haem Flashcards
in IDA how long shld u take iron for
- for 3 months after levels have come up
when might you see ferritin rise with low iron
- inflammation as ferritin is acute phase protein as well
where is most dietary iron absorbed from
- duodenum
IDA in postmenopausal/ male patients.. next step?
- UGI/LGI ix
confirmed IDA, Next steps
- PMP/Men = UGI/LGI ix
- Coeliac screen
- h pylori screen
- urine test for blood = malignancy
lmwh clearance mode
- renal
half lfie lmwh
12hrs
peak lmwh
4hrs
antixa is measured after lmwh in which circumstances
- pregnancy
- renal failure
- obese
UFH clearance mode
- reticuloendothelial
UFH half life
1.5hrs
how to give UFH
= IV infusion/sc
how to monitor UFH
- APTT
UFH reversal agent
- protamine
what does appttr measure
- intrinsic pathway
instrinsic pathway involves which factors
- common
- 12 and 11
extrinsic pathway involves which factors
- common
- 7
what is Heparin induced thrombocytopenia
= minor plt drop at 5days
- no rx
what is rx for Heparin induced thrombocytopenia with thrombosis
- stop hep
= use danaparoid
SE heparin
- HIT
- HITT
- Osteoporosis; duration and dose related
warfarin SE
- Bleed
- skin necrosis due to protein c deficiency
- teratogen; coumarin embryopathy at 6- 12weeks with doses >5mg. also increases foetal loss due to haemorrhage
what does protein c do
- stops fv and fv8 = anticoag
CI to dabigatran
<30 crcl
active bleed
risk of bleed
prosthetic heart valve
CI to rivaroxaban
crcl<15
active bleed
risk of bleed
prosthetic heart valve
CI to apixaban
crcl<15
acitve bleed
risk of bleed
sideroblastic anaemia inheritance pattern
= x linked
causes for acquired sideroblastic anaemia
- myelodysplasia
- myeloproliferative disorders
- myeloid leukaemia
- alcohol misuse and toxicity
- lead poisoning, copper deficiency
- vit b6 deficiency esp in isoniazid treatment
b12 or folate replacement first
- b12
- always check b12 if folate low otherwise can cause damage
causes of acquired haemolytic anaemia
- AIHA
- Transfusion
- MAHA
- Systemic
b thalaessaemia inheritence pattern
- AR on chr 11, point mutation
- extra hbf made as no beta for normal hba
beta thalassemia types
minor = b1 - 1 affected gene - reduced synthesis
intermedia = b2 - 2 affected genes from parents which reduce synthesis
major - 2 inherited genes both with lost trait - so none made
why do you get haemolysis in beta thalaessaemia
- too many alpha chains that are free so clump up and form inclusions and destroy rbc membrane = haemolysis
when does b thalaseemia major cause sx
- 3-6 months of life as hbf made till then
what iron issue can beta thalassemia patients have
- haemachromatosis
sx beta thalassemia
- those of anaemia
- FTT
- Chipmunk faces
- hair on end.
target cells = what anaemia
beta thaemolysis
dx GS for beta thalaessemia
- electrophoresis. b minor >3.5% hba2
rx beta thalassemia major/intermedia
- regular transfusions
but can cause iron voerload so worsen haemachromatosis. iron chelating agents, deferoxamine - splenectomy
Heinz bodies and bite cells are indicative of what problem
- G5PD deficiency
G6PD inheritance pattern
- X linked
Pyruvate kinase deficiency inheritance pattern
- AT
warm AIHA involves which abs
IgG
what happens in paroxysmal nocturnal haemoglobinuria
- acquired disorder
- increases destruction of Haem cells by complement due to lack of GPI on their membranes.
sx paroxysmal nocturnal haemaglobinuria
- VTE
- haem anaemia
- pancytopenia
- dark urin in the morning
- aplastic canaemia
how to dx paroxysmal nocturnal haemoglobinuria
- flow cytometry of CD59/CD55 = gs
- hams test; acid induced haemolysis
rx paroxysmal nocturnal haemoglobinuria
- transfuse
- anticoag
- eculizamab - mab for C5
- STCRT
where is folate absorbed in the body
- jejunum
symptoms of folate deficiency
- ulcers
- growing hair
- anaemia sx
what one iron studies marker is raised in anaemia of chronic disease but normal in IDA
hepcidin
which coagulation factor rises in the acute phase of systemic disease
- FV8
ddx for left shift in leukocytes (more immature cells)
- infection
- malignancy
- haemolytic crisis
- MI
ddx for right shift in leukocytes
absence of youn or imamture wbcs
- b12 and folate deficiency,
- chronic uraemia
- liver dsiease
tumour lysis syndrome abnormal blood results
- hyperk
- hyperphosphataemia
- hyperuricaemia
- hypoca
tumour lysis syndrome rx
- agressive pre hydration
- allopurinol/rasburicase
- manage hyperk
- dont replace ca
hyperviscosity syndrome triad of sx
- mucosal bleed
- blurry vision
- neuro disturbance
hyperviscosity rx
- urgent plasmapheresis or leukopheresis
how does acute promyelocytic leukaemia present
- leucopenia and bruising; DIC
chr transloaction in acute promyelocytic leukaemia
- chr 15:17, PML RARA
how to treat acute promyelocytic leuakemia
- ATRA and correct coagulopathy
CRABBI sx of myeloma
- Calcium is high
- Renal failure
- Anaemia and pancytopenia
- Bone disease; osteolytic lesions
- bleeding
- infection
NICE guidance, who to suspect myeloma in
> 60 with back pain or bone pain or unexplained fractures
intitial ix for myeloma
- fbc
- u and e
- Calcium
- Esr
- plasma viscosity
then if positive = bence jones and electrophoresis
what group of ix is done for myeloma diagnosis. clue = BLIP
- Bence hones
- Light chain assay serum
- Immunoglobulins serum
- Protein electropheoresis
and biopsy BM = dx. rouleux formation on peripheral blood film. in bipsy - too many plasma cells
rx multiple myeloma
- chemo
- SCT
- VTE prophylaxis if hypervisocse/ certain chemo = LMWH/aspirin
- bonnes; bisphosphonates
- RDT for pain
- surgery
- cement augmentation into fractures or lesions foe stabiltiy and pain
diagnostic criteria for multiple myeloma
- Monoclonal plasma cells in th BM >10%
- Monoclonal protein in serum or urine
- end organ damage
criteria for MGUS
- <10 % PLASMA CELLS
- ABSENCE OF BONE LESIONS, ANAEMIA OR OTHER SX
- <30G/L SERUM MONOCLONAL PROTEINS
WHAT IS AMYLOIDOSIS
- extraprotein deposits. due to inability of proteases to appropriately cleave or abnormal cleavage.
what is AL amyloidosis = systemic
- Amyloidosis with light chains
what is AA amyloidosis = primary
- misfolded protein becomes amyloid a in serum
sx amyloidosis
- low albumin and proteinuria - oedema - hyperlipdiaemia as albumin normally inhibits lipid production - restrictive cardiomyopathy
Ix amyloidosis
- congored stain post biopsy = pink +ve
- under poalrised light = apple green
what is gauchers disease
- enzyme disorder = accumulation of lipid in bone marrow and organs
RF for Hodgkins lymphoma
- HIV
- EBV
- RA/ sarcoidosis
- FHX
sx for hodgkins lymphoma
- non tender rubbery LN. worse pain with alcohol + b sx.
- cough
- itchy
- recurrent infection
ix hodgkins
- VBG - LDH
- LN biopsy; reed sternberg
ann arbor staging hodkins lymphoma
Stage 1: Confined to one region of lymph nodes.
Stage 2: In more than one region but on the same side of the diaphragm (either above or below).
Stage 3: Affects lymph nodes both above and below the diaphragm.
Stage 4: Widespread involvement including non-lymphatic organs such as the lungs or liver.
rx hodgkins lymphoma
- chemo –> leukaemia and infertility
- RDT –> Cancer, hypoTH
burkitt lymphoma is associated with which RF
- EBV and HIV
Diffuse large B cell lymphoma sx
- painless rapidly enalrging mass
RF non hidgkins lymphoma
- HIV
- EBV
- Hpylori if MALY
- Hep b /c
- trichlorethylene
- fhx
hodgkins lymphoma age group
- bimodal
- <25
- > 50
what type of lymphoma would hyperpigmentaion indicate
- t cell
SE of RDT earyl
- fatigue
- skin reaction
- n and v
- mucositis
- Diarrhoea
- dysphagia
- cystitis
- BM supression
SE of RDT late
- organ involvement
4 subtypes of hodgkins lymphoma
- nodular sclerosing
- mixed cellularity
- lymphocyte deplted
- lymphocyte rich
which subtype of hodgkins lymphoma has the best prognosis and is more common in women
- modular sclerosing
testicular mass can be found in which type of lymphoma
- non hodgkins
what does burkitts lymphoma look like on biopsy
- starry sky appearance
complications of lymphomas
- BM infitration
- SVC obstriction
- mets
- SCC
2 types of burkitts lymphoma
- endemic; african = maxilla and mandible
- sporadic = abdo tumorus. common in HIV
what gene translocation is in burkitts lymphoma
- cmyc gene. t8:14
which b cell non hodgkins lymphoma is more likely to be completely cured - indolent (low grade) or aggressive (high grade)?
- aggressive
drugs used to treat aggressive high grade b cell non hidgkins lymphoma
- rituximab and chemo
What type of leukaemia do you get gum hypertrophy and parotid enlargement in
- AML
philadephia chr is in which leukaemia mostly
- CML
enlarged rubbery non tender lymph nodes are found in which leukaemia
- chronic lympocytic - CLL
gout occurs in which leukaemia
- CML
auer rods are in which type of leukaemia
- AML
smudge cells are in which type of leukaemia
- CLL
which leuakemia is related to myelodysplastic syndrome-
AML
rx CML with ph chr
- Imatinib
CLL is associated with which haemolytic anaemia
- Warm aiha
Warm AIHA what is the defect in rbc
- spherocytosis
In Cold AIHA , what is the associations and triggers
associated;
- raynauds, acrocyanosis
trigger
- ebc, mycoplasma pneumonia
in Paroxysmal nocturnal haemoglobinuria what antibody is seen
- donath lansteiner - biphasic
what is a typical exam q for paroxysmal nocturnal haemaglobinuria
- child goes out to play and comes into warm house and starts developing sx. because it is biphasic - ab binds to rbc at 37 degrees and lyses at 20 degrees. trigger = viral infection
pentad sx for TTP
- thrombocytopenia
- fever
- renal failure
- evidenc eof MAHA on blood film
- confusion
blood results in TTP
- low plt
- low hb
- high bili
- high LDH
- high creat
- +/-ve trops
genetic defect in ttp
= adams13 = cleaving protease for vwf which is involved in ttp clotting, has an ab igg
ttp rx
- high dose steroids
- palsma exchange
- rituximab
- blood
- folic acid
- when recovered; aspirin and lmwh
what hip problem are those with SCD prone too
- avascular necrosis; steroids, core decomrpess and replace
do we give abx prophylaxis in SCD
- Lifelong OR full adherance tille age 5 = pen/erythro
what preventative durg other than abx shld you give SCD pts
- folic acid
f/up programme for SCD
- EVERY 3/12 in first 2 yrs then 6/12 3-5 yrs then annual
when is hydroxucarbamide given to patient with SCD
- > 3 EPISODES WITH HOSPITAL admission in 12months
- pain interferes with QOL
- 2 episodes of chest syndrome in 12 month
extra haemolytic manifestations of thalassemia
- ostoeporosis
- hypoth
- hypopth
- dm
- HPA axis issues
- hypogonadism
- iron overload
which part of the coagulation path does warfarin target
- extrinsic pathway
function of protein c and s
- inactivate factor 5 and 8
Why is there a higher thrombosis risk in cocp
- acquired increase in resistance to activated protein c
- reduced protein s levels
- reduced antithrombin levels
ITP rx
- if sympto or plt <20 = pred
drugs that cause hamolytic anaemia
- methyldopa
- quinine
- NSAIDs
- interferon
HUS triad
- AKI
- thrombocytopenia
- microangiopathic haemolytic anaemia = MAHA
hereditary spherocytosis dx
- osmotic fragility is no longer diagnostic
- if FH , typical sx, and lab investigations - spherocytes on blood film, high MCHC, high reticulocytes; no other tests
- if need further tests; EMA binding and cryohaemolysis test
Hereditary spherocytosis rx
- folate replacce
- splenectomy
Paroxysmal nocturnal haemoglobinuria GS
- HAMS TEST; acid induced haemolysis
causes of absolute polycythaemia
primary = polycythaemia vera
secondary either hypoxia or inappropraite epo secretion
- hypoxia causes; high altitude, chronic lung disease, cyanotic congenital HD, heavy smoking
- epo - HCC, RCC
polycythaemia rubra vera symptoms
- headaches
- tinnitus
- dizzy
- visual changes
- facial plethora
- gout due to urate excess from b/down rbc
- itchy post hot bath
- erythromyalgia (also in essential thrombo)
- burning fingers and toes
- splenomegaly
- thrombosis both arterial and venous
PCV rx
- venesect
- if high risk e.g. >60 = hydroxycarbamide/urea
- alpha interferon in child bearing age women
- aspirin lifelong 75
essential thrpmbocythaemi rx
- aspirin
- hydroxycarbamide in high risk
teardrop cells are in which disease
- myelofibrosis
what kind of biopsy is done in Myeloproliferative disorders
- trephine ; as cant aspirate due to dry tap
define essential thrombocythaemia
- > 2 months of plt>450
livedo reticularis occurs in which myeloprolif disorder
- Essential thrombocythaemia
myelofibrosis sx
- constitutional sx
- splenomegaly
what is the scoring system used for myelodysplastic syndromes
- cytopenias
- cytogenetics
- blast %
= RIPSS - prognostic
ITP epidemiology
- woman; chronic
- kids - acute, 2 weeks post infection. goes away
