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Pediatric Non-Hodgkin Lymphoma Clinical Presentation

  • Author: J Martin Johnston, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Mar 27, 2014
 

History

The presentation of patients with non-Hodgkin lymphoma is acute or subacute, in contrast to the indolent course that characterizes most lymphomas in adults.

The duration of symptoms before diagnosis is generally 1 month or less, with specific complaints varying according to the predominant sites of involvement.

Bone marrow involvement in non-Hodgkin lymphoma may cause generalized or migratory bone pain, but clinically significant cytopenias are uncommon, with their presence suggesting a diagnosis of acute leukemia.

Symptoms of localized disease

Localized disease can manifest as lymphadenopathy (usually with firmness and no tenderness), tonsillar hypertrophy, or a mass in virtually any location. In children, however, non-Hodgkin lymphoma is primarily an extranodal disease.

Patients with supradiaphragmatic disease (eg, lymphoblastic lymphoma) often report having a nonproductive cough, dyspnea, chest pain, and dysphagia.

Abdominal tumors (usually small noncleaved cell lymphoma [SNCCL] or B-cell large cell lymphoma [LCL]) are associated with abdominal pain, constipation, masses, or ascites. An acute abdomen occasionally is observed and may be mistaken for appendicitis. Rare primary non-Hodgkin lymphoma of the pancreas presents with the clinical picture of pancreatitis.[44]

Symptoms of large cell lymphomas

Constitutional symptoms are uncommon in non-Hodgkin lymphoma, except in patients with anaplastic large cell lymphoma (LCL). Many of these patients have low-grade fever, malaise, anorexia, and/or weight loss. Because LCLs are biologically disparate, however, these lesions have a varied presentation that may include chest or abdominal complications. In rare cases, an LCL appears as an isolated bone lesion in association with pain, swelling, and a risk of pathologic fracture.

Patients with anaplastic LCLs sometimes present with painful skin lesions, bone lesions, peripheral lymphadenopathy, and hepatosplenomegaly.[45, 46] The painful skin lesions may regress spontaneously. A finding less common than these is testicular, lung, or muscle involvement.

Anaplastic LCLs may also result in an apparent cytokine storm, with fevers, vascular leakage, and pancytopenia.

Symptoms of CNS involvement

Patients occasionally develop symptomatic CNS involvement before diagnosis. Headache, meningismus, cranial nerve palsies, and altered sensorium may be observed. Although CNS involvement is uncommon at the time of diagnosis, patients with non-Hodgkin lymphoma (particularly SNCCL) occasionally present with symptoms suggestive of meningoencephalitis.

Other

Among the less common lymphomas of childhood, primary cutaneous/subcutaneous involvement can be seen (eg, in cutaneous T-cell lymphoma or blastic plasmacytoid dendritic cell hematodermic neoplasm).

Next

Physical Examination

Patients with non-Hodgkin lymphoma generally appear mildly to moderately ill. They occasionally have a low-grade fever. Patients may present with pallor, respiratory distress, pain, and discomfort.

A jaw or orbital mass is present in as many as 10% of patients in industrialized countries, but this finding is particularly common in African patients with endemic Burkitt lymphoma.

Other clinical findings in non-Hodgkin lymphoma include the following:

  • Cervical or supraclavicular masses or adenopathy is/are firm, fixed, and nontender
  • Dyspnea or stridor may occur in patients with a mediastinal mass
  • In patients with superior vena cava syndrome, distended neck veins and plethora may be observed
  • Decreased breath sounds are secondary to bronchial obstruction or pleural effusion
  • Thoracic dullness to percussion may be present with pleural effusion.
  • Abdominal distention or a mass may be present with or without tenderness, rebound tenderness, and/or shifting dullness
  • Painful skin lesions suggest an anaplastic large cell lymphoma (LCL); the less common forms of cutaneous lymphoma (T-cell, blastic plasmacytoid dendritic) are typically nontender
  • Obtundation, agitation, and meningismus may be observed in individuals with CNS involvement.
  • Focal pain or swelling in the extremity may be present in patients with primary bone lymphoma.

Relatively uncommon physical findings include the following:

  • Nasopharyngeal mass
  • Parotid enlargement
  • Nephromegaly
  • Testicular enlargement
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Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

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

Disclosure: Nothing to disclose.

Acknowledgements

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.

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; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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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 LSA 2 L 2 Therapy in Children's Cancer Group Protocol 552
Phase Drug Route
Induction Cyclophosphamide, vincristine, daunorubicin IV
Ara-C, methotrexate IT
Prednisone PO
Consolidation Ara-C IV or SC
6-thioguanine PO
Methotrexate IT
L-asparaginase IM
BCNU IV
Phase Cycle Drug Route
Maintenance* 1 6-thioguanine PO
Cyclophosphamide IV
2 Hydroxyurea PO
Daunorubicin IV
3 Methotrexate PO
BCNU IV
4 Ara-C IV or SC
Vincristine IV
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
Phases Drug Route
Induction Prednisone, 6-mercaptopurine PO
Vincristine, daunorubicin, cyclophosphamide, Ara-C IV
L-asparaginase IM
Methotrexate IT
Consolidation 6-mercaptopurine PO
Methotrexate with leucovorin rescue IV
Methotrexate IT
Re-induction Dexamethasone, 6-thioguanine PO
Vincristine, doxorubicin, cyclophosphamide, Ara-C IV
L-asparaginase IM
Methotrexate IT
Maintenance 6-mercaptopurine, methotrexate PO
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 large cell lymphomas (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 Group Subjects,



Estimated %



Definition
A 10 All resected stage I or abdominal stage II tumors
B 65 Unresected stage I or II tumor, stage III, tumor, or stage IV tumor with no CNS involvement and < 25% marrow blasts
C 25 CNS involvement or >25% marrow blasts
Table 4. Standard Therapy in the International Trial for Patients With SNCCL, Group A *
Drug Route
Prednisone PO
Vincristine, cyclophosphamide, doxorubicin IV
Filgrastim (G-CSF), to enhance neutrophil recovery SC 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 in the International Trial for Patients With SNCCL, Group B *
Phase Drug Route
Reduction Prednisone PO
  Vincristine, cyclophosphamide IV
  Methotrexate/hydrocortisone IT
Phase Cycles Drug Route
Induction 2, starting 7 days after reduction Prednisone PO
Vincristine, methotrexate with leucovorin rescue, cyclophosphamide, doxorubicin IV
Methotrexate/hydrocortisone IT
Filgrastim (G-CSF) SC or IV
Consolidation 2 Methotrexate with leucovorin rescue, Ara-C  
Methotrexate/hydrocortisone, Ara-C/hydrocortisone  
Filgrastim (G-CSF)  
Maintenance** 1 Prednisone PO
Vincristine, methotrexate with leucovorin rescue, cyclophosphamide, doxorubicin IV
Methotrexate/hydrocortisone IT
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 in the International Trial for Patients With SNCCL, Group C *
Phase Drug Route
Reduction Prednisone PO
Vincristine, cyclophosphamide IV
Methotrexate/Ara-C/hydrocortisone IT
Induction, cycle 1 starting 7 days after reduction Prednisone PO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicin IV
Methotrexate/Ara-C/hydrocortisone IT
Filgrastim (G-CSF) SC or IV
Induction, cycle 2 Prednisone PO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicin IV
Methotrexate/Ara-C/hydrocortisone IT
Filgrastim (G-CSF) SC or IV
Consolidation, 2 cycles High-dose Ara-C, etoposide (VP-16) IV
Filgrastim (G-CSF), days 7-21 SC or IV
High-dose methotrexate with leucovorin rescue IV
Methotrexate/Ara-C/hydrocortisone IT
Maintenance 1 Prednisone PO
Vincristine, high-dose methotrexate with leucovorin rescue, cyclophosphamide, doxorubicin IV
Methotrexate/Ara-C/hydrocortisone IT
Maintenance 2 Ara-C, etoposide (VP-16) IT
Maintenance 3 Prednisone PO
Vincristine, cyclophosphamide, doxorubicin IV
Maintenance 4 Ara-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 According to the ALCL99 Protocol
Drug Route
Dexamethasone PO
Cyclophosphamide IV
Methotrexate/Ara-C/prednisolone IT
Ara-C = cytarabine; IT = intrathecal; IV = intravenous; PO = oral.
Table 8. Subsequent Therapy for Ki-1+ Anaplastic LCLs According to the ALCL99 Protocol (alternating cycles, repeated 3times each)
Cycle Drug Route
A Dexamethasone PO
Methotrexate with leucovorin rescue, ifosfamide, etoposide (VP-16), Ara-C IV
B Dexamethasone PO
Methotrexate with leucovorin rescue, cyclophosphamide, doxorubicin IV
Ara-C = cytarabine; IT = intrathecal; IV = intravenous; PO = oral.
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