Pediatric Non-Hodgkin Lymphoma Follow-up

  • Author: J Martin Johnston, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Apr 8, 2011
 

Inpatient & Outpatient Medications

Several classes of medications are used to support patients with non-Hodgkin lymphoma (NHL) undergoing aggressive chemotherapy. These are listed below.

  • Laxatives and stool softeners
  • Prophylactic antibiotics
    • Trimethoprim-sulfamethoxazole (against Pneumocystis carinii)
    • Fluconazole (against Candida species)
    • Nystatin (Candida species)
  • Antiemetics
    • Serotonin (5-hydroxytryptamine type 3 [5-HT3]) receptor antagonists (ondansetron, granisetron, dolasetron)
    • Phenothiazine
    • Lorazepam
    • Metoclopramide
    • Dexamethasone
    • Tetrahydrocannabinol
  • Antimucositic agents
    • Saline or bicarbonate rinse
    • Biotene rinse
    • Peridex rinse
    • Glutamine suspension
  • Histamine (H2) receptor antagonists: Famotidine and ranitidine help to prevent gastritis in patients receiving high-dose corticosteroids.
  • Contraceptives: Oral or injectable contraceptives can be used to suppress menses in female adolescents at risk for menorrhagia due to thrombocytopenia.
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Complications

Acute complications of non-Hodgkin lymphoma and its treatment are discussed in Mortality/Morbidity. With increasing survival rates, late effects of therapy are a growing concern and are described here.

Growth

Linear growth often slows during aggressive chemotherapy. Most patients have catch-up growth and eventually achieve a height in the normal range. Clinically significant long-term growth retardation is essentially confined to patients who receive cranial irradiation. Of interest, a notable minority of children treated for lymphoma eventually becomes obese; the basis for this effect is unclear.[60]

Neuropsychological sequelae

Neurotoxicity due to combined cranial irradiation and intrathecal chemotherapy is well described in patients with acute lymphoblastic leukemia (ALL). Neurotoxic effects range from mild learning disabilities to a profound necrotizing leukoencephalopathy. Patients with CNS lymphoma are at risk for developing these same complications.

In the absence of radiation, intrathecal chemotherapy appears to have little effect on neuropsychological function. However, recent data suggest that patients with non-Hodgkin lymphoma who survive without irradiation are more likely to require special education classes than their siblings are.

In a recent report from Finland, scholastic achievement was particularly impaired in survivors of childhood non-Hodgkin lymphoma and not in patients with Wilms tumor or Hodgkin disease.[61]

The peripheral neuropathy associated with vincristine occasionally leaves permanent deficits, particularly lower extremity weakness.

Fertility

Alkylating agents have particularly been implicated in acute gonadal dysfunction. The long-term effects of these agents among survivors of childhood cancer are somewhat unclear.

Prepubertal boys appear to be at low risk for eventually developing azoospermia or failure of sexual maturation. Older male adolescents are at some risk for developing temporary azoospermia; they might consider banking their semen before undergoing chemotherapy, if this is feasible.

Ovarian failure after high-dose alkylator therapy has also been described. However, in a recent report, female survivors had little or no apparent deficit in pregnancies.

Patients who have a relapse, particularly those treated with myeloablative chemotherapy and/or total body irradiation, have a particularly elevated risk of having permanent gonadal dysfunction.

Second malignancies

The oncogenic potential of therapeutic radiation is well documented, but the risk of second malignancies associated with chemotherapy is less obvious. One clearly implicated antineoplastic agent is etoposide. However, the risk of secondary acute myelocytic leukemia (AML) due to this drug appears to be insignificant at cumulative doses less than 1000 mg/m2.

Cyclophosphamide has also been identified as a potential carcinogen. The relative risk of second malignancies in children exposed to cyclophosphamide is estimated to be as high as 7.4 if the cumulative exposure is more 13 g/m2.

In one series, 86 survivors of pediatric non-Hodgkin lymphoma were evaluated for a mean of 11 years after diagnosis. Only 2 cases of secondary cancer were observed: 1 malignant melanoma and 1 spindle-cell sarcoma, which arose in a radiation field.[62] These findings suggest that, despite concerns about the effects of chemotherapy, patients who do not receive irradiation are unlikely to develop a second malignancy. Follow-up longer than this is needed to accurately assess the life-long risk of second malignancies. Fortunately, the risk appears to be decreasing over time, due to the recognition (and relative avoidance) of treatment-related risk factors such as radiation and high-dose epipodophyllotoxins.[63]

Cardiotoxicity

At high cumulative doses, doxorubicin is likely to cause delayed myocardial toxicity.[64] Irradiation of the heart exacerbates this effect.

In a recent report, 7 of 29 survivors (aged 2-39 y at diagnosis) who received doxorubicin 240-560 mg/m2 eventually developed left ventricular dysfunction approximately 10 years later. However, other reports have described anthracycline-related cardiotoxicity after cumulative doses as small as 100 mg/m2.

If patients have received more than 300 mg/m2 of doxorubicin, perform screening echocardiography every 2-4 years on an indefinite basis. Lower this threshold if mediastinal irradiation was also administered.

Skeletal toxicity

Long-term, high-dose steroid therapy is associated with osteoporosis and avascular necrosis of bone. In one report, long-term survivors of acute lymphoblastic leukemia and non-Hodgkin lymphoma exhibited low bone mineral density in roughly two thirds of men and one third of women. The effects of dexamethasone therapy, cranial radiation, and bone marrow transplantation appeared to be additive.[65] Avascular necrosis most commonly affects the femoral heads, and it may be associated with slipped capital femoral epiphysis. Avascular necrosis of bone is most often observed in adolescents and in female patients. The spectrum of disease ranges from asymptomatic radiographic findings to incapacitating joint destruction requiring restorative surgery.

Radiation therapy is associated with osteopenia. This may occur locally or, of interest, it may be observed diffusely after cranial irradiation.[66]

Viral transmission by means of blood products

Transmission of cytomegalovirus (CMV) is likely if unscreened blood products are administered. Patients who receive such products and who eventually require myeloablative therapies are then at substantial risk of acquiring disseminated CMV. Therefore, the use of CMV-negative products is ideal for patients who may eventually undergo bone marrow transplantation.

With modern transfusion practices, exposure to hepatitis B or C virus is rare. Nonetheless, patients occasionally demonstrate serologic evidence of exposure. Chronic active hepatitis and hepatocellular carcinoma are potential sequelae of this exposure.

Exposure to HIV is relatively unlikely but nevertheless possible.

Explain the risks of viral transmission to patients, their parents, and/or caregivers before transfusions are given.

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Prognosis

The overall prognosis for children with non-Hodgkin lymphoma has continued to steadily improve over the last two decades. Period analysis of SEER data for children less than 15 showed that 5-year and 10-year survival increased from 76.6% and 73.0% in 1990-1994 to 87.7% and 86.9% in 2000-2004. The projected 10-year survival rate for children diagnosed in 2005-2009 was 90.6%.[67]

Among patients with non-Hodgkin lymphoma, the major determinants of prognosis are histology and disease stage. The presence or absence of particular molecular markers (eg, ALK and/or CD56 in anaplastic large cell lymphoma [LCL]) has additional prognostic significance.[68]

Age at diagnosis is a significant prognostic factor when one considers the older pediatric patient (adolescent or young adult) with non-Hodgkin lymphoma. Broadly speaking, older patients have poorer outcomes.[69] There is increasing recognition that these patients need to be viewed as a unique population, in terms of disease biology and treatment tolerance.[70]

More recent studies have defined host (ie, nontumor) prognostic factors for patients with non-Hodgkin lymphoma. For example, polymorphisms of immune-related genes such as interleukin (IL)-10 and tumor necrosis factor show significant associations with treatment outcomes in adults with non-Hodgkin lymphoma.[71, 72] Similar data are not yet available in children.

Patients with relapsed or refractory non-Hodgkin lymphoma are candidates for salvage therapy, which often includes autologous or allogeneic hematopoietic stem cell transplantation. The likelihood of cure depends on diagnosis, initial therapy, and length of first remission.[73] Even patients who experience relapse after autologous transplantation are potentially salvageable with a second transplant procedure.[74]

Pediatric and adolescent patients have better outcome than adults with CNS lymphoma.[75] An ECOG performance status score of 0-1 is associated with improved survival. Higher dose methotrexate is associated with slightly better response.

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Patient Education

For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center and Cancer and Tumors Center. Also, see eMedicine's patient education articles Lymphoma and Cancer of the Mouth and Throat.

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Contributor Information and Disclosures
Author

J Martin Johnston, MD  Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Pediatric Hematology/Oncology, Backus Children's Hospital; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Kathleen M Sakamoto, MD, PhD  Professor and Chief, Division of Hematology-Oncology, Vice-Chair of Research, Mattel Children's Hospital at UCLA; Co-Associate Program Director of the Signal Transduction Program Area, Jonsson Comprehensive Cancer Center, California Nanosystems Institute and Molecular Biology Institute, University of California, Los Angeles, David Geffen School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, Society for Pediatric Research, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

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  75. Abla O, Weitzman S, Blay JY, O'Neill BP, Abrey LE, Neuwelt E, et al. Primary CNS lymphoma in children and adolescents: a descriptive analysis from the international primary CNS lymphoma collaborative group (IPCG). Clin Cancer Res. Jan 15 2011;17(2):346-52. [Medline].

 
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Incidence of lymphoma as a function of age per 100,000 population. Data are from the Surveillance, Epidemiology, and End Results (SEER) for 1990-1994.
Massive mediastinal T-lymphoblastic lymphoma. Note compression of the left mainstem bronchus and the pulmonary atelectasis.
Non-Hodgkin lymphoma of the terminal ileum. Note the doughnut sign, ie, intraluminal contrast material surrounded by a grossly thickened bowel wall. This appearance is highly suggestive of small noncleaved cell lymphoma (Burkitt type).
Malignant pleural effusion. Non-Hodgkin lymphoma of the terminal ileum was diagnosed; the doughnut sign (ie, intraluminal contrast material surrounded by a grossly thickened bowel wall) was present. A diagnosis of stage 3 Burkitt lymphoma was established by means of pleurocentesis. (The bone marrow was normal.) The patient was treated successfully and never required an abdominal procedure.
Massive left pleural effusion as a complication of an upper anterior mediastinal T-lymphoblastic lymphoma. Note the atelectatic left lung. The diagnosis was established by means of thoracentesis. This patient had presented with bilateral parotid gland enlargement.
Table 1. Modified LSA2 L2 Therapy in Children's Cancer Group Protocol 552
PhaseDrugRoute
InductionCyclophosphamide, vincristine, daunorubicinIV
Ara-C, methotrexateIT
PrednisonePO
ConsolidationAra-CIV or SC
6-thioguaninePO
MethotrexateIT
L-asparaginaseIM
BCNUIV
PhaseCycleDrugRoute
Maintenance*16-thioguaninePO
CyclophosphamideIV
2HydroxyureaPO
DaunorubicinIV
3MethotrexatePO
BCNUIV
4Ara-CIV or SC
VincristineIV
Source.—Children's Cancer Group.



Ara-C = cytarabine; BCNU = 1,3-bis(2-chloroethyl)-1-nitrosourea, or carmustine; IM = intramuscular; IT = intrathecal; IV = intravenous; PO = oral; SC = subcutaneous.



* A minimum of 5 repeated courses (total duration of therapy >18 mo) are noted. Each course of intrathecal methotrexate (day 0 of each course) consists of 4 cycles of rotating drug pairs that are administered every 2 weeks after blood counts have recovered.



Table 2. Therapy for Stage III and IV non–B-Cell Disease* According to BFM Protocol 86
PhasesDrugRoute
InductionPrednisone, 6-mercaptopurinePO
Vincristine, daunorubicin, cyclophosphamide, Ara-CIV
L-asparaginaseIM
MethotrexateIT
Consolidation6-mercaptopurinePO
Methotrexate with leucovorin rescueIV
MethotrexateIT
Re-inductionDexamethasone, 6-thioguaninePO
Vincristine, doxorubicin, cyclophosphamide, Ara-CIV
L-asparaginaseIM
MethotrexateIT
Maintenance†6-mercaptopurine, methotrexatePO
Source.—Berlin-Frankfurt-Munster Group.



Ara-C = cytarabine; IT = intrathecal; IV = intravenous; PO = oral; SC = subcutaneous.



* Diagnoses included lymphoblastic lymphoma of the T-cell or precursor B-cell type, immunoblastic T-cell lymphoma, and other peripheral T-cell lymphomas. Of note, patients with Ki-1+ anaplastic LCLs were not included.



† Continued until 24 months after diagnosis.



Table 3. Clinical Risk Groups in the International Trial for Patients With SNCCL (Children's Cancer Group study 5961)
Clinical GroupSubjects,



Estimated %



Definition
A10All resected stage I or abdominal stage II tumors
B65Unresected stage I or II tumor, stage III, tumor, or stage IV with no CNS involvement and < 25% marrow blasts
C25CNS involvement or >25% marrow blasts
Table 4. Standard Therapy for Subjects in the International Trial for Patients With SNCCL, Group A*
DrugRoute
PrednisonePO
Vincristine, cyclophosphamide, doxorubicinIV
Filgrastim (G-CSF), to enhance neutrophil recoverySC or IV
G-CSF = granulocyte colony-stimulating factor; IV = intravenous; PO = oral; SC = subcutaneous.



* See Table 3 for the definition of group A. All subjects received 2 cycles.



Table 5. Standard Therapy for Subjects in International Trial for Patients With SNCCL, Group B*
PhaseDrugRoute
ReductionPrednisonePO
Vincristine, cyclophosphamideIV
Methotrexate/hydrocortisoneIT
PhaseCyclesDrugRoute
Induction2, starting 7 d after reductionPrednisonePO
Vincristine, methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/hydrocortisoneIT
Filgrastim (G-CSF)SC or IV
Consolidation2Methotrexate with leucovorin rescue, Ara-C
Methotrexate/hydrocortisone, Ara-C/hydrocortisone
Filgrastim (G-CSF)
Maintenance1PrednisonePO
Vincristine, methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/hydrocortisoneIT
Ara-C = cytarabine; G-CSF = granulocyte colony-stimulating factor; IT = intrathecal; IV = intravenous; PO = oral, SC = subcutaneous.



* See Table 3 for the definition of group B.



Table 6. Standard Therapy for Subjects in International Trial for Patients With SNCCL, Group C*
PhaseDrugRoute
ReductionPrednisonePO
Vincristine, cyclophosphamideIV
Methotrexate/Ara-C/hydrocortisoneIT
Induction, cycle 1 starting 7 d after reductionPrednisonePO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/Ara-C/hydrocortisoneIT
Filgrastim (G-CSF)SC or IV
Induction, cycle 2PrednisonePO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/Ara-C/hydrocortisoneIT
Filgrastim (G-CSF)SC or IV
Consolidation, 2 cycles†High-dose Ara-C, etoposide (VP-16)IV
Filgrastim (G-CSF), days 7-21SC or IV
High-dose methotrexate with leucovorin rescueIV
Methotrexate/Ara-C/hydrocortisoneIT
Maintenance 1PrednisonePO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/Ara-C/hydrocortisoneIT
Maintenance 2Ara-C, etoposide (VP-16)IT
Maintenance 3PrednisonePO
Vincristine, cyclophosphamide, doxorubicinIV
Maintenance 4Ara-C, etoposide (VP-16)IV
Ara-C = cytarabine; G-CSF = granulocyte colony-stimulating factor; IT = intrathecal; IV = intravenous; PO = oral, SC = subcutaneous.



* See Table 3 for the definition of group C.



† For patients with CNS involvement, during consolidation cycle 1 only.



Table 7. Prephase Therapy for Ki-1+ Anaplastic LCLs in All Patients According to the BFM-90 Protocol
DrugRoute
PrednisonePO
CyclophosphamideIV
Methotrexate/Ara-C/prednisoloneIT
Ara-C = cytarabine; IT = intrathecal; IV = intravenous; PO = oral.
Table 8. Subsequent Therapy for Ki-1+ Anaplastic LCLs According to the BFM-90 Protocol
CycleDrugRoute
AMethotrexate with leucovorin rescue, ifosfamide, etoposide (VP-16), Ara-CIV
Methotrexate/Ara-C/prednisoloneIT
BDexamethasonePO
Methotrexate with leucovorin rescue, Ara-C, doxorubicinIV
Methotrexate/Ara-C/prednisoloneIT
AADexamethasonePO
Vincristine, high-dose methotrexate with leucovorin rescue, ifosfamide, Ara-C, etoposide (VP-16)IV
Methotrexate/Ara-C/prednisoloneIT
BBDexamethasonePO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicinIV
Methotrexate/Ara-C/prednisoloneIT
CCDexamethasonePO
Vindesine, high-dose Ara-C, etoposide (VP-16)IV
Methotrexate/Ara-C/prednisoloneIT
Ara-C = cytarabine; IT = intrathecal; IV = intravenous; PO = oral.
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