Haem Flashcards by Toni Whitaker

Managing sickle cell crisis

Analgesia
Rewarming
Oxygen
Antibiotics
IV fluids

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Indications for exchange transfusion in SCD

Chest crisis
Stroke
Priapism

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Indications for top up transfusion in SCD

Significant drop in Hb from baseline

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In child with limp + bone pain what is the most important diagnosis to exclude?

1: acute leukaemia
2: septic arthritis

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Managing ITP

2-10 yrs
Self limiting
No continued bleeding = outpatient
Continued bleeding + haemodynamic instability = fluid bolus + close monitoring
Platelets given will just be destroyed
Don’t give unless bleeding life threatening

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Most important management priority in newly diagnosed AML

Prophylactic hyper hydration, allopurinol/rasburicase+ electrolyte monitoring req from diagnosis to prevent tumour lysis syndrome:

hyperkalaemia
hyperphosphataemia
gout
fluid overload/ dehydration
raised urea + creatinine

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Complications of childhood malignancy

Short stature
Infertility
Educational difficulties
Haematological malignancy

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Type 1 vWD

AD
Partial defiance of vWF
Mild phenotype

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Type 2 vWD

Defective vWF

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Type 3 vWD

Complete vWF deficiency

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Fanconi’s anaemia

Aplastic anaemia

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Pica

Eating non food substances
Due to severe iron deficiency
Microcytic anaemia + low ferritin
Prolonged breastfeeding + delayed weaning
Iron stores sufficient for 4 months, then req supplementation

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Other consequences of delayed weaning

Hypocalcaemia

Poor weight gain

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ITP diagnosis

Platelets

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ALL

80% of childhood leukaemias
Splenomegaly, bruises, lethargy, pallor
More common in boys
Pk incidence 5 yrs
Higher incidence in caucasians

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AML

Far less likely that ALL however some conditions incr risk
Downs ALL= AML
Bloom’s syndrome
Neurofibromatosis

Beta thalassemia major

AR - point mutation Chr11
Complete lack of beta globin
No functioning HbA, reliant on HbA2 + HbF
Decr HbF in 1st yr -> FTT due to severe microcytic anaemia, normal ferritin
Mediterranean + Middle eastern ethnicity

Beta thalassemia minor

AR 
Heterozygous for beta chain mutation on Chr 11
Mild hypochromic, microcytic anaemia 
Usually asymptomatic
Incr levels HbA2 + HbF

Clinical features of beta thal major

FTT, lethargy, pallor + jaundice
Hepatosplenomegaly
Skull bossing, long bone deformity (excessive intramedullary haematopoiesis)

Treating beta thal major

Regular blood transfusions aiming to maintain Hb > 100
Death inevitable in first few years of life without treatment
Or allogenic BM transplant
Iron chelation therapy (desferrioxamine) required to prevent iron overload

Alpha thalassemia

South East Asia
One gene corruption: asymptomatic
Two gene corruptions: mild hypochromic anaemia
Three corruptions: HbH disease(beta chain tetramers)
Four corruptions: HbBarts (gamma chain tetramers) death in utero

Causes of microcytic anaemia

MCV

Thalassemias
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia

Causes of normocytic anaemia

MCV 85-105

Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia

Macrocytic anaemia

MCV>105

Failure of DNA synthesis
Megaloblastic anaemia: vit B12/folic acid defic/pernicious anaemia
Liver disease
Hypothyroidism

How would you treat ALL?

``` Supportive: transfusion + abx Chemotherapy: Remission induction Consolidation Maintenance Can take a number of years High risk of neuro disease = CNS prophylaxis: intrathecal methotrexate + radiotherapy Good prognosis with appropriate therapy ```

How is malaria diagnosed?

Thick blood film: parasite detection Thin blood film: detect species and determination of parasitaemia (% erythrocytes infected) Signet ring inclusions

Complications of malaria

Haemoglobinuria: due to severe haemolysis - black water fever Acute renal failure Cerebral malaria: fits + coma, main cause of death

Malaria treatment:

``` Oral quinine IV quinine SE: tinnitus, deafness + nausea OD: heart block Exchange transfusion with parasitaemia >10% ```

G6PD triggers

Fave (broad) beans Anti malarials + sulphonamide abx Infections Supportive treatment, occasionally transfusions required

Hodgkin's lymphoma

``` Asymmetrical painless lymphadenopathy Painful on alcohol ingestion B sx Reed Sternberg cells Early: CT scan + radiotherapy Advanced: CT scan, radiotherapy + chemo ```

Prognosis of Hodgkin's lymphoma

70% chance of cure | Incr age / systemic sx= poorer prognosis

What skeletal infections are children with ScD at greater risk of?

Septic arthritis Osteomyelitis Strep or salmonella

Causes of pica (eating non-nutritive substances)

Iron deficiency | Lead poisoning

Features of lead poisoning

``` Encephalopathic signs Pica, blue line on gums Hypochromic microcytic anaemia Basophilic stippling Measure blood lead levels Treatment= chelation with EDTA ```

Pernicious anaemia

AI attack of gastric parietal cells = no intrinsic factor -> Vit B 12 deficiency, normal ferritin Macrocytic anaemia Anti parietal antibodies (Fewer antiIF antibody) Peripheral neuropathy, smooth tongue, angular stomatitis, depression, dementia, subacute degen of the spinal cord

Dx and treatment of pernicious anaemia

Dx: schilling test Rx: 3 monthly IM hydroxycobalamin a vit b12 analogue