8- Paediatric Haematological Oncology 2/2 Flashcards
lymphoma background
- Malignancy of the lymphatic system
>10% of childhood cancers - More common in boys and older children
Types
1) Hodgkin’s lymphoma
2) Non-Hodgkin’s lymphoma - Just over 50%
- ## Broad range of diagnoses
Pathophysiology of lymphoma
- Not well understood
- Multifactorial causes
o Infection
o Genetic
o Environmental
Risk factors for lymphoma
- Epstein-barr virus
- Immunosuppressed patients e.g. those who have had a solid organ transplant
- males
Presentation of lymphoma
Palpable mass
- Non-tender
- Could also be a sign of metastatic malignancy from another site- more common in adults
B symptoms
- Weigh tloss
- Night sweats
- Fevers
Others
- Lethargy
- Anorexia
Mediastinal lymphadenopathy or superior vena cava obstruction may present with
- Cough
- Wheeze
- Difficulty breathing
- Airway compromise
investigations for lymphoma
- Bloods
–> FBC
–> U and E – tumour lysis syndrome can occur before treatment begins in lymphoma with rapid cell turnover
–> LDH levels usually elevated - Imaging
–> USS- identify other nodes and biopsy
–>CXR – if mediastinal node involvement
–> Full body CT- staging - Biopsy- definitive diagnosis
staging of lymphoma
Ann Arbor
Lymphoma is staged according to how many groups of lymph nodes or organs are involved:
- Stage 1: Disease is present in a single group of lymph nodes or a single organ
- Stage 2: Disease is present in 2 or more groups of lymph nodes or organs on the same side of the diaphragm
- Stage 3: Disease is present in lymph nodes or organs on both sides of the diaphragm
- Stage 4: There is diffuse involvement of lymph nodes and organs such as the liver and bones
The presence of “B symptoms” is associated with worse prognosis at all stages and a B is added to any stage if these are present (eg stage 1B, 2B etc).
Management of lymphoma
Immediate
- Emergency if mediastinal mass with airway compromise – treat with high dose steroids and airway support if required
- SVCO may also require stenting
- If tumour lysis syndrome -> hyperhydration
–>Allopurinol or rasburicase also used
Definitive
- Chemotherapy
- Radiotherapy
Prognosis
- Hodgkins lymphoma has a better prognosis than non-hodgkins
complications of lymphoma treatment
1) Tumour lysis syndrome
- Occurs when rapid lysis of tumour cells release a large amount of phosphorus, potassium and calcium leading to kidney damage
- Occurs usually when chemo has been commenced
2)Neutropenia
lymphoma follow up
life long
Leukaemia
Background
- Malignancy of the stem cells in the bone marrow
- Causes unregulated production of certain types of cells
leukaemia classification
Speed
- Chronic
- Acute
Cell line
- Myeloid
- Lymphoid
Most common leukaemia’s
The types of leukaemia that affect children from most to least common are:
- Acute lymphoblastic leukaemia (ALL) is the most common in children
- Acute myeloid leukaemia (AML) is the next most common
- Chronic myeloid leukaemia (CML) is rare
Pathophysiology of leukaemia
- Genetic mutation in one of the precursor cell in the bone marrow leads to excessive production of a single type of abnormal white blood cell
- Excessive production of one type of cell can lead to suppression of the other cell lines -> underproduction of all other types of cells -> pancytopenia
- Pancytopenia is low
o RBC (anaemia)
o WBC (leukopenia)
o Platelets (thrombocytopenia)
RF for leukaemia
Radiation exposure, for example with an abdominal xray during pregnancy, is the main environmental risk factor for leukaemia.
There are several conditions that predispose to a higher risk of developing leukaemia:
* Down’s syndrome
* Kleinfelter syndrome
* Noonan syndrome
Presentation of leukaemia
The presentation of leukaemia is typically non-specific. Symptoms can include:
- Persistent fatigue
- Unexplained fever
- Failure to thrive
- Weight loss
- Night sweats
- Pallor (anaemia)
- Petechiae and abnormal bruising (thrombocytopenia)
- Unexplained bleeding (thrombocytopenia)
- Abdominal pain
- Generalised lymphadenopathy
- Unexplained or persistent bone or joint pain
- Hepatosplenomegaly
