haematology Flashcards by Maryam Garkuwa

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.

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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

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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

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sick cell anemia is

AIHA tx and siagnosis

NORMOCYTIC

immunosuppresnat till yu get a negatuc coombs test

coombs test and spheroctosis !

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anemia of chronic disease

microcytic (lecture)or normochronic(google)

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haemolytic anemias are what type

pearson syndrome

normocyitc?

siderblastic anemia
mitcohondiral problem
macrocytic
liver prolems
lactic acidosis

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which 2 conditions have anemia but increased RBC

SICKELE CELL AND THALLASEMIA

fanconi, diamond, pearson

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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 !

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how to prove thalssemia

tx glucos 6 phosphate

electrophoresis of the chains

rarely need transfuison just stop the ttrigeer supportive tx xygen etc

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theory for hemmorrhaigc disease of newborn

IV vit k 1mg/kg bw for 3 days

2. FFP 10-15ml preterm

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TTP VS ITP

check tables

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TTP

platelet count not a slow as iTP
but clots form and platelets low
you have haemolytic anemia (shsitocytes)

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why would you do a urine electrophoresis

prove whether protein is tubular or glomerular

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Type of child is more likely to present with jaundice in a uni tract infection

<3months

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uti main symptoms

FEVER ESPCIALLI IN YOUNG KIDS
FAILURE TO THRIVE
JAUNDIC <3

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>3 YEARS

<3 urgent microscopy +culture

>3 leukocyte esterase +nitrate+dipstick

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criteria for pyelonephritis

> 10*3

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criteria for lower utilities

<10*3

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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

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decreased reticulocyte count

bone marrow failure

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normal reticulocyte count

NORMAL PLT COUNT

SEVERE THROMBOCYTPOENIA

0.5-2.5%

> 150

<20 -spontaneous bleeding

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lead poisoning

microcytic anemia and basophils stippling
gum grey line
constipatin and vomititng

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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

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normal blood volume of term baby

where is iron absorbed

80 ml

duodenum

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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

1. + fH 2. 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