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Haematology Flashcards

1
Q

What is primary haemostasis?

A

Through platelet adhesion, aggregation and activation (release granules that promote coagulation and trigger cascade)

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2
Q

What is the intrinsic factor activated by?

A

Activated by collagen and activated platelets

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3
Q

What factors are involved in intrinsic pathway?

A

12 12a
11 11a
9 9a
8 8a common pathway

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4
Q

What factor if important to factor 8?

A

Note, factor 8 activated by thrombin and needs to be bound to vWF factor or will deplete

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5
Q

What pathways do APTT and PT measure?

A

APTT- intrinsic (plays table tennis- indoors)
PT- extrinsic (plays tennis- outdoors, needs tissues , 7x world champion)

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6
Q

What is the extrinsic pathway activated by?

A

Tissue factor from damaged endothelial tissue

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7
Q

What factors are involved in extrinsic pathway?

A

Factor 7

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8
Q

What can prolong PT?

A

So obviously: deficiency in factors involved which are 7, 10, 5, 2, 1 and 13
Vitamin K related:
Vitamin K antagonists ie Warfarin
Haemorrhagic disease of the newborn (secondary to low Vit K which affects 2, 7 and 10)
Intestinal reabsorption disorders
Vit K deficiency
Liver failure- production issue
Fibrinolysis
DIC (consumptive)
Clotted samples
Very high doses of heparin
** NOTE: Factor 7 lower in healthy newborns vs older kids** Also applies to other Vit K dependant factors

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9
Q

What can prolong APTT?

A

Deficiency in factors involved: 12, 11, 9, 8, 10, 5, 2, 1, 13
Presence of inhibitors – lupus inhibitors/specific factor inhibitors. Lupus anticoagulant common prolongator post infection
Heparin therapy/accidental contamination (inactivate Xa and thrombin)
LMWH can affect APTT at higher doses
Presence of para protein ie IgA myeloma due to interference with fibrin polymerisation
Clotted sample due to consumption of fibrinogen
Haemophilia

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10
Q

What is Haemophilia A? Name route of inheritance and presentation

A

Factor VIII deficiency- most severe and most common
8 is in intrinsic pathway so affects APTT
X-linked recessive
Easy bruising (ecchymosis), haematomas, haemarthrosis

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11
Q

What is a side effect of factor replacement?

A

Can develop inhibitory alloantibodies, usually after 50-100 doses
Diagnosed by inability of pooled plasma to correct prolonged APTT of patient’s plasma

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12
Q

What is Haemophilia B? And mode of inheritance?

A

Deficiency in factor 9, X- linked reccessive

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13
Q

What is Haemophilia C? Mode of inheritance?

A

Factor 11 def. Autosomal and commonly seen in Ashkenazi Jews

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14
Q

What is a rare cause of delayed bleeding from umbilical stump?

A

Factor 13 deficiency, autosomal recessive. Very rare.

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15
Q

What is VWF and VWB disease?

A

VWF is a carrier for Factor 8 and in vWB disease, there is a deficiency which means factor 8 is consumed faster

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16
Q

What are the types of VWD?

A

Type 1: QUANTITATIVE def in 70%
Type 2: QUALITATIVE def
Type 3: Total def – like Haemophilia A. Autosomal recessive and can prolong APTT.

Ristocretin sign of qualitative measure

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17
Q

Treatment for VWB disease?

A

Treatment: DDAVP. Releases vWB from granules but finite resource

THis medication is also used for diabetes insipidis.

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18
Q

Whats the most common cause of prolonged APTT post viral illness?

A

Lupus anticoagulant

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19
Q

What are causes of congnital thrombocytopenia with SMALL platelets?

A

Wiskott Aldrich syndrome and X-linked thrombocytopenia. (x-linked essentially has less severe eczema)

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20
Q

Where does haematopoeisis first commence and what is the time period?

A

Yolk sac, on day 10-12 and then until weeks 10-12. Liver takes over post.

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21
Q

When does hepatic haematopoeisis commence and what is the time frame?

A

6-8 weeks and continues all through pregnancy but starts to diminish around second trimester as myeloid begins

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22
Q

When does haematopoeisis begin in the BM?

A

Around 4 months (20-24 weeks) and this is the predominant form in third trimester

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23
Q

When does neutrophil production commence?

A

10-11 weeks and becomes the primary granolucyte after 14 weeks. Neutrophils are low until the third trimester.

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24
Q

What are granulocytes?

A

Anything that ends in phil ie basophil, neutrophil, eosinophils

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25
Post-birth, where is EPO produced and when is the peak response?
90% kidney, 10% liver. Responds within minutes to hours, peaking at 24 hours
26
Where is EPO produced in the foetus?
Usually in liver in first and second trimester, and then kidney takes over
27
How many globin meoities bind to form a hmaebologin molecule?
4
28
What is HbA?
a2b2
29
What is HbF?
a2y2- (alpha and gamma) predominates from 8 weeks gestation and peak at 24 weeks (6 months.) 70% of Hb at birth and slowly drops to 2% by 6 months Foetal- alphabetical FG- gamma
30
Where is folate absorbed?
Proximal small intestine, as methyltetrahydrofolate
31
What common antiepileptics cause malabs of folate?
Phenytoin and phenobarbital F for folate, F for Fenytoin and Fenobarbitoil
32
What is the main function of B12?
B12/Colbalmin = Coverts homocystiene to methionine (H&M)
33
What is the criticial function of folate?
For DNA synthesis
34
How are B12 and folate related?
B12 is a co-factor for folate absorption so reduces utilisation of folate.
35
What is the treatment dose for iron?
6mg/kg/day for 3 months
36
What is the limit for cows milk inake in kids ?6 months
600mls.
37
What are the factors that increase Hb oxygen affinity?
INCREASED AFFINITY so LEFT SHIFT because for the same CONCENTRATION its REDUCED saturations. Decreased temp, decreased 2-3 DPG, Decreased H+, carbon monoxide and increased fetal Hb
38
What factors reduce oxygen affinity?
REDUCED affinity so RIGHT shift as MORE saturations for SAME oxygen conc. Increased H+, increased temperate, increased 2-3DPG (technically CO2 as it causes acidosis)
39
What and where is the protein defect in hereditary spheocytosis?
cytoskeletal protein defects in Ankyrin- protein 3.
40
What is the mode of transmission for spherocytosis?
Autosomal dominant, most common in northern european
41
What can trigger an aplastic crisis in spherocytosis?
Parvovirus
42
What is the treatment for spherocytosis?
Severe: transfusion, folic acid (1mg) and spleenectomy if transfusion dependant, FTT or gallstones
43
Hereditary elliptocytosis- protein defect?
Spectrin, protein 4.1
44
What are common triggers for G6PD? (4)
Fava beans, napthalene exposure, anti-malarials, sulfamethoxazole (sulfur containing drugs). Triggered by oxidative stress
45
What is the inheritance for G6PD?
X-linked recessive
46
What is seen on the film for G6PD?
Heinz body (REMEMBER FAVA HEINZ BEANS)
47
What is in the red pulp for spleen?
splenic cords and venous sinus. Essentially removes foriegn materia+erythrocytes and stores iron, RBC and platelets.
48
What does the white pulp of the spleen contain?
25% of bodies' lymphocytes and initiates responses to blood bourne antigens. Consists of periarterioral lymphoid sheath (PALS), follicles and marignal zone
48
What does the white pulp of the spleen contain?
25% of bodies' lymphocytes and initiates responses to blood bourne antigens. Consists of periarterioral lymphoid sheath (PALS), follicles and marignal zone
49
What is seen on the blood film for hyposplenism?
howell jolly bodies
50
What is TEC?
Transient erythroblastopaenia of childhood. Diagnosis of exclusion- essentially subacute pallor and becoming unwell. Normocytic, normochromic anaemia, low retics and erythroblasts in marrow. Usually recovers in 2-8 weeks.
51
What can trigger TEC? (transient erythroblastopenia of childhood)
Viral illness, drugs, malignancy, genetic (diamond-blackfan)
52
What enzyme def causes burr cells on film?
Pyruvate kinase deficiency. (also caused by urea exposure)
53
What causes fragmented red cells on film?
Haemolytic uremic syndrome
54
What causes teardrop cells on film?
Bone marrow stress- 'BM crying because its overworked' in iron def, pernicious anaemia, renal disease
55
What is most likely to cause late-onset haemorrhagic disease? (Vit K def bleeding)
Breastfeeding! Very low in Vit K. early onset can be due to maternal drugs ie phenytoid
56
What does AML commonly present with?
gingerval hypertrophy, oral candidiasis or herpetic lesions Also chloromas (leukemic deposits in skin), bleeding, hyperleukocytosis Think, all of innate immune system affected
57
In an otherwise well toddler with significant nutritional iron deficiency anaemia, what is the expected time interval between commencing iron therapy and a reticulocytosis becoming evident on a blood film?
3 days
58
What is an EMA used for? EMA (eosin-5-maleimide) is an eosin-based fluorescent dye
EMA (eosin-5-maleimide) is an eosin-based fluorescent dye that binds to RBC membrane proteins, especially band 3 and Rh-related proteins. It is a highly sensitive and specific test but false negative results may be seen in mild cases of hereditary spheorcytosis. It can be used to distinguish hereditary spherocytosis from AIHA.
59
What is in cryoprecipitate?
Cryoprecipitate is produced by freezing fresh plasma to under -65°C, then allowing to thaw 18 hours at 4°C. After centrifugation, cryoprecipitable proteins are separated. It contains factor VIII, fibrinogen and fibronectin and concentrations greater than those of plasma. It also contains factor XIII.
60
What chromosome is responsible for alpha thalaseemia? Think of alpha males
Chrom 16- deletion 16yo males think they are alpha
61
What chromosome is responsible for beta thalassemia?
Chrom 11- point mutation
62
What are the types of alpha thalassemia?
aa/a = carrier a-/a- = MINOR a-/-- = HbH disease --/-- = Hydrops
63
What are the types of beta thalassemia?
B+ / B OR B/B0 = minor (absent production 0 or reduced +) B+/ B+ = intermedia B0 / B0 = major
64
How do you test for haemoglobinopathies
Electropheresis (look at the most common Hb), genetic testing
65
What leads to basophillic stripping on a blood film?
Lead exposure/poisoning
66
What are the 3 haemoglobins in the human body?
Hb A = A2B2 (70%) HbA2= A2Delta2 (in beta thalassemia), HbF = A2Gamma2 (Fetal haemoglobin). HbH is with alpha thalaseemia when you have 1 a.
67
What is the timing of manifestation of alpha and beta thalassemia?
Alpha- in utero because alpha Hb production begins very early. Beta thalaseemia at 6 months when beta haemoglobin starts to increase in proportion.
68
What leads to falsely low HbA2?
Iron deficiency. Basically if have beta thalassemia and iron def, your HbA2 won't rise.
69
What is lower in thalassemia- Hb or MCV?
MCV lower than Hb
70
Which infection is more common in thalassemia or in patients recieving regular blood transfusions?
Yersinia - loves iron
71
What is the most common presentation for sickle cell disease?
Pain
72
What is the problem in sickle cell disease?
Defect in beta globin gene. When oxygenated, cells are plump and round. When deoxygenated, beta globin shrivels so RBC becomes sickle shaped. Aut recessive
73
What are common complications of sickle cell disease?
1. Vaso occlusive disease- infarcts in the brain, kidneys etc 2. Extra-meduallry haematopoeisis leading to chipmunk facies, hepatosplenomegaly \ 3. Acute chest syndrome 4. Splenic sequestration: life threatening. Eventually leads to functional hyposplenism.
74
How does hydroxyurea work in treatment of sickle cell?
Increases production of HbF- prevents reshaping of cells
75
What is methaemoglobinaemia?
Iron molecules in Fe3+ state rather than 2+. So cant release oxygen to tissues.
76
What convers metHb to Hb?
Cytochrome B5 reductase
77
What are 3 common triggers of metHb?
Local anaesthetic, dapsone, nitrates/nitrites.
78
What is the presentation of Diamond Blackfan anaemia?
Macrocytic anaemia and reticulocytopenia. No other cell lines affected. Think BIG DIAMONDS Also growth failure/IUGR, FTT. Features include craniofacial, opthalmic, neck and cardiac abnormalities.
79
What is the treatment for MetHb?
Napthalene blue, riboflavin
80
What is transient erythroblastopenia of childhood?
Temporary red cell aplasia, usually viral trigger. In >4mo, resolves with time.
81
What is warm reactive autoimmune haemolytic anaemia?
caused by IgG antibodies attacking red blood cells. Occurs at 37 degrees Warmth is GOOD
82
What is the trigger for warm AIHA?
Idiopathic, SLE, lymphoma, evan's syndrome
83
What is cold AIHA?
Intravascular haemolysis mediated by IgM.
84
What is the treatment for cold AIHA?
Keep warm
85
What are the triggers for cold AIHA?
mycoplasma pneumoniae, lymphoma, EBV
86
What is paroxysmal noctural haemoglobinuria's presentation?
Haemoglobunuria, intravascular haemolysis )anaemia) and increased thrombotic tendency. Classically dark coloured urine in the morning
87
What is Glanzmaan thrombasthenia?
congenital bleeding disorder caused by a defect and/or deficiency of a platelet integrin, alpha IIb beta3. Aut recessive so common in consanguienous
88
How are the platelets in Glanzmann thombasthenia?N
NORMAL size and number, no FUNCTION
89
What is Bernard Soulier syndrome?
LARGE platelets, aut recessive. Problems in vWF receptor. (SOUNDS FRENCH, THEY ARE VERY BIG and HATE VWF)
90
What are the 3 features of WAS?
Eczema, immunodef and SMALL platelets. X linked
91
What is seen in amegakaryocytic thrombocytopenia?
No radius/ulnar. No fusion.
92
What is pulpura fulminans a hall mark of?
Protein C and Protein S deficiency.
93
What is Kostmann syndrome?
Severe congenital neutropenia
94
What is the presentation for Schwanman Diamond syndrome?
Pancreatic insufficnecy, metaphyseal dystosis, bone marrow failure, MDS/AML Has neutropenia
95
What is the presentation for Schwanman Diamond syndrome?
Pancreatic insufficiency, metaphyseal dystosis, bone marrow failure, MDS/AML. HAS MONOSOMY 7.
96
What is the common cause of AIHA
Around half of paediatric AIHA are deemed secondary (occurring in association with another condition). This may be autoimmune (SLE, JIA, T1DM, Hashimotos), immunodeficiency (CVID, Wiskott-Aldrich syndrome), malignancy (lymphoma, leukaemia, post allogeneic BMT) or infection (mycoplasma, EBV, varicella, adenovirus). Anaemia may be the first sign of one of these underlying conditions.
97
What condition can falsely increased oxygen saturations? (measured bedsite?)
Met Hb- absorbs both frequencies
98
At what level of platelets are patients symptomatic with thrombocytopenia?
<10
99
What is the half- life of platelets?
8-10 days
100
What antigen on platelets is resposible for Glanzman thrombocytopenia and neonatal alloimmune thrombocytopenia?
HPA Glanzaman in Harry Potter Academy
101
What is NAIT?
Neonatal alloimmune thrombocytopenia. HDN equivalent for platelets. Sensitisation to PATERNAL antigens in second trimester--> IgG--> destruction
102
What is the risk of intracranial bleeding in NAIT?
10-20%. Much higher than routine ITP
103
What is the most common cause of thrombocytopenia in a neonate and how long does it last?
NAIT, lasts 2-6 weeks until antibodies fizzle out
104
What conditions is JMML assocaited with?
Noonans and NF1. Noonan's can have INFANTILE JMML
105
What is the prognosis for JMML in patients with Noonans?
Much better than without!
106
What is the most common bleeding disorder in childhood?
ITP
107
Name 5 syndromes associated with thrombocytopenia
Noonans, Trisomy 13+18, Downs and Noonan
108
What is the haematological complication of Down's?
Transient myeloproliferative disorder, specific to Downs and in 10% of patients. Spon resolution by 3 months
109
Name 2 syndromes causes SMALL platelets
WAS and X-linked thrombocytopenia
110
Name 2 causes of NORMAL sized platelets with thrombocytopenia
Thrombocytopenia with absent radi. Amegakaryocytic thrombocytopenia
111
Name 4 causes of LARGE platelets in thrombocytopenia
Gray platelet syndrome, Bernard-Soulier, MYH-9 related disorders and GATA-1 mtuation
112
What is the triad for MYH-9 disorders?
Thrombocytopenia Large platelets Dohle bodies
113
What is Emicizumab? What is it used for?
Binds to activated IX and X to trigger coagulation, replacing usual function of facor 8. Great for Hamophillia A. *NEW DRUG* E for factor EIGHT- Haemophillia
114
What is the treatment for ITP?
IVIG - rapid rise in platelets in 48 hours Prednisolone- takes few days to work
115
What is Chediak Nigashi syndrome?
Chediak is white and has peripheral neuropathy. So: albinism, peripheral neuropathy and large granular leukocytes
116
What is Schwanman-Diamond syndrome?
Metaphyseal dysplasia, pancreatic insufficiency, MDS. 'Diamonds are made under pressure in the PancreaSchanwaman..'
117
What is Cohen syndrome?
Hypotonia/intellectual disability/microcephaly. Causes secondary neutropenia/MDS
118
What is cyclical neutropenia?
Neutrophil count oscillates every 21 days with fever and inflammation of the skin ie ulcers
119
What is cyclical neutropenia?
Neutrophil count oscillates every 21 days with fever and inflammation of the skin ie ulcers
120
What is the most common inherited BM failure in childhood?
Fanconi Anaemia. AR can be an X-linked. Hyperpigmentation of trunk and neck, cafe-au-lait spots, short stature, absent thumbs.
120
What is the most common inherited BM failure in childhood?
Fanconi Anaemia. AR can be an X-linked. Hyperpigmentation and malignancies of trunk and neck, cafe-au-lait spots, short stature, absent thumbs.
121
What is HLH?
Life-threatening immune activation. Presents with fever, splenomegaly, bicytopenia, raisedtriglycerides, raised ferritin. Also seizures and PRES syndrome
122
What is the difference between cryo and FFP?
Cryo has fibrinogen, FFP more factors
123
Why are platelets the highest risk for sepsis when administred?
Stored at room temp
124
What is purpura fulminans caused by?
Protein C and S deficiency Purpura fulminans is an acute purpuric rash characterized by coagulation of the microvasculature, which leads to purpuric lesions and skin necrosis.
125
What is factor V leiden deficiency?
UNABLE TO CLOT. THOMBOPHILIA You LeiDEN and bleed everywhere
126
In allo immune thrombocytopenia, the most common human platelet antigen to cause sensitisation is what?
HPA-1a
127
Why is blood irradiated before transfusion?
To prevent GVHD
128
What is the most common inherited thrombophilia syndrome?
Factor V Leiden def. Aut DOMINANT mutation in factor 5 causing it to NOT be deactivated by protein C. Factor 5 is a dominant individual who CLOTS all over when he LeiDens because it wont come close to C.

RACP Written (18 decks)

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