haem Flashcards
Anaemia added tests
- inflam makers
- RF, anti CCP
- thal screen (protein electrophoresus)
- MM screen: immunofixation electrophoresis + serum free light chains.
- DAT/haptoglobin/reticulocyte count/K+/LDH
thrombocytopenia cause
- Sx: bleeding. menorrhagia. melena.
Ix:
- BLASTS on peripheral field (haematological malignancy) –> check for lymphadenopathy/hepatosplenomegaly + refer urgently to haem
- haemolysis found: HUS consider (TTP)
- abnormal coag: DIC
- dysplastic features on peripheral filme: laeukaemia, myelodysplastic disorder
always consider
- ITP
- HIT
- drug induced
- HIV
- post transfusion
- connective tiss disease
- thyriod disease
- malignancy, bone marrow failure.
- hereditary
- b12/folate dec.
if asymptoamtic, and no red flags / CLD ause obvious: and plate >100= watch/wait.
50-100 = non urgent referral
< 50 = refer.
Multiple myeloma
/ Monoclonal gammopathy of undetermined significance
MM = usually presents as its complications
- hypercalcaemia, renal failure, anaemia, bony lesions.
- always think of MM w anaemia but normal nutrients and no haemoylsis/loss
+ hypercalcaemia, PTH low and vit D normal.
+ no clear cause for AKI
+ bony pain w no evidence of lesions on imaging.
> 70 common MGUS.
Ix:
FBE, blood film, EUC/LFT/CMP, LDH, urate
–> Total protein (up with MM)
–> serum electrophoresis + immunofixation + free light chain analysis.
+ 24 hour urine protein electrophoresis studies (looking for bence jones protein)
then XR for lytic lesion assessment.
Dx: presence of monoclonal protein in serum/urine.
Mx: refer
MGUS –> watchful waiting, monitoring serial paraprotein and end organ damage
Leukaemias (wcc > 30)
Sx: gingival hypertrophy (AML), bone pain, easy bruising/bleeding, aneamia, susceptible to infection.
Si: pallor, petechiae, gum hypertrophy (AML), infection. hepatospleno megaly, lymphoadenopathy. bony tenderness especially sternal.
CML: very large slpenomegaly + see philadelphia chromosome. + B symptoms.
CLL : see more lymphadenopathy.
Ix: normochromic anaemia, pancytopenia w blast cells on peripheral film. platelts usually low
> > urgent refer, BM biopsy.
if adult, rapid progression to death. prognosis 20-30%. kids = 80%.
hodgkins/non hodgkins lymphoma
HL = constitutional Sx + pruritis
reed sternerg cells = pathognemonic.
alcohol induced pain in any enlarged LN.
NHL = usually more so lymphadenopathy. painless. pruritis uncommon. + constitutional sx
Ix> node biopsy w histological confirmation.
pathological LN
present for > 2 weeks post resolution/treatment of suspected cause.
> 1-3cm
rubbery, hard, fixed to surrounding structures
painless
progressive
splenectomy prophylaxis
malaria risk PCV 5 yearly Hib if not immunized int he past ACWY + men B every 2-5 years. influenza. penicillin prophylaxis: daily. any infection: hospital.
thrombocytosis
2 types (>400)
1) essential thrombocythaemia - myeloprolif disorder w increased platelets. sometimes PC: thrombosis/bleeding or asymptomatic
- - can also be due to polycythaemia vera/CML/myelodysplastic syndrome
2) secondary
due to reactive, infection, neoplasm, anaemia, tissue damaeg, exercise, reaction to meds.
OE: look for signs: inflam/malignancy/VTE. splenomegaly.
Ix: ferritin, CRP, HCT (if elevated PCV)
Mx:
if no Sx + not severe = repeat in 2-3 weeks (<600-1000)
- if > 600 + normal CRP /ferritin (excluded infection + iron def so essential thrombocytopenia) then start low dose aspirin + do JAK 2 mutation screen w haem review
G6PD def
X lnked
common in places malaria is common (protective historically)
mostly asymptomatic, but susceptible to haemlytic anaemia.
often cause prolonged neonatal jaundice . or when there is an infection: haemolysis.
or SFX from specific drug (nitro/antimalarial).
Rx: specialist. avoid broad beans!
myelodysplasia
group of disorders
manifest at neutorpenia/thrombocytopenia
can be secondary to Ctx/Rtx or primary.
often get progressive intractable neturopenia, thrombocytopenia or both –> progressive to fatal/terminating infection or haemorrhage, or acute leukamiae.
sometimes EPO/GCSF/infusions –> hame involvement
neutropenia
lots of causes
inc. myelodysplasia/leukaemias, hodgkin lymphoma/non hodkin lymphoma, CLD, tyhpoid, hiv, hepatitis , ebv etc.
investigate for cause
inc. w blood film for dysplastic features.
if mild (0.5-1) then repeat 4-6 weeks.
if persistents, then refer/cont to observe 3-6 months
if febrile: febrile neutorpenia (particularly if < 0.5-1)
polycythemia vera
primary
elevated Hb + HCt on > 2 occasions, not due to artefact.
JAK 2 mutation on peripheral blood film.
- if negative then v unlikely primary + go looking for secondary.
- also think to do BCR/ABL / phili chromo –> CML if pos.
+ EPO (high in secondary)
refer haem.
particularly if jak 2 neg and no clear secondary cause.
Rx: haem, consider aspirin, venesection w haem for HCt reduction
polycythemia (secondary)
cause:
- relative: dehydration, diuretics.
- chronic: chronic hypoxia (smoking, lung disease, OSA, obesity hypoventilation syndrome)
- cirrhosis
- CHD
- sometimes EPO secreting tumours: phaeochromo/adrenal adenomas
Jak 2 negative – in a non smoker, w normal sleep study = refer Haem
Rx: haem, consider aspirin, venesection w haem for HCt reduction
immunodef work up
normal for a child < 1 to have 10 URTI / year! but if more than that/other Sx then need to investigate
- severe infection from low pathogenic cause, or > 1 serious life threatening infections/unusual sites
OE: FTT warts eczema hepatosplenomegaly absence of tonsils s/t
Ix:
FBE for lymphocytes/neutrophils/thrombocytopenia
serum immunoglobulin quant (IgM/IgG.IgA – IgG most important)
consider immunophenotyping of lymphocytes w flow cytometry –> more haem.
