[MIM 300 221, 400 021, 236 000]

Malignant disease of the lymphoreticular system that accounts for 6 % of all childhood cancers. There is a strong female predominance. There appears to be a genetic predisposition (autosomal recessive transmission of a KLHDC8B gene mutation [3p21.31]) with an association with some HLA antigens. Viral factors are often suspected such as the herpes virus 6, the cytomegalovirus and the Epstein-Barr virus. Some states of immunodeficiency, constitutional (ataxia-telangiectasis syndrome) or acquired (HIV infection), increase the risk.

Clinical presentation: supraclavicular and cervical polyadenopathy, painless and constituted of firm lymph nodes at palpation. This polyadenopathy can extend into the mediastinum, causing a compression of the airways, of the great vessels or a pleuropericardial effusion, and, more rarely, to the peritoneal cavity. There may be progressive medullary infiltration with anemia, neutropenia and thrombocytopenia.

The clinical factors of severity include:

• unexplained fever above 39°C;
• loss of more than 10 % of body weight in the last 3 months;
• night sweats

Other non-specific symptoms with no prognostic value are often present: lethargy, anorexia, pruritus, low back pain increased by the ingestion of alcohol.

Diagnosis:  lymph node biopsy: Reed-Steinberg cells, large polynucleated cells characteristic of the disease. These monoclonal cells originate from the lymphocytic B-line germ cell .

Different histologic types:

• the form with nodular sclerosis: NS1 if Reed-Steinberg cells are rare, NS2 if they are numerous.
• the lymphocytes-rich form
• the form with mixed cellularity
• the form with lymphoid depletion.

The treatment depends on the extension of the disease (chest X-Ray, thoracoabdominal scanner, PET-scan, MRI) and on the biological disturbances (including liver function and alkaline phosphatases).

The disease is classified into four stages:

• stage I: only one lymph node area is involved;
• stage II: at least two lymph node areas on one side of the diaphragm are involved;
• stage III: lymph node areas on either side of the diaphragm;
• stage IV: involvement of one or more extralymphatic organs (lung, liver, bone, bone marrow, etc ...).

Note that the spleen is counted as a lymph node area; its invasion is noted by an 's.'

The treatment is based on chemotherapy (cyclophosphamide, procarbazine, vincristine or vinblastine, prednisone or dexamethasone, doxorubicin, bleomycin, dacarbazine, etoposide, methotrexate, cytosine arabinoside) which can be combined with low-dose radiotherapy.

With current treatments, the 5-year survival rate is around 95 %. Most relapses occur within the first 3 years. Resistant-to-treatment or relapsed patients with or without bone marrow transplant may be treated with brentuximab-vendotin (association of an antimicrotubules agent and a anti-CD30 monoclonal antibody).

Anesthetic implications:

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

-        major risk of tumor lysis: 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 (hypoplaquettosis, anemia, febrile neutropenia, neuropathy, mucitis, etc.)

-        in case of treatment with vincristine or cisplatin : check for the absence of any peripheral neuropathy before performing a regional block

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

Reference:

-        Schwartz CL.
Special issues in pediatric Hodgkin's disease.
Eur J Haematol Suppl 2005; 66:55-62.

-        Latham GJ, Greenberg RS.
Anesthetic considerations for the pediatric oncology patient- part 1: a review of antitumor therapy.
Pediatr Anesth 2010; 20:295-304.

-        Latham GJ, Greenberg RS.
Anesthetic considerations for the pediatric oncology patient- part 2: systems-based approach to anesthesia. 
Pediatr Anesth 2010; 20:396-420.

-        Latham GJ, Greenberg RS.
Anesthetic considerations for the pediatric oncology patient- part 3: pain, cognitive dysfunction, and preoperative evaluation.
Pediatr Anesth 2010; 20:479-89.

-        Gorton H, Wilson R, Robinson A, Lyons G.
Survivors of childhood cancers: implications for obstetric anaesthesia.
Br J Anaesth 2000; 85:911-3.

-        Hebl JR, Horlocker TT, Pritchard DJ.
Diffuse brachial plexopathy after interscalene blockade in a patient receiving cisplatin chemotherapy: the pharmacologic double crush syndrome.
Anesth Analg 2001;92:249-51.

-        Huettemann E, Sakka SG.
Anaesthesia and anti-cancer chemotherapeutic drugs.
Curr Opin Anaesthesiol 2005;18:307-14.

-        Ansell SM, Radford J, Connors J, Dlugosz-Danecka M et al.
Overall survival with Brentuximab Vedotin in stage III or IV Hodgkin’s Lymphoma.
New Engl J Med 2022; 387: 310-20.

Updated: January 2023