PEDIATRIC LYMPHOMAS

Lymphomas are diseases in which cancerous cells form in the system that makes lymph (fluid that contains white blood cells that protect the body against infection).The outlook for patients with lymphoma depends on the type of tumor and whether the tumor has spread outside the lymph nodes (small glands that circulate lymph around the body).

Types of lymphomas:

• Hodgkin lymphomas develop when abnormal B cells (an immune system cell) accumulate in the immune system.
• Non-Hodgkin lymphomas, which are more common than Hodgkin lymphomas, occur when the body produces too many white blood cells called lymphocytes.

Symptoms of Pediatric Lymphomas

Symptoms vary depending on the type of cancer. Many of the symptoms of childhood cancers can also be symptoms of other diseases. Be sure to consult your doctor if your child has symptoms that seem unusual for him or her.

1. Breathing problems

2. Fever

3. Night sweats

4. Shortness of breath

5. Swollen lymph nodes

6. Weight loss

7. Wheezing

Stages

Hodgkin lymphoma

Stage I: Cancer is confined to lymph nodes in one group, or it is found outside the lymph nodes but in only one organ.

Stage II: The cancer is in two or more different lymph node groups, or it is in one organ as well as nearby lymph nodes. But it is still limited to either the area above or the area below the diaphragm.

Stage III: The cancer is found in lymph nodes both above and below the diaphragm. The cancer may also be in one organ or tissue near the lymph nodes, or in the spleen.

Stage IV: Cancer is found in several parts of one or more organs or tissues such as the bones, the liver, or the lungs.

Non-Hodgkin lymphoma

In addition to being divided into four stages, non-Hodgkin lymphoma is referred to as low stage (it has not spread outside the area where it began) or high stage (it has spread beyond its original location). The specific stages are:

Stage I: Cancer is found in one group of lymph nodes (such as in the neck or under the arm), or it is not found in lymph nodes but in one organ or area, such as the liver or the lung.

Stage II: Cancer is found in two different groups of lymph nodes or in one group of lymph nodes and one organ outside the lymph nodes.

Stage III: Cancer is found in lymph nodes both below and above the diaphragm (the muscle separating the chest from the abdomen). The cancer may also be in organs nearby.

Stage IV: Cancer is found in the brain, bone marrow, or cerebrospinal fluid, or it is found in several different organs or areas of the body.

Concerns in enlarged LN:Size >1-2 cm Increasing size over 2-4 weeks Matted or fixed Supraclavicular LN Fevers >38.5 for 2-4 weeks Constitutional symptoms HSM

When to biopsy: Supraclavicular node Increasing size over 2-4 weeks Constitutional symptoms Asymptomatic enlarged node-not decreasing in size over 6 weeks or not normal after 8-12 weeks

Staging Evaluation: 

Staging Evaluation Laboratory: 

CBC with smear -Chem profile LHD, uric acid Disease specific -ESR, IL2R for HD -LP if head/neck NHL -BMA/Bx for all NHL, only IIB or higher HD Radiology -CXR (PA & Lat) -CT scans neck, chest, abdomen -Gallium, bone scan -PET scan

Lymphoma Staging:

Murphy Ann Arbor I: tumor at one site (nodal or extranodal -- “E”) II: two or more sites; same side of body (or resectable GI primary) III: both sides of body but not IV (& unresec. GI & mediastinal for NHL) IV: CNS or marrow involvement (Murphy); lung, liver, marrow, or bone for Ann Arbor (< 25% marrow) “B” sxs are defined for HD, as is “bulky disease” Head and neck (possibility of CNS involvement) is a further consideration for NHL PET or gallium Non-Hodgkin’s Lymphoma:Malignant solid tumor of immune system Undifferentiated lymphoid cells Spread: aggressive, diffuse, unpredictable Lymphoid tissue; BM and CNS infiltration High growth fraction and doubling time Dx and Rx ASAP Rapid CTX response; tumor lysis concern Incidence/Etiology - NHL:6% childhood cancer 60% of childhood lymphomas Peak age of 5-15; M:F ratio of 2.5:1 Increased with SCIDS, HIV, EBV post t-cell depleted BMT post solid organ transplant Geographic, viral, genetic & immunologic factors Types of NHL:Lymphoblastic (30-35%) 90 % immature T cells (very similar to T-ALL) remainder pre-B phenotype (as in ALL) 50-70% anterior mediastinum neck, supraclavicular, axillary adenopathy Classic: older child with intussusception Clinical Presentations:Abdomen: (35%): pain, distention, jaundice, GI problems, mass Head/neck (13%): lymphadenopathy, jaw swelling, single enlarged tonsil, nasal obstruction, rhinorrhea, cranial nerve palsies Mediastinum (26%): SVC syndrome CNS (rare): HA, V, irritability, papilledema +Fever, malaise, night sweats, wt. loss, Staging of NHL:I Single tumor /node NOT in mediastinum or abdomen II 1-2 nodes same side of diaphragm or resectable GI primary III 2+ nodes both sides of diaphragm; intrathoracic or extensive intra-abd IV Any of above with CNS and/or BM Prognosis affected by…:Incomplete remission in first 2 mos. Rx Large tumor burden (LDH >1000) Stages III and IV: CNS or BM involvement Delay in treatment Relapse **More favorable: Stage I or II, head/neck, peripheral nodes, GI tract

NHL Treatment:

Surgery for diagnostic bx or second look Radiation Therapy: emergency airway obstruction or CNS complication – may be used for local control of residual mass Chemotherapy: Combination chemo is usual, with overall cure rates 60-80+%; high risk of tumor lysis and hyperuricemia Relapse: Re-induction, followed by BMT

NHL chemotherapy overview:

Low-stage NHL’s are treated with CHOP (+/- rituximab – anti-CD20) Higher-stage lymphoblastic lymphomas are treated on leukemia protocols Higher-stage non-lymphoblastic NHLs require extremely aggressive chemotherapy with significant infectious risks, but still have generally good remission rates High-dose chemotherapy with stem cell rescue is considered an option for relapse, though without the success rates of HD; T cell disease probably requires an allogeneic response...

Source: Duke Cancer Health

by

Akshaya Srikanth

Pharm.D Intern

Hyderabad, India