66. Pediatric lymphomas Flashcards
Lymphoma
3rd most common malignancies in children (after leukemia & brain tumors)
Main groups of lymphoma: Hodgkin (HL) & Non-hodgkin (NHL)
Hodgkin lymphoma
A malignancy involving lymphoreticular system.
7% of all malignancies in childhood.
Typical age group: 15-19 yrs
Etiology: uknown, family burden, infections (EBV, CMV), socioeconomic factors
Hodgkin lymphoma classification
Classic Hodgkin lymphoma: 90-95% of cases
Nodular sclerosis: 80% in older children & adolescents, 55% in smaller children
Mixed cellularity: 50% in adolescents, 20% in children under 10 yrs
Lymphocytic depletion: rare in children
Lymphocytic predominance: rare
Nodular lymphocyte predominant type: 5-10%
Hodgkin lymphoma symptoms
Non-painful lymphadenomegaly (most often cervical)
Systemic “B sx”: tiredness, asthenia, fever (>38C), night sweats, weight loss
B symptoms —-> unfavourable prognosis
Advanced disease: hepatomegaly, splenomegaly
Hodgkin lymphoma clinical stages
Ann Arbor classification
Stage I- involved lymph nodes in a single area or affected single lymphatic organ (spleen, thymus)
Stage II- two or more groups of involved lymph nodes or organs either above or below the diaphragm
Stage III- involved lymph nodes or lymphoid structures on both sides of the diaphragm
Stage IV- diffuse or disseminated systemic disease
Hodgkin lymphoma diagnosis
Histology of affected lymph nodes- Hodgkin and Reed-Sternberg cells
Biopsy of affected lymph nodes
Bone marrow biopsy in clinical stages III & IV, overt systemic sx, suspected bone marrow involvement
CBC with ESR, renal and hepatic parameters, lactate dehydrogenase, CRP, iron, fibrinogen
Hodgkin lymphoma differential diagnoses
other malignant, infectious & inflammatory diseases with lymphatic involvement
Hodgkin lymphoma treatment
Combined chemo & radiotherapy regimens
High survival rates- up to 90%
Late complications of Hodgkin lymphoma treatment
secondary neoplasms
endocrine disorders
cardiovascular disorders
infertility
Hodgkin lymphoma relapse
Most commonly within 1-3 yrs after diagnosis
Tx of relapse: myeloablative chemo with autologous HSCT & immuno &/or targeted therapy
Non-Hodgkin lymphoma
a hetereogeneous group of lymphoproliferative disorders.
spread out in organs
aggressive clinical course
progress rapidly
high rates of extranodal involvement & bone marrow & CNS infiltration
60% of childhood lymphomas
In Africa, Burkitt lymphoma has the highest incidence in age group 5-9 yrs, higher incidence in boys. Primary localisation in mandible.
In other geographical areas, NHL is sporadic, mainly in children <9 yrs. Primary localisation in abdomen.
Non-Hodgkin lymphoma etiology
Viruses (EBV)
Genetic predisposition
Immunodeficiency conditions
Non-Hodgkin lymphoma classification
Histological classification of NHL:
Burkitt lymphoma
Precursor B-cell lymphoblastic lymphoma
Anaplastic large-cell lymphoma
Diffuse large B-cell lymphoma
Burkitt-like lymphoma
Precursor T-cell lymphoblastic lymphoma
Peripheral T-cell lymphoma
Main subgroups of non-Hodgkin lymphoma
B-cell lymphoma: primary tumor localisation is abdominal, Burkitt centroblasts, CD19+, t(8;14)
Lymphoblast lymphoma: primary tumor localisation is mediastinal with or without LNs, Lymphoblasts, CD10+, t(1;14)
Anaplastic large-cell lymphoma: primary tumor localisation is skin with or without LNs, CD30+, t(2;5)
Non-Hodgkin lymphoma symptoms
Sx depends on primary tumor localisation.
B symptoms:
fever, sweating, asthenia, weight loss
