Childhood Cancer and Functional Impacts Across the Care Continuum (2021) / Chapter Skim
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5 Selected Hematologic Malignancies and Histiocytoses
5 Selected Hematologic Malignancies and Histiocytoses
Pages 245-274
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From page 245... ...
The functional limitations associated with these cancers and their treatments are addressed in Chapter 4. This chapter covers two groups of hematologic malignancies found in children and adolescents -- leukemia and lymphoma -- as well as the distinct disease histiocytosis, briefly described below: • Leukemia is a type of cancer that starts in blood-forming tissue, such as the bone marrow, most often in white blood cells.
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From page 246... ...
For childhood Hodgkin lymphoma, additional diagnostic tests include laboratory studies (CBC, chemistries, and markers of inflammation) , anatomic and functional imaging, lymph node biopsy for pathologic examination of the tumor cells, and bone marrow biopsy and aspirate (PDQ Pediatric Editorial Board, 2020c)
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Leukemia Acute Lymphoblastic Leukemia As noted, ALL, also called acute lymphocytic leukemia, is the most common hematologic malignancy in childhood. ALL is an aggressive type of leukemia that is characterized by the presence of too many premature white blood cells, called lymphoblasts, in the bone marrow and peripheral blood.
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From page 248... ...
There are varying approaches to patients with refractory or relapsed disease, depending on the biology and genetics of the subtype, the time of relapse, and the number of relapses. These treatments may include further multi-agent chemotherapy, stem cell transplantation, and/or chimeric antigen receptor (CAR)
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The risk of alkylator-associated late effects increases in survivors exposed to relapse therapy or stem cell transplantation. Acute Myeloid Leukemia AML is characterized by malignant transformation of myeloid cells.
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ATRA is given orally, and arsenic is given by intravenous infusion. Risk of late effects in AML patients is associated with the cumulative doses of chemotherapy and, for those undergoing stem cell transplantation, exposure to total-body irradiation.
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Patients who have an inadequate response to tyrosine kinase inhibitors or have advanced-phase disease are treated with allogeneic stem cell transplantation. The 5-year survival rate is about 80–90 percent (Deininger et al., 2009)
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With stem cell transplantation, the 5-year survival rate is about 64 percent (Locatelli et al., 2005)
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The cure rate following salvage with autologous stem cell transplantation with or without radiotherapy and with or without maintenance brentuximab vedotin is more than 50 percent (Satwani et al., 2015)
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Still another type of mature B cell non-Hodgkin lymphoma is primary mediastinal B cell lymphoma, which develops from B cells in the mediastinum and may spread to the lymph nodes and distant organs. This disease is more likely to occur in older adolescents.
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. Low-risk organs include the skin, bone, lungs, lymph nodes, gastrointestinal tract, pituitary gland, thyroid, thymus, and CNS (PDQ Pediatric Editorial Board, 2020e)
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5-7 Participation in clinical trials is considered the standard of care for many children with hematologic malignancies. Conclusions 5-1 Clinical trials are needed to improve or maintain survival in chil dren with hematologic malignancies while limiting acute toxic ity and mitigating late effects of treatment, including secondary malignant neoplasms (SMN)
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2016. Outcomes of allogeneic hematopoietic cell transplantation in children and young adults with chronic myeloid leukemia: A CIBMTR cohort analysis.
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2018. Temporal patterns in the risk of chronic health conditions in survivors of childhood cancer diagnosed 1970–99: A report from the Child hood Cancer Survivor Study cohort.
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2005. Hematopoietic stem cell transplantation (HSCT)
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:3765–3771. PDQ Pediatric Editorial Board (Physician Data Query® Pediatric Treatment Editorial Board)
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2011. Hematopoi etic stem cell transplantation for advanced myelodysplastic syndrome in children: Results of the EWOG-MDS 98 study.
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• Lymph node biopsy (lymphoblastic lymphoma) Acute myeloid • Bone marrow biopsy and leukemia aspiration with genetic characterization • Laboratory testing, including Acute promyelocytic serum electrolytes, LDH, uric acid, leukemia BUN, creatinine, and liver function tests
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References • Age Standard 95% Hunger et al., • White blood cell (low) risk 2012 (WBC)
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264 CHILDHOOD CANCER AND FUNCTIONAL IMPACTS ANNEX TABLE 5-1 Continued Cancer Type Subtype Diagnostic Evaluation Myelodysplastic • Bone marrow biopsy and syndrome aspiration with genetic characterization Chronic • Bone marrow biopsy and myelogenous aspiration with genetic leukemia characterization • Quantitative polymerase chain reaction for BCR-ABL1 • Laboratory testing, including serum electrolytes, LDH, uric acid, BUN, creatinine, and liver function tests Juvenile • Bone marrow biopsy and myelomonocytic aspiration with genetic leukemia characterization • Laboratory testing, including serum electrolytes, LDH, uric acid, BUN, creatinine, and liver function tests Lymphoma Hodgkin • Biopsy • Bone marrow aspiration and biopsy (for patients with systemic symptoms or high-risk disease) • Laboratory studies, including complete blood count (CBC)
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SELECTED HEMATOLOGIC MALIGNANCIES AND HISTIOCYTOSES 265 Survival Rate (5-year overall sur Risk vival [OS] unless Risk-Determining Factors Category otherwise noted)
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266 CHILDHOOD CANCER AND FUNCTIONAL IMPACTS ANNEX TABLE 5-1 Continued Cancer Type Subtype Diagnostic Evaluation Non-Hodgkin Burkitt • Biopsy • Bone marrow biopsy and aspiration Diffuse large B cell • Lumbar puncture lymphoma • CT or MRI of neck, chest, Primary mediastinal abdomen, and pelvis, and if bony B cell lymphoma involvement, appropriate limb • PET scan Anaplastic large-cell • Laboratory testing including serum lymphoma electrolytes, LDH, uric acid, BUN, Posttransplant creatinine, and liver function tests lymphoproliferative disorder Pediatric-type follicular lymphoma Marginal zone lymphoma Primary central nervous system lymphoma Histiocytoses Langerhans cell • Laboratory testing, including histiocytosis CBC, serum electrolytes, and liver function tests • Biopsy • Bronchoscopy • Urine analysis • Water deprivation test • Skeletal survey • Bone scan • MRI or CT scan of involved sites • Brain MRI to examine pituitary • Bone marrow biopsy and aspirate
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et al., 2013 98% Ronceray et al., 2018 63% (3-year OS) Thorer et al., 2014 • Number of sites or Low risk 99% Gadner et al., systems 2013 • High-risk organs (liver, spleen, and bone marrow)
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A genetic test identifying a mutation in one of the genes involved in the condition 2. At least 5 of the following 8 signs and symptoms: • Fever • Enlarged spleen • Cytopenia • Elevated triglycerides or low fibrinogen in blood • Hemophagocytosis on bone marrow or lymph node biopsy • Decreased natural killer cell activity in blood • Elevated ferritin level in blood • Elevated blood levels of CD25
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SELECTED HEMATOLOGIC MALIGNANCIES AND HISTIOCYTOSES 269 Survival Rate (5-year overall sur Risk vival [OS] unless Risk-Determining Factors Category otherwise noted)
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270 CHILDHOOD CANCER AND FUNCTIONAL IMPACTS ANNEX TABLE 5-2 Selected Hematologic Malignancies and Histiocytoses: Treatment Information Cancer Type Subtype Stage or Risk Category Leukemia Acute lymphoblastic Leukemia Standard (low) risk leukemia High risk Lymphoblastic lymphoma Very high risk Relapse Acute myeloid leukemia Acute promyelocytic leukemia Myelodysplastic syndrome Chronic myelogenous leukemia Juvenile myelomonocytic leukemia Lymphoma Hodgkin Low Intermediate High Non-Hodgkin Burkitt Diffuse large B cell lymphoma Primary mediastinal B cell lymphoma Lymphoblastic lymphoma Anaplastic large-cell lymphoma Posttransplant lymphoproliferative disorder Pediatric-type follicular lymphoma
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SELECTED HEMATOLOGIC MALIGNANCIES AND HISTIOCYTOSES 271 Treatment Duration of Treatment* • Multi-agent chemotherapy 2–3 years • Multi-agent chemotherapy with or without cranial or 2–3 years craniospinal radiation • Multi-agent chemotherapy with or without cranial or 2–3 years craniospinal radiation • Varies Varies • Multi-agent chemotherapy with or without bone 4–5 months marrow transplant • Targeted agents or monoclonal antibodies added for patients with specific biomarkers or genetic markers • All-trans retinoic acid/arsenic with or without 5–6 months, followed anthracycline chemotherapy, followed by by 2–3 years of maintenance maintenance • Stem cell transplantation 1–2 months • Oral tyrosine kinase Indefinite • Bone marrow transplant for inadequate response or advanced phase • High-dose chemotherapy with stem cell 3–4 months transplantation • Multi-agent chemotherapy with or without radiation 2–4 months • Multi-agent chemotherapy with or without radiation 4–5 months • Multi-agent chemotherapy with or without radiation 5–6 months • Surgery only stage 1–2; multi-agent chemotherapy 2–4 months with or without rituximab • Surgery only stage 1–2; multi-agent chemotherapy 4–6 months with or without rituximab • Chemotherapy and rituximab 4–6 months • Treat according to acute lymphoblastic leukemia 2–3 years regimens • Multi-agent chemotherapy 4–6 months • Surgery, reduction in immunosuppression Varies by treatment • Rituximab • Chemotherapy • Surgery only Varies by treatment • Chemotherapy with/without rituximab continued
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From page 272... ...
lymphoma Histiocytoses Langerhans cell Low risk histiocytosis High risk Hemophagocytic lymphohistiocytosis * In addition to the treatment durations listed, which are based on standard treatment protocols, the committee estimates, based on the members' clinical expertise, that patients typically require an additional 3–18 months to recover from the acute effects of treatment with radiation and/or chemotherapy.
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From page 273... ...
chemotherapy • Treat underlying condition (e.g., infection) 2 or more months +/– time for stem cell • Chemotherapy and immunotherapy transplantation • Stem cell transplantation
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