymphoma.
Diagnosis
NHL The patient is examined according to the evaluation scheme listed below. After a detailed history and physical examination, complete blood count, peripheral smear examination, serum electrolytes, kidney and liver functions are evaluated. Blood is sent for serum LDH levels. Serological examination is performed for EBV, CMV, Herpes virus, Varicella virus, HIV and hepatitis A, B, C viruses. Bilateral bone marrow aspiration and biopsy from the patient, examination of the cerebrospinal fluid taken by lumbar puncture and, if any, fluid taken from the abdomen, lungs or around the heart are performed. Chest radiography, lung tomography if necessary, abdominal ultrasonography, contrast-enhanced abdominal tomography, gallium and bone scintigraphy, MRI of the relevant area if necessary, and dental examination should be performed in patients with Burkitt lymphoma.
Staging
A detailed staging should be performed to determine how much the disease has spread, the degree of organ involvement and biochemical deterioration. Surgical staging has no place in NHL. Since the disease arises from more than one region, the main treatment method is chemotherapy. Testicular involvement is seen in less than 4% of men. NHL is not treated only with radiotherapy depending on the location of involvement.
Treatment
Two life-threatening complications are observed in children with NHL2.
- Superior vena cava syndrome (mentioned above ).
- Tumor lysis syndrome. This picture is a metabolic imbalance that occurs after cell destruction caused by chemotherapy in Burkitt or Burkitt-like lymphoma.
Approach to tumors in the chest cavity
The patient should always be kept in a sitting position and, if possible, General anesthesia should not be given. For diagnosis:
> - Tilt If there is fluid in each lung, a diagnosis is made by removing fluid or removing a peripheral lymph node with a biopsy.
If the patient's clinical condition prevents intervention, then the patient is treated with limited dose radiotherapy and corticosteroids and after the mass is sufficiently reduced, general Diagnostic intervention should be performed under anesthesia.
Correction of pericardial effusion
Pericardial effusion can turn into life-threatening tamponade. If tamponade occurs, pericardial fluid should be drained and cytological examination of the fluid should be performed. Chemotherapy should be started immediately to prevent fluid accumulation.
Approach to gastrointestinal complications
Abdominal disease is usually seen in burkit lymphoma or burkit-like lymphoma. The tumor may obstruct the small intestine at the level of the terminal ileum or cecum or cause the bowel segments to become intertwined. In about 25% of cases, the tumor can be removed surgically. Chemotherapy should be started immediately after surgery. In case of gastric bleeding or perforation, endoscopic examination should be performed to understand the location and amount of bleeding and how much the tumor and the area is involved. In high-risk patients, the tumor should be removed surgically.
The proliferation of lymphoblasts occurs very rapidly and in Burkitt lymphoma type, the number of cells usually doubles within 24 hours. The reason why tumor growth is so rapid is that the drugs used in the treatment are given in divided doses and some in the form of continuous infusion. In treatment, drugs such as steroids, vincristine, daunorubicin, doxorubicin, asparaginase, cyclophosphamide, cytarabine, methotrexate and mercaptopurine are used in various schemes and in various combinations depending on the type and stage of the disease. Central nervous system prophylaxis should be given to patients by giving intrathecal chemotherapy.
Radiation therapy is not a form of treatment that is generally used electively. It should only be used in cases that are resistant to initial chemotherapy and are life-threatening, such as superior vena cava syndrome. Cranial radiotherapy should not be given in B-cell NHL with central nervous system involvement. The use of cranial radiotherapy for central nervous system prophylaxis is controversial.< br />
The role of surgery in NHL is limited. If the tumor can be completely removed with surgery without causing any problems for the patient, it can be tried (localized intestinal lymphoma). Patients with diffuse lymphoma are not candidates for surgery.
Prognosis depends on the appropriateness of the protocol used. The 5-year event-free survival in localized lymphoblastic lymphoma is around 85%. In localized T-cell lymphoma, this rate is around 100%. The 6-year event-free survival in stage III and IV lymphoblastic lymphoma is 90-95%. In localized Burkit, Burkit-like, and diffuse large cell lymphoma, 3-year event-free survival decreases from 98% to 89% depending on increasing stage. In anaplastic large cell lymphoma, 5-year event-free survival varies between 579 and 100%, depending on the stage of the disease.
Treatment of recurrent disease
When there is recurrent disease, the location of recurrence, tumor histology, initial The prognosis is poor regardless of factors such as prognostic factors at diagnosis, previous treatment given, or how long after diagnosis there is a recurrence. For this reason, it is important to provide the first treatment in the most appropriate way. After starting treatment with chemotherapy in lymphoblastic lymphoma and B-cell lymphoma, stem cells should be collected and treatment should be continued with stem cell transplantation after complete or partial recovery is achieved. Chemotherapy-resistant patients generally have a low chance of recovery with stem cell transplantation. Patients with CD20-positive B-cell lymphoma may benefit from the addition of rituximab to treatment. Stem cell transplantation generally does not increase treatment success in anaplastic large cell lymphomas. Patients can be put into remission with chemotherapy. Cis-retinoic acid + interferon alpha may keep some patients in remission for a long time.
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