BCR TBL 9: Disorders of Red Blood Cells, Platelets and Clotting

What globin subunits is HbA1 made of

2 Alpha 2 Beta

What Globin subunits are HbA2 made of

2 Alpha 2 Delta

What are some common symptoms of anaemia

Breathlessness Fatigue Headaches Palpitations Dizziness

Clinical Signs of Anaemia (Not presentation

)

Pallor Tachycardia Systolic Murmur Cardiac Failure

Rare signs of Anaemia

Koilonychia Jaundice Bone Deformities Leg Ulcers

What is Koilonychia

Spoon-shaped Nails

What kind of anaemia is jaundice found in

Haemolytic anaemia

if you have low Mean corpuscular volume what kind of anaemia might you have

Hypochromic

if you have normal MCV what kind of anaemia might you have

Normochromic

if you have highh MCV what kind of anaemia might you have

Hyperchromic

What are the 4 main causes of anaemia

Inherited Haematinic Deficiency EPO Deficiency Bone Marrow issues

What are Haematinic Deficiencies

Iron B12 Folic Acid

What are the 3 main inherited issues

Red cell membrane Haemoglobin Metabolic

What are 2 examples of red cell membrane defect anaemia

Spherocytosis Elliptocytosis

What are some haemoglobin abnormality anaemia

Thalassaemia Sickle Cell

What are the 6 main acquired anaemia categories

Immune Drug induced Acquired membrane Mechanical Infection Secondary to systemic disease

What are some autoimmune anaemia reasons (Antibody)

Warm Cold Biphasic

What are some Alloimmune reasons for anaemia

Transfusion

What is an example of an acquired membrane defect anaemia

Paroxysmal Nocturnal Haemoglobinuria

If you have iron deficiency what happens to your red blood cell produce in terms of size

Smaller

If you have B12 or Folic Acid deficiency what happens to your red blood cell produce in terms of size

Bigger

What is a RBC lifespan

3 Months

How long does it take to produce a red cell

3 Weeksq

What are the stages of red cell maturation after stem cell

BFU erythroid CFU erythroid Pro Erythroblast Erythroblast Reticulocyte Erythrocyte

How long is EPO dependence in red cell maturation

10-13 days

How long is iron Dependence in red cell development

10 days

Where is iron mainly stored

Liver

What transmembrane channel is iron taken in by

DTM-1 HCP-1

How much iron can we absorb when its in haem form

20-25%

What is the protein that stores iron

Ferritin

What is the transporter that puts iron into blood

Ferroportin

Why dont you want free iron in blood

Toxic

What converts ferrous iron to ferric iron

Hephaestin

What is free iron binded to to make it non toxic

Transferrin

what regulates iron absorption

Hepcidin

What allows iron to be released from transporter in vesicles in consumer cell

Lowered pH

Where is iron stored in Liver

Parenchymal cells kupffer cells

What is the main function of Kupffer Cells

Phagocytosis Breakdown of old RBC

Where is iron stored in Kupffer cells

Haemosiderin

What detects low or high iron

Iron responsive elements

What regulates production of iron transporters

Iron Regulatory Protein

What does hepcidin do

Inhibit Absorption of Iron

What is stomatitis

sore tongue and mouth

What is angular Cheilitis

Inflammation of corner of mouth

How to diagnose iron defeciency

Ferritin and transferrin saturation

If someone has iron deficiency what will their Transferrin Saturation and Ferritin be

Less that 20% Low

What is the risk of intravenous iron

Anaphylaxis

What does intracellular folate act as in dna synthesis

Coenzyme

Where do you find folate

Raw Green Veg Liver

What can Methotrexate(a chemotherapy) cause

Pancytopenia

3 main presentation of folate deficiency

Pancytopenia Macrocytic anaemia Glossitis

What is glossitis

Burning sensation on tongue

What can hyperlobulated nucleus in neutrophils show in regards to folate

Folate deficiency`

how do you deal with methotrexate folate issues

Intravenous Folate

how is B12 isolated in body (Before intestine)

Separated from protein by stomach acid

What binds to B12 to protect it from degradation

Haptocorrin

What do gastric cells secrete thats needed for B12 absorption

Intrinsic Factor

Where is B12 Stored

Liver

What is Achlorhydria

Stomach isnt acidic enough

Presentation of B12 deficiency

Macrocytic Anaemia Pancytopenia Glossitis Neuropathy

What kind of bone marrow in Aplastic anaemia

Hypocellular with no infiltrate or fibrosis

What kind of bone marrow is this

Normal

What kind of bone marrow is this

Aplastic

Blood Results for Aplastic Anaemia for severe and Very Severe

Neutrophils < 0.2 Platelets <20 Reticulocytes < 60

Bone Marrow Cellularity for Aplastic Anaemia for severe and Very Severe

<25%

How to treat Aplastic Anaemi

Immunosuppression Transfusion Stem cell transplant

What is the Immunosuppressant in aplastic anaemia

ATG

How does Aplastic Anaemia present

Normocytic with reticulocytopenia thrombocytopenia neutropenia

How does pure red cell aplasia present

Normocytic with reticulocytopenia

What binds to haemoglobin so it can be metabolised by liver

haptoglobin

if there isnt enouigh haptoglobin what happens to haemoglobin`

oxidised into methaemoglobin

What binds to heme groups so it can be metabolised in liver by macrophages

Hemopexin

What does heme bind to when Hemopexin is saturated and what does it form

Albumin Met-Haemalbumin

Methaemoglobin is metabolised in kidney. What does it become

Haemosiderin

What kind of antibody is IgG (Warm , Cold, Biphasic)

Warm Biphasic

What kind of antibody is IgM (Warm , Cold, Biphasic)

Cold

blood tests for Haemolysis

FBC Reticulocytes Increased Bilirubin and LDH DAT

What is Warm Autoimmune Haemolytic Anaemia (wAIHA) due to (Antibodies)

IgG

What is the usual presentation of Autoimmune Hemolytic Anemia

Acute with severe anaemia and jaundice

How to confirm wAIHA

DAT positive IgG C3D complement

Spherocyte

Reticulocyte

What is the first line treatment for wAIHA

steroids folic acid transfusion

What is the second line treatment for Autoimmune Hemolytic Anemia

Splenectomy Intravenous Immunoglobulins Immunosuppressants

What is Cold Haemagglutinin Disease (CHAD) due to (Antibodies)

IgM

How to confirm Cold Haemagglutinin Disease

DAT positive for C3d

Red Cell Agglutination

How to treat Cold Haemagglutinin Disease

Keep Warm Steroids Transfusion Folic acid Immunosuppressants Complement Targeted Treatment

What is Paroxysmal Cold Haemoglobinuria (PCH) due to (Antibodies)

IgG

How to confirm PCH

Donath Landsteiner Stest

What is the Donath Landsteiner Test

Incubate Blood at 4 degrees Warming causes Haemolysis

Presentation of Spherocytosis

Jaundice at birth Chronic Haemolysis Gallbladder Stones Hyperbilirunemia

What is Cholelithiasis

Gallbladder Stone

How to treat Spherocytosis

Folic Acid Splenectomy Cholecystectomy

What is Elliptocytosis

Defect of Protein 4.1 Which is autosomal recessive

What doe sG6PD do

Protect Red Cells from damage

What happens if you have G6PD deficiency

Acute Intravascular Haemolytic Crisis

What is Pyruvate Kinase Deficiency due to

Autosomal Recessive PKLR gene

What kind of disorder is Pyrimidine 5’ Nucleotidase Deficiency

Autosomal Recessive

What is Paroxysmal Nocturnal Haemoglobinuria caused by

Acquired Membrane defect Phosphatidylinositol Glycan Class A (PIGA)