Haem Docs Flashcards
What condition is a pencil cell seen in
Iron deficiency
Name 2 scenarios in which a tear drop poikilocyte would be seen
Myelofibrosis
Extra-medullary haumatopoeisis
When would a basket cell be seen in
Oxidative damage e.g G6PD deficiency
What condition could Bite cells be seen in
G6PD deficiency
or any haemolysis
Name 2 common and 2 rare causes of Microcytic, Hypochromic anaemia
Thalassaemia + Iron Deficiency anaemia
Lead poisoning + Sideroblastic anaemia
What kind of anaemia would haemolytic anaemia present with
Normochromic, Normocytic
What are the two types of macrocytic anaemia
Megaloblastic
Non-megaloblastic
Name the most common cause of Megaloblastic, Macrocytic anaemia
Vitamin B12 or folate deficiency
Name 3 causes of Non-Megaloblastic, Macrocytic anaemia
Alcohol
Liver disease
Hypothyroidism
What does kolionychia indicate
Iron deficiency anaemia
Bone lesions + anaemia suggest which underlying disease process
Thalassaemia major
What does a low reticulocyte count indicate and name 2 situations when this might occur
Low count = decreased production
Chronic Kidney Disease + Aplastic anaemia
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
A beefy tongue indicates what pathology
B12/folate deficiency
Angular stomatitis indicates what pathology
Iron deficiency anaemia
How is functional iron status assessed
Haemoglobin levels
How is iron transport status assessed
Serum iron
Transferrin
Transferrin saturation
What is a normal transferrin saturation level
20-50%
What are bound and unbound transferrin molecules called
Apotransferrin –> unbound
Holotransferrin –> bound
Name a situation when increased transferrin saturation and decreased transferrin saturation could be seen
Increased –> iron overload
Decreased –> iron deficiency
How is storage iron assessed
Serum Ferritin
Name the mucosal and serosal iron transporters
Mucosal –> DMT-1
Serosal –> Ferroportin
Which substance inhibits Ferroportin and when might this process be faulty
Hepcidin
Heriditary Haemochromatosis
What kind of anaemia will Anaemia of Chronic disease most commonly present as
Normochromic, Normocytic anaemia
sometimes mildy microcytic
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
