Haematology Flashcards by John Darlington

What is Haemophilia A? What are the sub-categories?

Factor VIII Deficiency

Mild - surgical bleeding

Moderate - minor trauma bleeding

Severe: - spontaneous bleeding

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How is haemophilia A transmitted?

X-linked

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How does Von Williebrand disease present?

How is it transferred?

Mucosal Surface bleeding

Heavy periods

Woulds bleed heavily

*autosomal dominant

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What other factor will be low in Von Williebrand disease, and why?

Factor VIII - it prolongs the half life of the factor.

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Name two severe platelet disorders and the underlying pathology:

How are these transferred?

Glansmanns Thrombasthemia:
- lack GP IIb/ IIIa

Bernard Soulier Syndrome:
- lack GPIb/ V/ IX

*autosomal recessive

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In Lupus Anti-coagulant disease, what would you expect to see on tests, and how does this correlate to in the body?

Increased APTT time

Paradoxically increased thrombus formation in the body.

DRVVT pre-longed. fixed with excess phosoplipids.

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What is the pathology of Lupus causing increased APTT?

Antiphospholipid antibodies

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How much Fe2+ is in the R.E.S?

200-500mg.

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What disease may skew the serum ferritin results and why?

Inflammatory disease such as RA.

Because Serum ferritin is a inflammatory protein.

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What “other” symptoms may be present in iron deficient anemia?

Koilonychia

Atrophic Glossitis

Angular Stomatitis

Oesophageal web

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If 200mg of ferrous sulphate is given, what is the elemental amount of iron absorbed?

60mg - 30%

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Why do people develop anaemia in pro-longed inflamamtion/ disease?

prolonged inflammation increases Hepcidin.
this reduces absorption and release from R.E.S

ILs released from inflammation may also shut down marrow activity.

EPO is also supressed

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In anaemia of chronic disease, what different lab findings would you have to iron deficient anaemia?

ESR will be raised

Ferritin levels will be raised

Total iron levels will be reduced

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What receptors do B12 bind with in the gut and where are these predominantly found?

Cubulin

in the ileum.

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How is B12 transported in the blood?

Transcobalamin

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What are some symptoms of B12 deficiency?

Anaemic symptoms

Mild jaundice - ineffective RBC production

Neurological disturbances - especially the posterior pyramidal tracts

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In megoblastic anaemia, what do the blood cells look like?

Macrocyctic

Very Red

HIgh MVC

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What chromosome is globin A made on?

Chromosome 16

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What chromsome is Globin B, Gamma and delta made on?

Chromosome 11

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How many genes code for Alpha globin?

4 genes

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If there is 1 gene missing from Alpha globin, what is the disease state?

Alpha thalassemia trait - mild microcytosis

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If the person has 2 gene missing for alpha globin, what is the disease state?

Alpha Thalasemia minor

microcytosis
Low MVC
increased red count

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If there is 3 genes missing from alpha globin, what is disease state?

Hb Disease

leads to haemolysis in spleen due to bizzare shape

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what is it called when there is 4 genes missing from alpha genes?

Barts Hb.

incompatible with life.

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What is beta thalassaemia major?

both genes for beta globin missing. Symptoms appear after fetal globin is gone. Extra-medullary areas try and produce RBCs - especially skull

What is another complication of Beta thalassaemia?

Haemochromotosis - hepcidin is reduced in order to try and increased Fe2+ levels.

What is the genetic mutation in sickle cell disease?

Non conservative single base pair change to the Beta globulin chain. - thus affects children >6 months glutamie to valine.

What are some serious complication of sickle cell disease?

Vaso-occlusion - tissue hypoxia - stroke - infarction

What disease causing changes in the membrane leading to sphere like appearance and what is the protein defective?

Hereditary spherocytosis. Spectrin

What is the outcome of Hereditary spherocytosis?

haemolysis in the spleen. RBCs unable to fit through mesh.

What disease leads to RBCs being unable to cope with oxidative tress?

Glucose - 6 - Phosphate DEhydrogenase Deficiency. lack of Glutathione

What immunoglobulin is indicating in warm type haemolytic anaemia?

IgG

What immunoglobulin is indicated in cold type haemolytic anemia?

IgM

What is the initial treatment for warm haemolytic anaemia?

Steroids blood transfusion is patient is gravely ill and can't wait for steroids to work Splenectomy

What is the name of Glandular fever? and what virus causes it?

Infectious mononucleosis Epstein Barr virus

What two important LFT may be elevated in haemolysis of RBCs?

Bilirubin Lactate dehydrogenase

In Immunothrombocytopenia would you expect to see an enlarged spleen?

No - the platelets are too small

Can amoxicillin be given for Glandular fever?

No. causes bad rash. if antibiotics are needed then only Penicillin B

What factors are used up in Haemolytic Uraemic Syndrome?

Platelets

If the spleen is removed, what kind of RBCs may be seen that still contain their DNA?

Howell Jolly Body cells

Which disease is much more severe when the spleen is removed?

Malaria

If a person has sphere shaped RBCs and a coombes test is performed and it comes back negative, what does this mean?

means they don't have auto immune disease and that their sphere shaped cells is due to autosomal dominant sphereocytosis

What blood disease is associated with other autoimmune disease, and will cause CNS dysfunction?

Pernicious anemia

What is a paediatric vasculitis that causes strange rash formation?

Henoch Scholien

If there is a purpuric rash, what type of meningitis can this be a symptom off?

meningococcal sepsis

Thombophilia Inherited affects what? and how is it inherited?

Anti-thrombin Protein C Protein S Autosomal dominant

How is Beta thalaseamia inherited?

Autosomal recessive

Who is most likely to inherit Spherocytosis?

Northern Europeans - autosomal dominant

How is G-6-PD inherited and who is most likely to get it?

X-linked. Africans.

What activates protein C and protein S, and what does it need to bind with to be activated?

thrombo-modulin. Needs to be bound to IIa

What infections are of particular concern when a person has their spleen removed and how is this managed?

Nisseria Meningitis Haemophilus Influenza strep pneumonia Treated with prophylactic amoxicillin for life and vaccinations

What disease may Heinz bodies and bite cells be seen?

Glucose - 6 - dehydrogenase deficiency

What is something you must always ask a Sickle Cell patient?

Have they got chest pain, or cough. thinking about acute chest syndrome

What kind of haemolysis is sickle cell disease?

Intravascular

Where is an aspirate of bone marrow taken from?

Iliac Crest

Whats it called when CO2 dissolves into the RBC?

Chloride Shift

How do the macrophages of the RES get iron?

Break down of old RBCs

How much is each mmol of Ferritin inequivalent too of iron stores?

1mmol/L = 8mg of Iron

What is it called where there is a reduction in the RES Iron stores, but the bone marrow maintains a normocytic anaemia by using what there is?

Latent Iron Deficiency - 20% of females have this Low ferritin but normalcyctic cells

Whats the most common anaemia in hospital patients?

Anaemia of chronic disease

Generally speaking what does roulex in the RBCs mean?

High ESR but can be associated with myeloma

What are the key roles of B12?

Methylation of homocysteine to methionine Methylone isomerization of CoA

What skin conditions may need increase folate and B12 intake?

Psoriasis

If you have petechia, what is this suggestive off?

Small capillary bursts with platelet disorders

How do you treat platelet disorders?

Platelets transfusion - HLA matched Tranexamic Acid

What may someone have if they continually get DVTs?

Thrombophilia Deficiencies: Antithrombin *most common Protein C Protein S

Name the types of warm haemolytic anaemia, and the management of this:

Primary - idiopathic Secondary - acquired. SLE, CCL, Non-hodgkin lymphoma Steroids Blood transfusion - those that can't wait for steroids to work Folic Acid Splenectomy - S. Pneumoniae - H. Influ - N. Meningitis

What your investigations into Anaemia?

FBC Blood Film Iron Ferritin Transferrin/ TIBC B12 Folate LFTs - haemolytic causes Chest x-ray - suspicious of cancer *check for bleeding Serum electroplates if suspect Bence jones Comb's test if suspecting anything

What is the relationship between Ferritin and TIBC?

Indirect - as one goes up the other will go down. this is important to establishing between anaemia of chronic disease and iron loss anaemia

Haematology Flashcards

(69 cards)