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PaedsY5 Haematology > A > Flashcards

A Flashcards

1
Q

What is aplastic anaemia?

A

Pancytopenia associated with bone marrow hypoplasia.

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2
Q

What is the aetiology types of aplastic anaemia?

A

Idiopathic
Acquired
Inherited

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3
Q

What is the inherited aetiology of aplastic anaemia?

A

· Fanconi anaemia: autosomal recessive, DNA repair issue. Associated with growth retardation, forearm bone abnormalities, heart and renal defects, horseshoe kidney, skin pigmentation.

· Dyskeratosis congenita: sex linked, dyskeratosis of skin and nail atrophy

· Schachmann syndrome: pancytopenia in 25%

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4
Q

What is the idiopathic aetiology of aplastic anaemia?

A

40%. May be due to auto-immune destruction of progenitor cells in the BM by cytotoxic T cells. Phenotypically normal but have genetic marrow failure syndromes.

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5
Q

What is the acquired aetiology of aplastic anaemia?

A

· Viral infection (HIV, parvovirus, CMV)

· Drugs (alkylating agents, chemotherapy)

· Radiation

· Chemicals (DDT, benzene)

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6
Q

What is the epidemiology of aplastic anaemia?

A

2/100k py.

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7
Q

What is the history and examination in aplastic anaemia?

A

Present with symptoms of anaemia, infections, and bleeding.

Signs of anaemia (pallor) bruises, bleeding gums, bacterial or fungal infections. NO HEPATOMEGALY OR SPLENOMEGALY or lymphadenopathy.

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8
Q

What are the investigations in aplastic anaemia?

A

Blood count and film: low Hb, low Pl, low WCC. Exclude leukemia.

BM tephrine bippsy: for diagnosis and exclusion of other causes (infiltration, leukemia, malignancy)

Chromosomal abnormalities: random breaks in peripheral lymphocytes in FA.

Ham test: paroxysmal nocturnal haemoglobinuria.

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9
Q

What is the management of aplastic anaemia?

A

Treat the underlying cause

Supportive: blood and platelet transfusion, cover with vaccinations.

May require immunosuppression if autoimmune, cyclosporine A, androgen for FA.

CVC/portacath placement for transfusions or phlebotomy, HCST is the only definitive cure, from HLA identical family member.

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10
Q

What are the complications and prognosis of aplastic anaemia?

A

Bleeding, infections, sepsis and risk of developing tumors or myeloplastic disorders. HCST complications such as graft rejection, GVHD, infection.

Low counts lead to low prognosis.

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11
Q

What is the definition of haemolytic anaemia?

A

Premature haemolysis leading to low erythrocyte survival and anaemia secondary to BM activity being unable to compensate.

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12
Q

What is the aetiology of haemolytic anaemia?

A

Immune

· Haemolytic disease of the newborn, transfusion reaction

· AIHA (warm or cold antibodies)

Non immune inherited

· Structural defects: spherocytosis, elliptocytosis.

· Metabolic defects: G6PD deficiency

· Hb defects: Sickle cell, Thalassaemia.

Non immune acquired

· Trauma -> RBC fragmentation, TTP/HUS/DIC/malignant HTN / Pre eclampsia.

· Infection -> Malaria

· Paroxysmal nocturnal haemoglobinuria.

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13
Q

What is the epidemiology of haemolytic anaemia?

A

Hereditary: African, meditarranean and middle eastern.

HS: Northern Europe.

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14
Q

What is the history and exam of haemolytic anaemia?

A

Jaundice, anaemia, hepatosplenomegaly

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15
Q

What are the investigations for haemolytic anaemia?

A

Bloods: low Hb, high BR/LDH, low haptoglobin. RC G6PD and PK analysis.

Blood film: leukoerythroblastic picture -> low RBC, high reticulocytes, nucleated RBC.

Urine: high urobilinogen, ?PNH. Osmotic fragility for spheroctosis.

DAT Coombs test: AI vs non AI. Ham’s test for PNH.

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16
Q

What is the management of haemolytic anaemia?

A

Contributing factor avoidance (ie cold exposure, drugs in G6PD etc).

AI: prednisolone, splenectomy, AZA/CycloPhosphamide.

PNH: transfusions (leucocyte depleted)

17
Q

What are the complications and prognosis of haemolytic anaemia?

A

Renal failure, PNH can transform to aplastic anaemia, HS can cause gallstones and aplastic anaemia in parvovirus infection. Crises, leg ulcers

18
Q

What is IDA?

A

Anaemia with low MCV (<80fl) and depleted iron stores

19
Q

What is the cause of IDA?

A

WHO definition: Hb<11g/dl aged 1-2, Hb <11.2 g/dl aged 3-5 years.

Occurs in 3 stages: Fe depletion, Fe deficient erythropoiesis, and Fe deficiency anemia.

Low Fe stores at birth: prematurity, multiple pregnancy, perinatal bleeding, early cord clamping, and maternal deficiency.

Low intake: Exclusive breastfeeding past 6m, excessively early cows milk introduction, high fruit juice/milk intake in first year of life, Crohn’s/Coeliac.

High loss: Acute haemorrage (Meckels diverticulum, intestinal duplication cyst, NEC) Chronic haemorrage (CMPA, IBD, intestinal polyps) menstruation, parasite

High demand: growth, prematurity, IUGR, post malnutrition, Thalassaemia

20
Q

What is the epidemiology of IDA?

A

Incidence 5-40%, most common nutritional deficiency worldwide.

21
Q

What would you find on history and exam of someone with IDA?

A

General: gradually tired, poor exercise tolerance, fail to thrive, delay, headaches
Anorexia, pica, irritability, impaired concentration

General: signs of anaemia (pallor, tachycardia) and systolic flow murmurs.
Rarely: brittle nails, kolynichia, spoon shaped nails, glossitis, angular stomatitis, hepatosplenomegaly.

22
Q

What is the investigations of IDA?

A

Blood film (microcytic hypochromic anaemia, anysocytosis/poikliocytosis), and count (low Fe, low Hb, High MCV, low Ferritin, high TIBC)

Hb electrophoresis if need to exclude thalassaemia.

BM: erythroid hyperplasia, low BM Fe.

23
Q

What is the management of IDA?

A

Preterm: fortify breast milk with Fe/formula.

Infant: increase absorbable Fe sources ie. Meat and fish, eating Vit C rich foods. Oral Fe SO4 may be considered. ? blood transfusion if severe with CHF.

24
Q

What are the complications of IDA?

A

Impaired mental and psychomotor development. HOCF in some cases. Good outcome if it is nutritional and prompt action is taken.

PaedsY5 Haematology (6 decks)

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