Paediatric Haematology/oncology Flashcards
State some of the main causes of paediatric anaemia
Reduced production RBCs/haemoglobin:
- Dietary deficiency of iron, folic acid or B12
- Bone marrow aplasia
- Leukaemia / other bone marrow tumours
- Bone marrow replacement by fibrous tissue or granulomas
- Anaemia of chronic disease
Haemolysis / increased red blood cell destruction - genetic or acquired:
Genetic
- RBC membrane defects e.g. hereditary spherocytosis
- RBC enzyme abnormalities e.g. G6PD deficiency
- Haemoglobinopathies e.g. sickle cell disease / thalassaemia
Acquired
- Infections e.g. malaria
- Autoimmune haemolysis
- Haemolytic disease of the newborn / blood transfusion reactions
- Drug- and toxin-induced
- DIC
- Hypersplenism
Acute/ongoing blood loss:
- Acute trauma / blood loss
- Head menstruation
- GI losses
State some symptoms and signs of anaemia in children
Can be asymptomatic
Symptoms:
- Fatigue
- SOB
- Failure to thrive
- Symptoms related to underlying disease pathology - e.g. acute pain in sickle cell crises
Signs:
- Pallor
- Tachycardia
- Systolic flow murmur
- Growth limitation
- Jaundice (haemolytic anaemia)
State some investigations to consider for paediatric anaemia
Bloods:
- FBC
- Reticulocyte count
- Blood film
- Haemoglobin electrophoresis (haemoglobinopathies)
- RBC enzyme studies (G6PD and pyruvate kinase deficiency)
- Coombs’ test (autoimmune haemolytic anaemia)
- Folate, vitamin B12 levels
- Other diagnostic tests e.g. bone marrow biopsy for leukaemia
State some conditions associated with DIC in children generally and in neonates
Sepsis – leading cause in children
General
- ARDS (acute respiratory distress syndrome)
- Major trauma/surgery/burns – especially head trauma
- Pancreatitis
- Malignancy (especially haematological malignancy)
- Severe temperature dysregulation
- Severe transfusion reaction
Neonates:
- Necrotising enterocolitis
- Respiratory distress syndrome
- HIE
- Protein C/S or antithrombin deficiency
How is DIC managed
Mainly resolve the underlying cause
Can give blood if actively bleeding (platelets, FFP and cyroprecipitate)
State some conditions associated with hyposplenism / splenectomy
- Congenital asplenia
- Sequestering disease e.g. sickle cell disease, malaria
- Hereditary haemoglobinopathies
- Autoimmune conditions e.g. SLE
- Splenic artery occlusion
- Trauma
- Post-splenectomy
State how patients with hyposplenism / splenectomy should be managed
Immunisations against (encapsulated organisms):
- Streptococcus pneumoniae
- Haemophilus influenzae
- Neisseria meningitidis
High suspicion for bacterial infections in febrile illness
State the types of haematological malignancies
(Acute) leukaemias:
- Acute lymphoblastic leukaemia (ALL)
- Acute myeloid leukaemia (AML)
+ chronic lymphoblastic leukaemia or chronic myeloid leukaemia
Lymphomas:
- Hodgkin lymphoma (Reed Steinberg cells)
- Non-Hodgkin lymphoma (B cell or T cell)
Leukaemia - state the following:
- 2 main types and peak ages to develop type of leukaemia
- Pathophysiology
- Presentation
- Diagnosis
- Management
2 main types and peak ages to develop type of leukaemia
- Acute lymphoblastic leukaemia (ALL) peak age = 2-3
- Acute myeloid leukaemia (AML) peak age = under 2
Pathophysiology:
- Cancer of the stem cells within the bone marrow
- Genetic mutation in lymphoid or myeloid progenitor cells
- Leading to unregulated production of single type of white blood cell, which then suppresses other types
- Either lymphoid or myeloid (acute or chronic)
Presentation:
- Unexplained fever
- Night sweats
- Weight loss
- Failure to thrive
- Petechiae / explained bruising e.g. frequent nose bleeds
- Bone or joint pain
- Hepatosplenomegaly
- Lymphadenopathy
- Pallor
- Pancytopenia or: anaemia / thrombocytopenia / leukopenia
- Persistent fatigue
Diagnosis:
- Very urgent blood count
- Peripheral blood smear
- Bone marrow biopsy
- Lymph node biopsy
- Further staging scans e.g. chest x-ray, CT scan, lumbar puncture, genetic analysis immunophenotyping of abnormal cells
Management:
- Chemotherapy predominantly
- Radiotherapy / bone marrow transplant / surgery
Lymphoma - state the following:
- 2 main types of lymphoma
- Pathophysiology and tissues affected
Lymphoma types:
- Non-Hodgkin lymphoma
- Hodgkin lymphoma peak age
Pathophysiology:
- Cancer of the solid organs
- Affects: lymph nodes, spleen, MALT, bone marrow, thymus
Outline the difference between Hodgkin and non-Hodgkin lymphoma
Hodgkin lymphoma:
- Local contagious spread from one lymph node to another
- Has Reed Steinberg cells (B cell)
Non-Hodgkin lymphoma:
- B cell or T cell lymphoma
- NO Reed Steinberg cells
Non-Hodgkin lymphoma - state the following:
- Pathophysiology
- Presentation
- Diagnosis
- Management
More common
Pathophysiology:
- Genetic mutation in lymphocyte
- Tends to collect in the lymph nodes
- Most cases occur in people over the age of 50
Presentation:
- Non-tender lymphadenopathy
- Unexplained fever
- Night sweats
- Weight loss
- Failure to thrive
- Body itching
Diagnosis:
- Lymph node biopsy (excision)
- Further staging scans e.g. CT scan, MRI, bone marrow biopsy
Management:
Depends on type
- Chemotherapy (high-grade aggressive)
- Monoclonal antibodies
- Radiotherapy
- Bone marrow transplant
Ensure vaccinations are up to date!
Hodgkin lymphoma - state the following:
- Pathophysiology
- Presentation
- Diagnosis
- Management
Pathophysiology:
- Genetic mutation in lymphocyte
- Tends to collect in the lymph nodes
- Most cases occur in people between ages of 20-40 (young adults)
Presentation:
- Non-tender lymphadenopathy (generally cervical or supraclavicular)
- “B” symptoms (unexplained fever, night sweats, weight loss)
- Body itching
Alcohol-induced painful lymphadenopathy is a suggestive symptom
Diagnosis:
- Lymph node biopsy (excision) would show a Reed Steinberg B cell
- Further staging scans e.g. CT scan, MRI, bone marrow biopsy
Management:
- Chemotherapy (mainstay)
- Radiotherapy
- Bone marrow transplant
State 2 risk factors for leukaemia in children
- Radiation exposure e.g. maternal x-ray during pregnancy
- Genetic conditions e.g. Down’s syndrome, Noonan’s syndrome, Kleinfelter’s syndrome, Fanconi’s syndrome
State blood test results you would see in leukaemia (FBC and blood film)
FBC:
- Very high WCC
- Pancytopenia
- Anaemia
- Thrombocytopenia
- Leukopenia
Blood film:
- Shows blast cells
State some complications of chemotherapy for leukaemia patients
- Stunted growth and development
- Infertility
- Immunodeficiency and infections
- Toxicity (neuro and cardio)
- Secondary malignancy
- Failure to treat leukaemia
State the general prognosis for children with acute lymphoblastic leukaemia (ALL)
ALL = generally around 80%
AML = generally poorer than ALL around 60%
But depends on individual factors
State some differentials for leukaemia
- Non-Hodgkin lymphoma
- EBV / infectious mononucleosis
- Aplastic anaemia
- Myelodysplastic syndromes
State 2 infections that are associated with development of Hodgkin lymphoma
- EBV / infectious mononucleosis
- HIV (+ immunosuppression)
+ smoking
State the staging system used for lymphomas
Ann Arbor staging system
Breifly outline pancytopenia and roughly how it can come about
Pancytopenia - reduction in all 3 cellular elements of blood
- RBCs (anaemia)
- WBCs (leukopenia)
- Platelets (thrombocytopenia)
1) Decreased production due to bone marrow failure
2) Immune destruction of blood cells
3) Sequestration in the periphery/spleen (non-immune)
State some differential causes for pancytopenia
1) Decreased production due to bone marrow failure
- Chemotherapy / radiotherapy
- Severe megaloblastic anaemia
- Bone marrow infiltration e.g. secondary malignancy
- Acute leukaemias
- Infections e.g. HIV
2) Immune destruction of blood cells
- Drug induced e.g. Methotrexate
- Autoimmune
3) Sequestration in the periphery/spleen (non-immune)
- Liver disease e.g. Hepatitis C
- Acute and chronic infections e.g. Brucellosis
- Myeloproliferative disorders
Outline steps for investigation of a patient presenting with pancytopenia
Urgent referral to hospital within 24-48 hours
Further bloods:
- Peripheral blood film
- Coagulation profile
- Liver function tests
- Viral serology
- Autoimmune profile
Bone marrow aspirate and biopsy
Idiopathic thrombocytopenic purpura - state the following:
- Pathophysiology
- Most common age
- Presentation
- Investigations
- Management
Pathophysiology:
- Type 2 hypersensitivity reaction characterised by spontaneous thrombocytopenia (low platelet count) with a non-blanching purpuric rash
- Caused by production of antibodies that target and destroy platelets
- Can be idiopathic or triggered e.g. by a viral infection (often history of viral illness)
Most common age:
- Usually under 10 years old
Presentation:
Often history of viral illness, symptoms present over 24-48 hours
- Purpuric/petechial non-blanching rash
- Bruising
- Bleeding e.g. epistaxis, menorrhagia
Investigations:
- Urgent FBC for platelet count
- Consider ruling out other causes of low platelet count e.g. leukaemia or heparin-induced
Management:
Depends on how low platelet count falls - often just requires monitoring
~70% will resolve spontaneously after 3 months
If platelets very low (below 10)
- Prednisolone
- IV immunoglobulins
- Blood transfusions
- Platelet transfusions (only work temporarily - antibodies also destroy these too)
State some differentials for a non-blanching rash
- Meningitis (meningococcal)
- Henoch-Scholein purpura
- Idiopathic thrombocytopenia (ITP)
- Haemolytic uraemic syndrome
- Acute leukaemia
- Mechanical e.g. forceful coughing or vomiting
- Viral illness
- Non-accidental injury
State some general lifestyle advice to give to a patient with thrombocytopenia (low platelet count)
- Avoid contact sport
- Avoid IM injections or invasive procedures e.g. lumbar puncture
- Avoid blood thinning medications e.g. Aspirin, NSAIDs
- Safety net for any injury that may cause internal bleeding e.g. car accident / head injury
State some investigations to consider in a child presenting with a purpuric non-blanching rash
- FBC (anaemia, low platelets, high WCC)
- Coagulation screen
- CRP / ESR (infection)
- U&Es (haemolytic uraemic syndrome or henoch-scholein purpura)
- Blood culture (meningococcal)
- Meningococcal PCR
- Lumbar puncture
- Urine dipstick (haemolytic uraemic syndrome or henoch-scholein purpura)
State some blood tests done as an intitial clotting screen for a patient presenting with bruising
Basic clotting screen:
- FBC
- APTT
- INR
- Blood film
State the clotting test results for haemophilia A (same seen for haemophilia B but it’s 6xs less common)
Prolonged APTT, but normal INR & FBC
Is jaundice within the first 24 hours of life physiological? Suggest some potential causes
No, always pathological
Causes:
- Sepsis
- Haemolytic disorders (G6PD deficiency, spherocytosis)
- Rhesus / ABO incompatibility
- Congenital infections (TORCH screen indicated)
List some causes of physiological jaundice
- Breast milk feeding
- Dehydration
- Biliary atresia
State some complications of Henoch-Schonlein vasculitis
- Renal impairment (acute)
- Arthritis / arthralgia (mainly knees and ankles)
- Intussusception
- Pancreatitis
