Hdp 3 Blood And Anaemia

Front 1

What are the four types of microcytic anaemia?

Back 1

Iron Deficiency anaemia, anaemia of chronic disease, sideroblastic anaemia, thalassemia.

Front 2

Define anaemia

Back 2

Anaemia = decrease or a fall in the level of haemoglobin in the blood below the reference level normal for age and sex of indiv.

Front 3

Classify anaemia

Back 3

1. Microcytic (low Mean Cell Volume) 2. Normocytic (normal MCV) 3. Macrocytic (high MCV), 4. Haemolytic (normo- or macro-cytic)

Front 4

What are the clinical features of anaemia (common)

Back 4

Sym: fatigue, h/a, faintness, breathlessness, angina, palpitations, intermittent claudication. Signs: pallor, tachycardia, systolic flow murmur, cardiac failure. May be affected by speed of onset, severity, age.

Front 5

What is def, aetiology, pathogenesis, clinical features, management of iron deficiency anaemia

Back 5

Def: IDA develops when there is inadq iron for haemoglobin synth, usually assoc with depleted iron stores. A: blood loss, poor diet, increased demand, decrease absorp, poor intake. CF: spoon nails, brittle hair, glossitis, dysphagia, poor thermoregulation. Invest: Normal blood film - hypochromic microcytic anaemia. decreased MCV. Iron Studies - serum FE low, increase TIBC. Mgt: identify and treat cause, oral iron, parenteral iron.
DD: Microcytic anaemia, megaloblastic anaemia.

Front 6

What is role of B12 and folate in body and how does xu lead to anaemia

Back 6

B12 and Folate: required in DNA (esp RBC) production

Front 7

What are the cellular components of blood?

Back 7

RBC (99%), WBC (granular and agranular), platelets, thrombocytes, plasma

Front 8

Define haemoglobin and what is its function?

Back 8

Defin: globin protein consisting of 4 polypetide chains
Fx; Transports O2 and buffers H+ ions

Front 9

What are the phases of haemostasis?

Back 9

Vasoconstriction
Gap plugging by platelets (delayed bleeding from cuts, bleeding into skin and mucous membranes)
Fibrinolysis / limitation of coagulation
Coagulation cascade (delay causes bleeding into joints and muscle)

Front 10

What are haemopoitic growth factors?

Back 10

Regulate differentiation and prolif; Erythopoietin (EPO) - produced in kids, increase RBC precursors; Thrombopoietin (TPO) - hormone from liver, stimulates platelet formation; Cytokines are local hormones of bone marrow - may stimulate proliferation of other marrow cells, colony-stimulating factor (CSF) and interleukin stimulate WBC prodn.

Front 11

What is haemoglobin?

Back 11

Globin protein consisting of 4 polypetide chains; one heme pigmen attached to each polypeptide chain and each heme contain an iron ion that can combine irreversible to one o2 molecule.

Front 12

Define acquired sideroblastic anaemia

Back 12

A RBC disorder characterised by ineffective erythropoiesis, leading to increased iron absorption, iron loading and haemosiderosis (endocrine, liver, cardiac damage due to iron deposition). Acquired usually idiopathic. Hyperchromic RBS are seen on blood film with ring sideroblasts in the marrow. Treat by removal of cause, B12, blood transfusion.

Front 13

Define anaemia of chronic disease

Back 13

Microcytic hypochromic anaemia found in patients suffering chronic diseases; caused by poorly managed infection, malignant, inflammatory diseases

Front 14

What is the aetiology of Anaemia of chronic disease

Back 14

Chronic inflammatory disease eg: SLE, RA, polymyalgia rheumatica, crohn's disease; chronic infective diseases eg: Infective endocarditis, TB, osteomyelitis.

Front 15

What is the pathogenesis of Anaemia of chronic disease

Back 15

Mediated by inflammatory cytokines
Decreased release of iron from body stores for developing erythroblasts
Inadequate EPO response
Decrease RBC survival times

Front 16

What investigations do you do for Anaemia of chronic disease

Back 16

Mild normocytic anaemia (Hb >8g/dL), ferritin normal of increased. Decreased serum iron and TIBC (total iron binding capacity)

Front 17

What is treatment for Anaemia of chronic disease

Back 17

Unresponsive to iron therapy
Treat underlying condition
Trials of oral Fe + EPO promising in RA

Front 18

What is the aetiology of acquired sideroblasti anaemia

Back 18

Inherited on linked X chromosome, appearing in infants
Acquired primarily by myelodysplastic syndrome (MDS) in elderly.
Secondarily acquired myeloid leukaemia, chronic mylelogenous leukemia (CML)
Drug use, alcohol use, lead poisoning, chronic inflammation

Front 19

What is the pathogenesis of acquired sideroblasti anaemia

Back 19

Inherited on X linked chromosome, or acquired defect in the enzyme gamma-aminolaevulaic acid (ALA) synthetase which is resp for first step in haem synth.
Accumulation of iron in erythoblasts, but in absence of normal haem, iron accumulates in mitochrondria, forming a stainable ring around nucleus = ring sideroblast.

Front 20

What is the treatment for acquired sideroblasti anaemia

Back 20

Transfusions, B12 and folate, withdrawal from drugs, alcohol, lead.

Front 21

Define normocytic anaemia

Back 21

Erythocyte is normal in size but there is a low number of erythocytes.

Front 22

What is the aetiology of normocytic anaemia

Back 22

Blood loss, endocrine disease (hypopituitarism, hypothryroidism), haematological disease, liver disease, renal failure, chronic inflammation, malignancy, common cause in pregnancy. Invest: B12 and folate levels are normal.

Front 23

What is macrocytic anaemia

Back 23

is where MCV >96fL. PA is most common Macrocytic anaemia. Causes are 1. megaloblastic anaemia - b12 xu, folate xu, cytotoxic drugs. Presence of erythroblasts with delayed nuclear maturation in BM due to defective DNA synthesis,
2. Non megaloblastic: alchol, reticulocytosis, liver disease, hypothyroidism, pregnancy.
3. Other - myelodysplasia, myeloma, aplastic anaemia.

Front 24

What is pernicious anaemia

Back 24

PA is atrophy of gastric mucosa with consequent failure of intrinsic factor production which results in B12 malabsorption.
Occurs in 1 in 800 people over 60 esp in fair caucasians. A&P: autoimmune, atrophy, parietal cell antibodies, IF antibodies.

Front 25

What is A and P of PA

Back 25

autoimmune in nature; other autoimmune associations; pathological changes in Stomach, mucosal atrophy etc, parietal cell antibodies in 90% patients; IF antibodies in 50% patients

Front 26

What are clinical features of PA? Investigations?

Back 26

Anaemia, jaundice, glossitis (irreversible), neurological changes eg: progressive polyneuropathy, parestheisa, loss of proprioception, weakness and ataxia, paraplegia, dementia, delusions.
Invest: antibodies, schillings test

Front 27

What is folate deficiency anaemia

Back 27

Caused by folate xu, RBCs are large (megalocytes). May be known as megaloblastic anaemia. Caused by nutritional inadequacy.

Front 28

What is aplastic anaemia

Back 28

Collection of disorders in which the bone marrow does no produce enough Blood Cells.

Front 29

What is pathology of aplastic anaemia

Back 29

decreased number of pluripotential stem cells in Bone Marrow and faults in those remaining, failure of just one cell line may occur in isolation rarely, can sometimes evolve into myelodysplasia and ML.

Front 30

What causes aplastic anaemia

Back 30

It is a collection of d/o where bone marrow doesn't produce enough blood cells reustling in pancytopenia and aplasia of BM.
Caused by faulty immune reaction against pluripotent cells, chemical agents, radiation.

Front 31

What is inherited haemolytic anaemia

Back 31

A range of disorders characterised by increased RBC destruction. Caused by defective membrane synth. Can be intravascular or extravascular (in liver, spleen, BM). Can be grouped as inherited, acquired, misc.

Front 32

What is thalassaemias

Back 32

genetic diseases of unbalance HB synthesis, due to underproduction (or no prod) of one globin chain.
Occurs as a result of either genetic deletion or mutation.
Unmatched globins developed, damaging RBC membranes, causing haemolysis while still in the marrow.

Front 33

What is beta thalassaemia

Back 33

Usually caused by point mutations in beta-globin gens on chromosome 11, leading to decrease beta chain production of absence. B-thal minor is carrier, mild anaemia (dd; Fe xu anaemia). B-thal major - both beta-genes, severe anaemia and failure to thrive. Extramedullary haematopoiesis.

Front 34

Descibe beta thalassaemia minor

Back 34

caused due to decreased syn of beta chain. common in mediterranean area, asymptomatic, Haemoglobin usually 100-130g/l, MCV 60-70fl, RCC normal or increased

Front 35

Describe beta thalassaemia major

Back 35

Much more severe condition, little of no beta chain production, diagnosed 3-6 months.

Front 36

What are clinical findings of beta thalassaemia

Back 36

Hepatospenomegaly
Slight jaundice
Marked bone changes due to bone marrow extension
Blood transfusion req to maintain Hb levels, however this leads to iron overload. 50% mortality by early adulthood.

Front 37

What is alpha thalassaemia

Back 37

Gene defect on one of four alpha chains. Treat with blood transfusions, folate supplementation, splenectomy, bone marrow transplants.

Front 38

What is acquired haemolytic anaemia

Back 38

Increased destruction of RBC therefore low erythorcyte count and low Hb., Caused by Red cell membrane defect, Hb abnormality, acquired (drug induced, transfusion reaction = autoimmune response).

Front 39

What is normal reference range for haemoglobin?

Back 39

Men; 13-18g/dL
Women; 11.5-16g/dL

Front 40

What is normal reference range for platelets?

Back 40

150-400 x 10 to the 9th/L

Front 41

What is normal reference range for WBCs

Back 41

4-11 x 10 to the 9th/L

Front 42

What is normal reference for Mean cell volume

Back 42

76-96fL

Front 43

What is pancytopenia?

Back 43

an abnormal deficiency in all blood cells (red blood cells and white blood cells and platelets)

Front 44

Compare IDA, PA, AA, Sickle Cell by Character of RBC, Etiology and other effects

Back 44

IDA: microcytic, hypochromic, low Hb production, E: decreased dietary intake, malabsorption, blood loss.
PA: megaloblasts, short life span, E, deficit of IF due to immune reaction, Other: neuro damage, achylorhydria.
AA: normal cells, pancytopenia, E; BM damage, Other: xs bleeding and infections.
SCA: RBC elongates and hardens, haemolysis, E: recessive inheritance, other; painful crisis, infarctions, hyperbilirubinemia.

Front 45

What is sickle cell anaemia

Back 45

Inherited haemolytic anaemia where RBC membrane is defective,

Front 46

what is the tx approach for congenital haemolytic anaemia

Back 46

Thalassaemia: folate supplement, transfusions*, iron-chelators to stop deposition, Vit C to increase excretion, splenectomy, marrow transplant*. * for SSA

Front 47

What are the two haemoglobinopathies?

Back 47

SSA and thalassaemia