Haematology Flashcards
Indications for CMV negative and irradiated blood: which of the following? granulocyte tranfusions intra-uterine tranfusions neonates up to 28 days delivery pregnancy (elective transfusions during pregnancy only, not labour/delivery) bone marrow/stem cells transplants immunocompromised (chemo, congenital) patients with previous Hodgkin lymphoma HIV
granulocyte tranfusions: both
intra-uterine tranfusions: both
neonates up to 28 days delivery: both
pregnancy (elective transfusions during pregnancy only, not labour/delivery): CMV neg
bone marrow/stem cells transplants: irradiated
immunocompromised (chemo, congenital): irradiated
patients with previous Hodgkin lymphoma: irradiated
HIV: neither
If a DVT is likely (2 points on Wells Score), you should…
hint: US scan +ve...? US scan -ve...? 4 hours not possible...? scan is -ve but DD +ve...?
carry out proximal leg vein US scan within 4 hours
○ if the result is positive then a diagnosis of DVT is made and anticoagulant treatment (DOACs) should start
○ if the result is negative a D-dimer test should be arranged. A negative scan and negative D-dimer makes the diagnosis unlikely and alternative diagnoses should be considered
if a proximal leg vein ultrasound scan CANNOT be carried out within 4 hours a D-dimer test should be performed and interim therapeutic anticoagulation (DOACs e.g. apixaban/rivaroxaban) given whilst waiting
if the scan is negative but D-dimer is positive: stop therapeutic coagulation, offer repeat proximal leg vein US scan a week later.
If a DVT is unlikely ( point or less on Wells Score), you should…
perform D-dimer test (within 4 hours)
If not possible, give interim therapeutic anticoagulation until result is available.
If -ve, DVT is unlikely. Consider other causes.
If +ve, then carry out proximal leg vein US scan within 4 hours. If not possible, give interim therapeutic anticoagulation until result is available.
Name a few of the 10 things on the two-level DVT Well’s Score. They all have 1 point each, remember!
active cancer
paralysis/paresis/recent immbolisation of extremities
bedridden for 3 days +/ major surgery within 12 weeks
localised tenderness in region of deep venous system
entire leg swollen
calf swelling at least 3 cm
larger than asymptomatic side
pitting oedema (only on symptomatic leg)
collateral superficial veins (non-varicose)
previous DVT
Management after DVT diagnosis (1st and 2nd line)
1) apixaban or rivaroxaban (DOACs)
2)LMWH followed by dabigatran or edoxaban
OR
LMWH followed by a vit K antagonist (warfarin)
- if there is severe renal impairment, then LMWH, unfractionated heparin or LMWH followed by warfarin.
How long should anticoagulation be for patients after VTE?
Minimum of 3 months (if it was provoked*).
If unprovoked, then typically for 6 months.
If the patient has active cancer, then between 3-6 months.
*provoked= due to obvious precipitating event e.g. immobilisation after surgery.
What is the pathophysiology of G6PD deficiency?
↓ G6PD → ↓ NADPH → ↓ glutathione → increased red cell susceptibility to oxidative stress
Name some of the features of G6PD deficiency
male (X-linked recessive) african +mediterranean descent intravascular haemolysis certain drugs can precipitate haemolysis Heinz bodies (and bite cells) on blood film inherited haemolytic anaemia* neonatal jaundice* gallstones* infection can precipitate haemolysis*
*features shared with Hereditary Spherocytosis
Name some features of hereditary spherocytosis
Male + female (autosomal dominant) northern european descent extravascular haemolysis spherocytes on blood film (round, lack of central pallor) inherited haemolytic anaemia* neonatal jaundice* gallstones* infection can precipitate haemolysis*(although tend to have more chronic symptoms)
*features shared with G6PD deficiency
Drugs that can precipitate haemolysis
antimalarials (primquine)
ciprofloxacin
sulph-group drugs: sulphonamides (antibiotics), sulphasalazine (DMARD), sulfonylureas (T2DM drugs, work by increasing release of insulin from the pancreas)
3 types of Non-Hodgkin’s lymphoma
The most rapidly progressing +aggressive tumour, associated with Epstein-Barr virus, malaria and HIV. Microscopy gives “starry sky” appearance: Burkitt’s Lymphoma
Affects the Mucosa-Associated Lymphoid Tissue, usually around the stomach. It is associated with H. pylori infection. Less aggressive: MALT lymphoma
Often presents as a rapidly growing painless mass in patients over 65 years. Aggressive: Diffuse B-cell lymphoma
Complications of tumour lysis syndrome (rapid response following chemo)
What can be given before chemo to reduce risk of developing tumour lysis syndrome? (works by catalysing uric acid->allantoin which allows for better renal excretion)
- Hyperkalaemia
- Hyperphosphataemia
- Hypocalcaemia (due to the high phosphate binding with the all the calcium).
- Hyperuricaemia
- Acute renal failure
Rasburicase
Types of Hodgkin’s lymphoma
Nodular sclerosing: most common (~70%), good prognosis, more common in women, associated with lacunar cells
Mixed cellularity: (~20%), good prognosis, associated with lots of Reed-Sternberg cells
Lymphocyte predominant (~5%), best prognosis!!
Lymphocyte depleted (rare), worst prognosis :(
Name the B symptoms (systemic symptoms of lymphoma)
note: B symptoms imply poor prognosis
Fever
Weight loss (>10% in last 6 months)
Night sweats
Other symptoms of Hodgkin’s lymphoma/ Non-Hodgkin’s lymphoma (same presentation, can only be differentiated when biopsied)
Lymphadenopathy: the enlarged lymph node or nodes might be in the neck, axilla (armpit) or inguinal (groin) region. They are characteristically non-tender and feel “rubbery”. Some patients will experience pain in the lymph nodes when they drink alcohol. Fatigue Itching Cough Shortness of breath Abdominal pain Recurrent infections
Risk factors for Non-Hodgkin’s lymphoma
HIV Epstein-Barr Virus H. pylori (MALT lymphoma) Hepatitis B or C infection Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes Family history
Types of Hodgkin’s lymphoma (in order of incidence)
Bonus: most common age at incidence
nodular sclerosing (70%)-good prognosis. Associated with lacunar cells. More common in women.
mixed cellularity(20%)- good prognosis. Assoc with lots of Reed-Sternberg cells
lymphocyte predominant(5%)- best prognosis
lymphocyte depleted(rare)- worst prognosis
BONUS: bimodal age distributions (20y/o’s and 60s)
1 in 5 lymphomas are Hodgkin’s lymphoma.
Findings in Hodgkin’s lymphoma
proliferation of lymphocytes
raised LDH (lactate dehydrogenase) is a common, but nonspecific finding
lymph node biopsy finds Reed-Sternberg cells- diagnostic!
CT, MRI and PET can be used for staging.
Ann Arbour staging is used for what?
What are the stages?
Both Hodgkins and Non-Hodgkins lymphomas.
The system puts importance on whether the affected nodes are above or below the diaphragm.
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.
Features of polycythaemia vera
myeloproflierative disorder caused by clonal proliferation of marrow stem cells leading to increased red cell volume, often accompanied by overproduction of neutrophils and platelets.
Splenomegaly (abdominal pain)
Portal hypertension (ascites, varices and abdominal pain)
Low platelets (bleeding and petechiae)
Thrombosis is common(hyperviscosity)
Raised red blood cells (thrombosis and red face)
Low white blood cells (infections)
5-15% of patients progress to myelofibrosis/acute myeloid leukaemia
peak incidence= 60 years old
Management of polycythaemia vera
- Aspirin (reduces risk of clots)
- Venesection(1st-line to keep haemoglobin in normal range)
- Chemo (hydroxyurea (slight increased risk of secondary leukaemia), phosphorus-32 therapy)
Features of (multiple)myeloma
• bone disease
○ bone pain
○ osteoporosis + pathological fractures (typically vertebral)
○ osteolytic lesions
• lethargy
• infection
• hypercalcaemia
○ primary factor: due primarily to increased osteoclastic bone resorption caused by local cytokines (e.g. IL-1, tumour necrosis factor) released by the myeloma cells
○ much less common contributing factors: impaired renal function, increased renal tubular calcium reabsorption and elevated PTH-rP levels
• renal failure
• other features: amyloidosis e.g. Macroglossia, carpal tunnel syndrome; neuropathy; hyperviscosity
Investigations for (multiple) myeloma
- Monoclonal proteins (usually IgG or IgA) in the serum and urine (Bence Jones proteins)
- Increased plasma cells in the bone marrow
- It is recommended to do whole body MRI to look for bone lesions
- X-rays: raindrop skull. Differential: pepperpot skull (primary hyperparathyroidism)
e.g. bloods can show: Low vit D (hypercalcaemia) Low Hb (bone disease) Low platelets (bone disease) High urea (kidney damage) High creatinine (kidney damage)
