haematology Flashcards
glanzmaen thrombostenia
bleeding disorder that is characterized by prolonged or spontaneous bleeding starting from birth. People with Glanzmann thrombasthenia tend to bruise easily, have frequent nosebleeds (epistaxis), and may bleed from the gums.
Immune thrombocytopenic purpura alternative name and what is it
Werholf
is a type of thrombocytopenic purpura defined as an isolated low platelet count with a normal bone marrow in the absence of other causes of low platelets so diagnosis requires exclusion. autoimmune + .
characteristic rash palpable
type of anemia in iron deficiency
diamond blackfan labs cause
microcytic
red blood cell aplasia - a plastic anemia congenital, all rbc lines will be gone so no rteiculocytes its a macrocytic anemia, dodgey thumbs
cause seems to be ribosomopathy
TX- CS , RBC transfusions
sick cell anemia is
AIHA tx and siagnosis
NORMOCYTIC
immunosuppresnat till yu get a negatuc coombs test
coombs test and spheroctosis !
anemia of chronic disease
microcytic (lecture)or normochronic(google)
haemolytic anemias are what type
pearson syndrome
normocyitc?
siderblastic anemia
mitcohondiral problem
macrocytic
liver prolems
lactic acidosis
which 2 conditions have anemia but increased RBC
SICKELE CELL AND THALLASEMIA
fanconi, diamond, pearson
constellation of thalassemia
fanconi anemia and siagnosis
low hb, low MCV! increased abc
congenital , short, onset around 4 years old
cafe au lait spots
malformations of urinary trcat
increased chromosome fragility !
how to prove thalssemia
tx glucos 6 phosphate
electrophoresis of the chains
rarely need transfuison just stop the ttrigeer supportive tx xygen etc
theory for hemmorrhaigc disease of newborn
- IV vit k 1mg/kg bw for 3 days
2. FFP 10-15ml preterm
TTP VS ITP
check tables
TTP
platelet count not a slow as iTP
but clots form and platelets low
you have haemolytic anemia (shsitocytes)
why would you do a urine electrophoresis
prove whether protein is tubular or glomerular
Type of child is more likely to present with jaundice in a uni tract infection
<3months
uti main symptoms
FEVER ESPCIALLI IN YOUNG KIDS
FAILURE TO THRIVE
JAUNDIC <3
<3
>3 YEARS
<3 urgent microscopy +culture
>3 leukocyte esterase +nitrate+dipstick
criteria for pyelonephritis
> 10*3
criteria for lower utilities
<10*3
atypical UTI
non immune causes of hemolutic anemia
poor urine flow , failure to respond to tx whiting 48 h, non e.coli organisms
membrane deficits : minkowski
enzyme : glucose 6 p
microangiopathic : dic, ttp, hus
macroangiopathic : prosthetic devcies
decreased reticulocyte count
bone marrow failure
normal reticulocyte count
NORMAL PLT COUNT
SEVERE THROMBOCYTPOENIA
0.5-2.5%
> 150
<20 -spontaneous bleeding
lead poisoning
microcytic anemia and basophils stippling
gum grey line
constipatin and vomititng
tx of anemia of chronic disease
need to treat the underlying disease if you just give iron - useless because its not the fact that you dont have enough its that YOU CANT USE THE IRON THERE
normal blood volume of term baby
where is iron absorbed
80 ml
duodenum
cafe au lait spots
falcon anemia
pancytopenia
typically presents around 8 years old
BM transplant + androgen therapy(raised ur rb + plt)
type of anemia of aplastic anemia
charcarcteristics of aplastic anmeia (lecture slide)
MACRO
necrotic lesions at entry sites - buccal mucosa and perianal lesiosn (neutophils) lack of lymph nodes and abscenes of HSM
SIGNS OF THALAMSEIA MAJOR
FRONTAL BOSSING
MAXIALLARY OVERGROWTH
EXTRAMEDULLARY HEMATOEPOESISASIS
TX thalsemmia
chelating : desferroxiamine
splenectomy
bm transpalnt
alpha thalseemia major
not compatible with life most fetuses are still born or die in utero- hydrops fetalis
when should you suspect thalssemia
when mcv is way lower than hb
thalaseemia labs
MCV- very low
HB low
high levels of fatal hb >90%
HBA - absent
what would electrophoresis show in thalassemia
increase hb f or increased a2
specific cells thalseemia
target cells
Guthrie test
Best for sickle cell anaemia heel prick test newborn
tx sickle cell
increase fetal hb
hydrocarbamide
BM TRANSPLANT IF FAILS
Minkowski chauffeur disease inheritance
triad
ad
hemolytic crisis
splenomegaly
spherocytes
diagnosis of Minkowski
Enzyme is typically low but during the crisis it actually will be high so you do repeat after
Minkowski chauffer symptoms
gallstones
family history
splenomegaly
abnormal rbc morphology(spherocytes))
Heinz body
glucose 6 phospahte
treatment for Minkowski
folate supplements
rbc transfusions
splectomy- but try to avoid till after 7
Minkowski chauffer type of anemia
normocytic
osmotic fragility test done for
glucos 6 ph symptoms
thlassmia and hedrideaty spehrocytosi
fever, jaundice, adominal pain, DARK COCO COLA URIEN
tx for minnowki
folate supplements !!!!!!! splenectomy > 7 to avoid sepsis before splenectomy give vaccines blood transfusion in crisis cholecystectomy
blood smear minnows
diagnose for G6P
spheorcytes
enzyme assay but only after reticulocytes have normlaised!!!
lack central pallor and smaller than rbc
labs minnkowski
drinking goats milk you are deficent in
increased br indirect
LDH
increased reticulocytes
decreased haptoglobin (haemolytic)
folate!!!
signs of hemolysis
ldh
decreased haptoglobin
br
exotic face is seen in
coca cola urine implies
thalassemia
intravscular hemolysis! like g6pd
specific values for minkwoski
normocytic (size)
but mch is increased (hb)
dx of glanzeman disease
normal with ristocetin but abnormal with adrenalin and adp
dx of vwf
abnormal with ristocetin
aptt increased
main type of bleeding in VWF
MUCOSAL IS LEADING
dead definition of hypertension
> 95% on at least 3 different occasions
classification of blood pressure
Normal: <90th BP percentile
x Prehypertensive: 90th -95th
x Stage 1 HTN: 95th – 99th (+5mmHg)
x Stage 2 HTN >99th (+5mmHg)
What type of hypertension are babies more likely to have
secondary
HTN In teens
most likely essential hypertension
causes of hypertension in newborns
umbilical artery catheterisation + renal artery thrombosis
Metanephrines
used to check for pheocytochroma
1st line treatment for hypertension in kinds
calcium channel blocker
signs of hypertensions
headache seizures nose bleeds stroke, mi
Hypertensive criss vs hypertensive urgency
crisis: acute increase in both systolic BP and DBP with end organ damage.g stroke, encephalopathy
urgency: without end organ damage
tx for HTN crisis
ICU, parenteral drugs CA channel blocker nicarpidine , labetelol, esmolol, sodium itroprusside . once stabilised then be on Aral antihypertensives over 1-2 days
tx urgency hTN
ORAL 1-2 DAYS
what’s the most important thing to rememebr about a child with UTI
if the child has a fever but there are no other signs of infection! UTI !!!!
markers for increased consumption
thrombin time (DIC has thrombin excess!)
d dimers
which lab parameter does vit k mainly affect
PT time ( extrinsic) as majority factors are there
pathophys of minkowski
change of shape, loose concavity and become spherocytes and more prone to stasis and so get stuck in the spleen.EXTRAVASCULAR hemolysis
what is the mutation in SS
glutamate replaced by valine
HEMOPHILIA A
factors dependant on vitamin K
factor 8
2 7 9 10 +protein C +S
diagnosis of hemophilia
- fH
- apTT prolonged but normal pT
when would parents typically bring child in
when learning to walk
keep bleeding after teeth eruption or circumcision
dx of hemophilia
MONOARTHRITIS - you cant tell the difference the only clue perhaps would be brusing in hemophilia
complications for hemophilia
immobility due to stiff muscles which healby fibrosis - hematomas
nerve damage
vasculitis?
joint defromities
what drugs are CI in hemophilia
aspirin and nsaids (upper gi bleeding risk)
when can we use desmopressin
hemophilia A its uneffective for hemophilia B
also for VWF
what does VWF do
has 2 functions
1) allows the platelets to adhere together
2) binds to factor 8 to keep it activated
type of bleeding in VWD and tX
mucousal bleeding
desmopressin + factor 8 transfusion
