Non-Hodgkin lymphomas represent about 60 % of childhood lymphomas and 8-10 % of cancers that occur before the end of adolescence. Most often, the disease appears de novo but can develop in patients who have constitutional or acquired immune disorders, especially after certain viral infections (Epstein-Barr virus, HIV), or specific genetic syndromes (ataxia-telangiectasis syndrome, Bloom syndrome, Wiskott-Aldrich syndrome) (see these terms).

The 4 most common forms of non-Hodgkin lymphoma are:

• Burkitt lymphoma (40 % of cases) [MIM 113,970]: from germ cells of the B-line; translocation of the MYC gene (8q24.21) caused by the EBV oncogene. It is characterized by a rapid development (short doubling time).
  Different forms:

• sporadic Burkitt lymphoma
• endemic Burkitt lymphoma: in Africa
• Burkitt's immunodeficiency-related lymphoma: in the context of AIDS.
• Burkitt's leukemia
  
  

• lymphoblastic lymphoma (30 % of cases): from the germ cells of the T-line;  similar to acute lymphoblastic leukemia (see this term). In fact, the terms leukemia or lymphoma can be used depending on the presentation of the disease : in both situations, the same population of blast cells is involved but the clinical presentation differs :

-        lymphoblastic leukemia : the bone marrow is invaded and there is a leukemic phase

-        lymphoblastic lymphoma : the bone marrow is less involved but there are leukemic masses (lymph nodes, mediastinum, GI tract…)

• B-cell large cell lymphoma (20 % of cases): from germ cells of the B-line. More often in adolescents.
• anaplastic large cell lymphoma (10 % of cases): from the germ cells of the T or B-lines (20 %).

Childhood lymphomas are more aggressive than adult lymphomas and are often diagnosed in advanced stages of the disease.

Clinical presentation: painless but rapidly invasive polyadenopathy with numerous extra-ganglionary manifestations depending on digestive, mediastinal, spinal and/or cerebral involvement. This result in an important symptomatic polymorphism with three major acute complications:

• superior vena cava compression syndrome due to an anterior mediastinal mass
• paraplegia secondary to the invasion of the spinal cord or the collapse of an involved vertebra;
• metabolic syndrome caused by spontaneous or treatment-induced tumor lysis syndrome (see this term): hyperuricemia, hyperphosphatemia, hyperkalemia and hypercalcemia (see this term)

The diagnosis is biological and the treatment depends on the evaluation of the extension and/or the presence of acute complications.

Lymphomas are classified into 4 stages (St. Jude classification):

Stage 1

The lymphoma remains located in only one site: it is either extraganglionary or in a group of lymph nodes, such as those of the neck, groin or axilla. No lymphoma is present in the chest or abdomen.

Stage 2

The lymphoma is not present in the chest, but one of the following statements applies:

• the lymphoma is found in a group of lymph nodes and there is one extraganglionary tumor.
• the lymphoma is found in at least two groups of lymph nodes that are all located on the same side of the diaphragm.
• there are two extraganglionary tumors that are both located on the same side of the diaphragm.
• there is a tumor in the digestive tract that can be removed by surgery. The lymphoma may also have spread to nearby lymph nodes.

Stage 3

One of the following statements applies.

• there are two extra-ganglionary locations: one is located above the diaphragm and the other under the diaphragm.
• the lymphoma is found in at least two groups of lymph nodes on either side of the diaphragm.
• the lymphoma is found in the chest.
• the lymphoma is found in the abdomen and cannot be removed by surgery.
• the lymphoma is found in an area near the spine.

Stage 4

The lymphoma involves the central nervous system (brain, spinal cord), bone marrow or both.

Treatment: systemic chemotherapy combined with intrathecal chemotherapy. In some cases (Burkitt's lymphoma and large B-cells) immunotherapy (rituximab) or tyrosine-kinase inhibitors (crizotinib) are often associated with chemotherapy. At the beginning of the treatment, hyperhydration and anti-uricemia drugs (allopurinol or, urate-oxidase) should be administered to control the metabolic effects of tumor lysis. Emergency surgical decompression of the spinal cord  is sometimes necessary.

Despite the very aggressive nature of these lymphomas, their overall prognosis is good to excellent depending on whether the lymphoma remains localized (survival rate > 90 %) or is very invasive (survival rates between 60 and 90 %).

Anesthetic implications:

-        diagnostic process: pancytopenia ? presence of a mediastinal mass ? (a chest X-Ray is mandatory, to be supplemented with a CTscan and a  echocardiography if the chest X-Ray is abnormal). Pediatric lymphomas often grow rapidly (short doubling time), so a very recent imaging is required.

-        major risk of tumor lysis syndrome: uricemia ? kaliemia ? Avoid the administration of corticosteroids (prophylactic antiemetic dexamethasone, for example, without the advice of a hematologist)

-        long-term venous access: Broviac or Hickman, "PICC," an acronym for Peripherally Inserted Central Catheters), or implantable chambers (port-a-cath).

-        during treatment: total blood count, immunosuppression, side effects of treatment (thrombocytopenia, anemia, febrile neutropenia, neuropathy, mucitis, etc.)

-        after recovery/remission: echocardiography (toxicity of anthracyclines)

Reference:

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Updated: March 2021