Haem Docs Flashcards by Shubham Agwan

What condition is a pencil cell seen in

Iron deficiency

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Name 2 scenarios in which a tear drop poikilocyte would be seen

Myelofibrosis

Extra-medullary haumatopoeisis

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When would a basket cell be seen in

Oxidative damage e.g G6PD deficiency

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What condition could Bite cells be seen in

G6PD deficiency

or any haemolysis

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Name 2 common and 2 rare causes of Microcytic, Hypochromic anaemia

Thalassaemia + Iron Deficiency anaemia

Lead poisoning + Sideroblastic anaemia

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What kind of anaemia would haemolytic anaemia present with

Normochromic, Normocytic

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What are the two types of macrocytic anaemia

Megaloblastic

Non-megaloblastic

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Name the most common cause of Megaloblastic, Macrocytic anaemia

Vitamin B12 or folate deficiency

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Name 3 causes of Non-Megaloblastic, Macrocytic anaemia

Alcohol
Liver disease
Hypothyroidism

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What does kolionychia indicate

Iron deficiency anaemia

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Bone lesions + anaemia suggest which underlying disease process

Thalassaemia major

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What does a low reticulocyte count indicate and name 2 situations when this might occur

Low count = decreased production

Chronic Kidney Disease + Aplastic anaemia

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What does a high reticulocyte count indicate and name 2 situations when this might occur

High count = increased loss/destruction of RBC’s

e.g. bleeding or haemolysis

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A beefy tongue indicates what pathology

B12/folate deficiency

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Angular stomatitis indicates what pathology

Iron deficiency anaemia

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How is functional iron status assessed

Haemoglobin levels

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How is iron transport status assessed

Serum iron
Transferrin
Transferrin saturation

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What is a normal transferrin saturation level

20-50%

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What are bound and unbound transferrin molecules called

Apotransferrin –> unbound

Holotransferrin –> bound

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Name a situation when increased transferrin saturation and decreased transferrin saturation could be seen

Increased –> iron overload

Decreased –> iron deficiency

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How is storage iron assessed

Serum Ferritin

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Name the mucosal and serosal iron transporters

Mucosal –> DMT-1

Serosal –> Ferroportin

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Which substance inhibits Ferroportin and when might this process be faulty

Hepcidin

Heriditary Haemochromatosis

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What kind of anaemia will Anaemia of Chronic disease most commonly present as

Normochromic, Normocytic anaemia

sometimes mildy microcytic

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What will serum iron and serum ferritin levels be like in anaemia of chronic disease

serum iron reduced | serum ferritin normal or raised

Where are alpha and beta thalassaemia's more common respectively

Alpha --> Far east | Beta --> Mediterranean

What is HbH disease and how would it present

only one alpha gene remains so excess beta chains form tetramers called HbH Presentation --> Microcytic anaemia + jaundice + splenomegaly

How is HbH disease treated

Splenectomy and transfusions

What is Bart's Hb

Gamma tetramers | Seen in Barts Hydrops Fetalis

What is the difference in types of mutations in alpha and beta thalassaemias

Alpha --> Deletions | Beta --> Point mutations

Which blood result is diagnostic of Beta thalassaemia trait

Raised HbA2

What disease causes a "hair on end" appearance of the skull and why

Beta Thalassaemia Major | Extra-medullary haematopoeisis

Name a serious endocrine, cardiac and hepatic consequence of iron overload

Cardiac --> hypertrophy Hepatic --> cirrhosis Endocrine --> diabetes

How can iron overload be managed

Iron chelating drugs such as desferrioxamine

What kind of mutation on which codon causes sickle cell anaemia

Point mutation on codon 6

What is hand-foot syndrome and which serious disease can it be the presenting complaint of

Redness, swelling, and pain on the palms of the hands and/or the soles of the feet Sickle cell anaemia

How is sickle cell anaemia treated

Folic acid supplementation Hydroxycarbamide --> induce HbF production Penicillin + vaccination if hyposplenism

Why might Hyposplenism occur in Sickle cell anaemia

Multiple infarcts

If a patient with sickle cell anaemia contracts the Parvovirus B19, what could occur

Aplastic crisis | low reticulocytes

If someone with sickle cell anaemia presents with abdo pain, hepato-splenomegaly and severe haemolytic anaemia --> what is going on

Sequestration crisis: pooling of blood in spleen

Which two substances are released by platelets to stimulate aggregation and adhesion

Thromboxane A2 | ADP

How would primary haemostasis failure present

Purpura Easy bruising Mucosal bleeding --> epistaxis, conjunctival bleeding, menorrhagia, GI bleeds

What do platelets bind to each other via

Glycoprotein IIb/IIIa

Name the 4 vitamin K dependent clotting factors

II, VII, IX, X

What are the 2 main factors in the extrinsic pathway and how is the pathway tested

``` Tissue Factor & Factor VII Prothrombin Time (PTT) ```

What are the 2 main factors in the intrinsic pathway and how is the pathway tested

Factors VIII & IX | Activated Partial Thromboplastin Time (APTT)

Name a serine protease which controls haemostasis

Anti-Thrombin III

Name 2 naturally occurring anti-coagulants

Protein C and S

What is haptoglobin and what does a low level suggest

Binds free haemoglobin in the blood to prevent oxidative damage Low level suggests intravascular damage

Which disease is an autoimmune condition in which antibodies are produced against platelets

Idiopathic Thrombocytopenic Purpura (ITP)

What are Howell-Jolly bodies and when are they seen

Basophilic nuclear remnants seen in blood cells | Indicate Hyposplenism

Schistocytes on blood film + fever + mucosal bleeds + CNS signs should make you think of which disease

Thrombotic thrombocytopenic purpura (TTP)

How is Thrombotic thrombocytopenic purpura (TTP) managed

Plasmapheresis Steroids + immunoglobulin Splenectomy

Which disease can you use desmopressin for and how does it work

vWF disease | Stimulates vWF release from endothelial cells

Which emergency consists of thombosis and haemorrhage together

Disseminated Intravascular Coagulation (DIC)

How is Disseminated Intravascular Coagulation (DIC) treated

Treat underlying cause Consider RBC + platelet transfusions Give cryoprecipitate after fresh frozen plasma

Which type of heparin only inhibits factor Xa

LMWH | e.g. dalteparin

Name 2 antibiotics that potentiate the effect of warfarin (increase INR)

Erythromycin | Cirprofloxacin

When should warfarin be stopped with regards to an upcoming surgery

5 days before

Name a direct thrombin inhibitor and it's antidote

``` Dabigatran Idarucizumab (antidote) ```

Which anti-coagulants don't require monitoring

NOACS: Noval Oral Anti-coagulants

Name the 3 parts of Virchow's triad

Stasis Hyper-coagulability Vessel wall damage

What is the age threshold, under which you should consider thrombophilia, if a VTE occurs

< 40

What is the most common acquired thrombophilia

Anti phospholipid syndrome

What are the primary sites of haematoiesis in kids

Long bones of the limbs

What are the primary sites of haematoiesis

vertebrae, sternum and ribs

At what stage of the red cell development does Hb begin to be produced

Polychromatic normoblast

At what stage of the red cell development does the nucleus shrink

Orthochromatic normoblast

What process do red blood cells rely on for energy

glycolysis

What does a red cell use NADH for and why

maintain iron in the Fe2+ (ferrous) state as it needs to be in this state to bind oxygen

What is the precursor cell of platelets

Megakaryocytes

Which cells have a segmented nucleus

Neutrophils

Name 2 conditions in which neutropenia may be present

Aplastic anaemia | Pancytopenia

Eosinophilia may be present in which blood cancer

Hodgkin's lymphoma | CML

Which granulocyte is least common

Basophils

When might you find a basophilia

CML | Polycythaemia Rubra Vera

Where is iron absorbed and how much is needed per day

Duodenum | 1mg

How is iron overload defined

Total iron stores > 5g

If a woman has symptoms of iron deficiency anaemia and webs in her throat, what syndrome should you suspect

Plummer Vinson syndrome

What are heinz bodies and when are they seen

Clumps of denatured Hb | Seen in Intravascular Haemolysis

What are Pappenheimer bodies

abnormal basophilic granules of iron found inside red blood cells

What disease might Pappenheimer bodies be seen in

Beta Thalassaemia major

What are the two antibodies that may be present in Pernicious Anaemia and which one is more specific

Anti-gastric parietal cell | Anti-intrinsic factor (more specific)

What is MAHA (microangiopathic heamolytic anemia )

Intra vascular heamolysis due to mechanical disruption of red cells leading to their fibrosis and release of intracellular components

Donath Landsteiner antibodies = what

Paroxysmal cold heamoglobinuria

What does cigar shaped RBC's suggest

Heredity Epliptocytosis

What are the two main causes of cytopenia

Hypersplenism | Reduced production

Which blood cancer is Fanconi Anaemia associated with

Increased risk of AML

Which chains are produced slightly more than the other, light or heavy

Light chain

Which immunoglobulins are most myelomas

IgG | IgA

Which paraprotein disease has excess IgM production

Waldenstrom's Macroglobulinaemia

What appearance can someone's skull have on X-ray if they have myeloma

Pepper-pot skull

``` What will be seen on : Blood film Serum Electrophoresis Urine electrophoresis of someone with myeloma ```

Blood --> Rouleaux formation Serum --> Paraprotein Urine --> Bence- Jones protein

Which effect of myeloma can cause strokes

Hyper-viscosity syndrome

What are the parameters of Paraprotein (g/l) and bone marrow plasma cells (%) under which you would diagnose Monoclonal Gammopathy of Unknown Significance (MGUS)

< 30g/l paraprotein | Bone marrow plasma cells <10%

Lymphoplasmacytoid Lymphoma describes which disease

Waldenstrom's Macroglobulinaemia

What are the 3 B symptoms of lymphoma

Night sweats Fever Weight loss