Haem Flashcards by Erin McHugh

sdefine ideroblastic anaemia

red cells fail to completely form haem

How well did you know this?

Not at all

Perfectly

siderblastic anaemia causes

congenital: x-linked, rec or dom
acquired: drug/toxin related (alcoholism, lead, isoniazid), myelodysplastic syndromes, RA, carcinomas

How well did you know this?

Not at all

Perfectly

sideoblastic anaemia blood results

increased ferritin
increased iron
increased transferrin

How well did you know this?

Not at all

Perfectly

sideroblastic anaemia treatment

treat underlying

pyridoxine may help

How well did you know this?

Not at all

Perfectly

thalassaemia inheritance

autosomal recessive

How well did you know this?

Not at all

Perfectly

thalassamia definniton

abnormal haemaglobin production

How well did you know this?

Not at all

Perfectly

a thalassaemia presentation

jaundice
fatigue
facial bone deformities

How well did you know this?

Not at all

Perfectly

b thalassaemia presentation

severe symptomatic anaemia at 3-6 months age
frontal bossing
maxillary overgrowth
hepatosplenomegaly

How well did you know this?

Not at all

Perfectly

a thalassaemia management

blood transfusions
stem cell transplant
splenectomy

How well did you know this?

Not at all

Perfectly

b thalassamia management

regular blood transfusions

iron celating agents to reduce risk of iron overload

How well did you know this?

Not at all

Perfectly

anaemia of chronic disease blood results

+/- feritin
decreased iron
decreased TIBC
decreased transferrin

How well did you know this?

Not at all

Perfectly

aplastic anaemia blood results

anaemia
low plts
low neutrophils

How well did you know this?

Not at all

Perfectly

primary aplastic anaemia causes

fanconis anaemia

idiopathic

How well did you know this?

Not at all

Perfectly

secondary aplastic anaemia causes

drugs (NSAIDs, chloramphenicol), infection, PNH

How well did you know this?

Not at all

Perfectly

aplastic anaemia features

anaemia
infeciton
easy bruising

How well did you know this?

Not at all

Perfectly

aplastic anaemia management

any susp of infection - broad spec anti-bs

severe: stem cell transplant (<40)
severe: immunosupression (>40)

How well did you know this?

Not at all

Perfectly

intravascular haemolytic anaemia causes

G6PD
autoimmine
acute transf reaction
mechanical injury

How well did you know this?

Not at all

Perfectly

extravascular haemolytic anaemia causes

sickle cell

spherocytosis

How well did you know this?

Not at all

Perfectly

G6PD inheritance

x linked

How well did you know this?

Not at all

Perfectly

G6PD triggers

fava beans
illness
henna

How well did you know this?

Not at all

Perfectly

G6PD characteristic features

bite and blister cells
heinz bodies
dark urine

How well did you know this?

Not at all

Perfectly

G6PD causes

anti-malarials (primaquine)
ciprafloxacin
sulph-group drugs

How well did you know this?

Not at all

Perfectly

autoimmune warm type mediated by

IgG

How well did you know this?

Not at all

Perfectly

warm type autoimmune HA management

steroids +/- rtiux

How well did you know this?

Not at all

Perfectly

cold type autoimmune HA mediated

IgM

hereditary spherocytosis inheritance

autosomal dominant

hereditary spherocytosis presentaiton

neonatal jaundice gallstones splenomlegaly

hereditary spherocytosis diagnosis

EMA binding + cryohaemolysis test | electrophoresis analysis

hereditary spherocytosis management

acute: supportive, transfuse if necessary | long term: folate, splenectomy (if less than 5yo = curative)

sickle cell disease definition

genetic condition where normal haemoglobin has a tendency to form abnormal haemoglobin molecules upon deoxygenisation

sickle cell disease inheritance

autosomal recessive | glutamic acid replaced by valine on 6h position of B chain

sickle cell blood film

sickle cells target cells howel joly (hyposlen)

sickle cell testing

haemoglobin electrophoresis +/- genetic testing

long term sickle cell treatment

hydroxyurea vaccines folic acid

screening of primary haemostatic failure

platelet count

extrinsic pathway factors

TF/VIIa

extrinsic pathway tets

intrinsic pathway factors

VIII/IXa

intrinsic pathway test

APTT

VWD inheritance

autosomal dominant

VWD stablises clotting factor

VIII

VWD clotting result

prolonged bleeding time | may be prolonged APTT

VWD management

TXA desmopressin factor VIII conc

haemophilia inheritance

X-linked

haemophilia A def

VIII

haemophilia B def

haemophilia blood results

increased APTT

haemophilia investigations

factor VIII and IX assay

haemophilia treatment

mild - desmopressin | severe - factor VIII/IX infusions

DIC blood results

increased PT/APTT increased FDP (d-dimers) decreased platelets decreased fibrinogen

DIC treatment

replacement therapy (platelet, plasma, fibrinogen transfusions)

polythemia vera mutation

JAK2

pruritis following hot baths

polythaemia vera

arterial thrombosis treatment

aspirin | clopidogrel

venous thrombosis treatment

heparin warfarin DOACS

most common acute leukaemia in adults

AML

most common childhood cancer

ALL

definitive diagnosis of leukaemias

immunophenotyping

characteristic AML biopsy findings

auer rods

CML classic feautres

massive splenomegaly weight loss tiredness fever

most common leukeamia in adults

CLL

CLL characteristics

smudge cells

hodgkins lymphoma characteristics

reed-sternberg cells orderly lymph node spread alcohol induced pain

non-hodgkins lymphoma characteristics

B symptoms more common | painless, multiple sites

myeloma pathophysio

plasma cell proliferation - abnormal clonal proliferation of post germinal B cells

myeloma investigations

bence johns urine | peripheral blood film: rouleaux formation

myeloma treatment

stem cell transplant | MPT if not suitable

philidelphia chromosome assoc with what malignancy

chronic myeloid leukemia