Haematology Flashcards
Febrile non haemolytic transfusion reaction
Fever > 38
Raise in body temp 1-2 C during/after transfusion (up to 4 hrs)
Chills/rigours or asymptomatic
Other vitals normal
Treatment FNHTR
Tx : paracetamol & monitoring
Stop transfusion, give normal saline
Resume transfusion when symptoms and fever subside
Observe for 15-30 mins
Acute hemolytic transfusion reaction (AHTR)
Features (5)
Can be fatal Starts w/in minutes** of transfusion Fever + hypotension/shock Pain @ transfusion site \+/- DIC \+/- haemoglobinuria \+/- feeling of impending doom
Types of leukaemia
CLL CML
ALL AML
CML features (6)
- age
- examination findings
- labs/smear
- genetics
40-50 yrs old
Massive splenomegaly - does not always have it
“Crazy massive large spleen)
WBC > 100x10^9
Granulocytes*** @ all stages w/o blast cells!
Neutrophilia - myelocytes, basophils, eosinophils
Ph chromosome - Philadelphia chromosome
CLL features(4)
- age
- presentation
- labs/smear
- > 60 years old
- asymptomatic / anemia + recurrent infection , lymphadenopathy
Usually no splenomegaly - raised dysfunctional WBCS with B lymphocyte predominance
- smear = mature lymphocyte with smudge cells
ALL features
- age group
- presentation
- labs
- aspiration/biopsy findings
Children - up to 15 yrs Pancytopenia - hb - anemia, fatigue -wbc - recurrent infections ( wbc can be normal/hi/low) -plts - thrombocytopenia - bleeding
Bone morrow aspiration = numerous blasts
AML features
- age group
- blood film
- clinical features
- aspiration/biopsy
Adults - 20-30 yrs
Auer rods on blood film
Gingivitis , gum bleeding
Numerous blast calls * on biopsy
Think of lymphoma if
Painless cervical lymphadenopathy + B symptoms
+- splenomegaly
B = unintended weight loss, unexplained fever, drenching night sweats
TB vs Lymphoma
TB: Hx of travel - South Asia, sub Saharan Africa
Tender * firm LNs
Usually chronic productive cough +- bloody sputum
+- erythema nodosum
Lymphoma - painless lymphadenopathy
RFs for TB
Homeless
Drug abuse
Smoking
Low socio-economic class
Most common HIV related lymphoma
Dx test
Non Hodgkin lymphoma
Burkitt lymphoma
Diagnostic test - LN biopsy
DIC
Features
Sepsis + bleeding
- purpurin, petechia, ecchymosis , bleeding e.g. GIT,ENT
Sx - blood clots blocking blood vessels = chest pain SOB leg pain etc
Common causes DIC (5)
Sepsis surgery Major trauma Cancer Complications of pregnancy
Dx of DIC
Low platelets & fibrinogen
Raised PT PTT INR D-dimer
High bleeding time
Treatment DIC
Treat underlying condition
Platelet/FFP transfusion
Raised PTT + bleeding into muscles
Suspect
Haemophilia
Raised PTT & bleeding time + mucosal bleeding
Think ___
VWD
Raised PT PTT & bleeding time
+ bleeding at any site
Suspect
DIC
Isolated low platelets + bleeding +- hx of URTI
Idiopathic thrombocytopenic purpura
Polycythemia rubra Vera (4)
Primary polycythemia - malignancy
JAK2 mutation
RBC WBC platelets raised , HCT >55%
Hyperviscosity of the blood
Presentation of PRV (5)
Fatigue, lethargy Pruritic - esp after hot shower Splenomegaly Burning sensation in fingers toes Gout - due to increased cell turnover
Headache,hepatomegaly, low or normal erythropoietin
PRV vs 2ry polycythemia
PRV - low or normal erythropoietin , RBC WBC Plt raised
2ry - high erythropoietin , only hgb raised
Treatment PRV
1.Venesection (phlebotomy)
- reduces the elevated Hct + blood viscosity
2. Aspirin - low dose
75md OD for fear of thrombosis
3.Chemotherapy
= <40 - interferon ; >40 - hydroxyurea
Important cause of 2ry polycythemia
Other causes
Chronic hypoxia - stimulates kidneys to produce more erythropoietin - stimulates bone marrow to produces RBCs so they can carry more O2
e.g chronic smokers , COPD
Other : high altitude, cyanosis congenital heart disease
G6PD deficiency
G6PD enzyme def - decreased glutathione - RBCs vulnerable to oxidative damage - hemolysis
X-linked recessive = male
African/Mediterranean
Hx of neonatal jaundice
Jaundice , red/dark urine , back abdominal pain
G6PD triggers
- primaquine, fava beans , infection- severe hemolysis
DX
Treatment G6PD def
Avoid triggers
IV fluids
If severe hemolysis - blood transfusion
Definitive Dx - G6PD enzyme activity 6 weeks after hemolytic attack
G6PD = hemolytic anemia
Test used in cross-matching blood transfusion
Indirect Coomb test
= detects antibodies in serum (unbound)
Used in cross match , antenatal antibody screening (igG that can cross placenta and cause hemolysis)
Direct vs indirect Coombs test
Direct - detects antibodies or complement on SURFACE of RBC
- autoimmune, drug induced, hemolytic disease of newborn
Indirect - antibodies in SERUM (unbound, floating)
Can’t detect complement
Osmotic fragility test detects
Hereditary spherocytosis
Multiple myeloma
Main symptoms
Cancer of plasma cells
- plasma cell overgrowth +overproduction of non functioning Igs
Bone pain + hypercalcemia + anemia
- back + ribs
Renal failure*, recurrent infections
Investigations for multiple myeloma
- diagnostic
- blood film
- imaging
- labs
D - bone marrow biopsy = abdundant plasma cells
Serum protein electrophoresis - monoclonal immunoglobulin spike
Urgent protein “ - Bence jones protein
Film - Rouleaux formation
Xray - lytic lesion
Hypercalcemia + normal ALP + anemia + high ESR
Impaired renal fn - low GFR , high U&C - in 50%
Most common lab finding in Multiple myeloma
Anemia
ALL vs Aplastic anemia
Pancytopenia in both
Blast cells in ALL
AA - hypoplastic bone marrow
Hypercalcemia differentials
Bone mets
SCC of lung
MM - ALp normal
1ry hyperparathyroidism
ECG finding hypercalcemia
Short QT. Interval
Low hb low MCP low ferritin
High TIBC , High RDW
IDA
Features of IDA
Angular stomatitis - also seen in B12 def
Red sore tongue
Koilonychia
Most common cause of IDA
GI blood loss
Indications for blood transfusion
hb <7 - regardless of whether they are symptomatic
<8 + symptoms of anemia
<9+ known CVD
What deficiency can methotrexate cause?
Folate deficiency (macrocytic anemia)
Anaemia of chronic disease
Normocytic normochromic
However,
Can be micro cystic hypochromic in rheumatoid A, and chronic disease
HSP features
Non blanching purpura - buttocks, LL
Precipitated by URTI
All bloods - normal
Arthralgia, raised ESR IgA
Rarely - impaired renal function
Treatment HSP
Self limiting
Aspirin for arthralgia unless renal impaired
HSP- PAAN *= purpura arthralgia , abd pain , nephropathy (hematuria/proteinuria)
Dx of aplastic anemia
2/3 must be present
1. Hb <10
Plt s<50
Neutrophils<1.5
Irregular folded or ridges white patches on sides of tongue
What is this?
How do you treat?
Hairy leukoplakia
- supportive of HIV disease
EBV * invades was immense system in HIV - can’t be scraped off
Benign - no treatment needed
Target INR
Mostly - 2-3 (incl warfarin intake)
Mechanical valve replacement (metallic valve) = 3-4
ITP features
Children > adults
Usually preceded by infection in children
Isolated thrombocytopenia
Bleeding, epistaxis, petechia, menorrhagia , sometimes asymptomatic
Treatment ITP
Prednisolone
IV Ig
Life threatening hemorrhage (<20 platelets = emergency platelet transfusion
What is the normal
PT
PTT
Bleeding time
PT - 10-14 s - extrinsic pathway PTT - 35-45 s -instrinsic pathway Bleeding - 3-9 mins
Hemophilia A vs B
A: 90% of cases
Factor 8 deficiency
Tx - desmopressin , if major bleeding give recombinant factor 8
B: factor 9 deficiency , tx - give recombinant factor 9
(No role of desmopressin)
Christmas disease
Hemophilia B
Genetic inheritance of hemophilia
X- linked recessive
Affects males
Anemia + high bilirubin
Hemolysis
Markers of hemolysis
Appropriate investigation
Raised LDH, indirect bilirubin
+ reticulocytosis
Direct coomb test
Polychrome said + spherocytosis seen on peripheral blood smear
Dx?
Spherocytosis - seen in heriditary spherocytosis + autoimmune hemolytic anemia
Differentiate by direct Coombs test
HS - -ve ; AHH +ve
Osmotic fragility test +ve in HS
HELLP vs AFLP vs DIC
HELLP - low hb + elevated liver, low platelet
AFLP = ELLP ^ +low glucose +- high ammonia
DIC - high PT, PTT, bleeding time , low plts + fibrinogen
Von Willebrand disease mode of inheritance
Autosomal dominant - mostly
Cytochrome p450 inducers
Effect on warfarin
CRAP GPs
Decrease warfarin effect - decrease INR
Can’t use w/ COCPS
Carbamazepine, rifampin, chronic alcohol , phenytoin , phenobarbital , sulphonylureas
P450 inhibitors
Effect on warfarin
SICK FACES . COM
Increase effect of warfarin, raise INR
Sodium valproate, isoniazid, cimetidine, ketoconazole, fluconazole, acute alcohol, erythromycin (macrolides), sulfonamides, cipro, omeprazole, metronidazole
Management of high INR in patients of INR Major bleeding (intracranial bleed, GI bleed)
Stop warfarin
IV vitamin K (phytomenadione) 5mg
Prothrombin complex concentrate
^if not available - FFP
Management of high INR in patients of INR INR >8 INR 5-8 INR <5 \+- Minor bleeding (epistaxis, hematuria)
INR > 8
Stop warfarin + IV/oral vitamin K
5-8
Stop warfarin, check INR next day
Restart when INR <5
<5 (by higher than target )
Reduce/or omit a dose of warfarin
Measure INR in 2-3 days
B12 deficiency
Features
Treatment
Macrocytic anemia - oval macrocytic RBCs
Hypersegmented neutrophils
Peripheral paresthesia
Features of anemia
Tx - hydroxycobalmin
RFS for DVT
Smoking
Immobility
Long sitting
Major surgery
JAK mutation screen requested in
1ry polycythemia
What test requested in 2ry polycythemia
Erythropoietin
TTP
Features (5)
Haemolytic anemia Uraemia ( acute renal failure) Thrombocytopenia (low platelets) Neuro manifestations Fever
HUS
Features (3)
Haemolytic anemia
Uraemia (acute renal failure)
Thrombocytopenia
hematuria proteinuria raised U&Cs
*CHILDREN
Cause of HUS
Eating contaminated/ undercooked food
E.coli O157 - produces verotoxin- profuse diarrhoea > bloody diarrhoea- uremia
= hematuria proteinuria raised U&Cs
Cause of TTP
ADAMTS 13 factor deficiency or inhibition
Treatmentt of HUS
Supportive - IV fluids +- transfusion , dialysis if required
If severe - plasma exchange
NO ABX - more toxins released if e.coli dies
Most common cause of AKI in children
HUS
Ddx for opacity/ mass in superior mediastinumt
5 T’s Terrible lymphoma Thymoma Thoracic aortic aneurysm Thyroid goitre/neoplasm Teratoma
Troisier’s sign
Virchows node
- left supraclavicular mass indicative of gastric carcinoma
Right supraclavicular mass seen in
Oesophageal ca
Lung ca
Hodgkin’s lymphoma
Pancoast tumour
Tumour @ apex of lung
Spreads to nearby tissue - ribs , vertebrae
Most tumours - non small cell lung ca
Pernicious anemia
Autoimmune gastric atrophy - loss of intrinsic factors - impaired B12 absorption = B12 deficiency
High MCV (macrocytic) + associated autoimmune disease = hypothyroidism, vitiligo, T1DM
Treatment of pernicious anemia
IM hydroxycobalmin
Treatment of megaloblastic anemia - with B12 + folic acid are both deficient
IM B12 (hydroxycobalmin) Then give oral folic acid once B12 normal
B before F
Tumour lysis syndrome
Features
UK Pc Hyper: Uricemia Kalemia Phosphatemia
Hypocalcemia
Cause of tumour lysis syndrome
Occurs mainly in ALL & lymphoma (burkitts especially)after start of chemo
Chemo/radio/surgery - rapid lysis of tumour cell- excessive UKP released in blood
Complications & Management of tumour lysis syndrome
Hemato-ontological emergency = leads to retail failure, cardiac and neuro complications
M= IV fluid , rest is complicated
Single best investigation in tumour lysis syndrome
Serum urate
Urticaria acid + urate
Hereditary spherocytosis
Features
High MCHC
Hemolytic anemia - jaundice, reticulocytosis, high bilirubin
Gallstones
Spherocytes & reticulocytosis on blood film
Important complication of h.spherocytosis
Aplastic crises - due to PVB19 infection
^ it causes aplastic crises in SCA ,
Management of h.spherocytosis
Steroids
Folic acid
Splenectomy ‘
How does hemolysis cause gallstone
Bro if hemolysis causes increased bilirubin excretion and pigment gallstones
How long do you stop warfarin before surgery
3-5 days before
Heparin started instead = heparin bridging
Surgery when INR <1.5
Heparin bridging - why?
When do yo resume warfarin?
More easily and rapidly reversed than warfarin
On evening of operation when INR @ therapeutic levels (not always)
PVB19 aplastic crises vs splenic sequestration crises
In SCA
PVB19 - severely low Hb + LOW reticulocytes
SScrises- severely low Hb + HIGH reticulocytes
Crises in SCA
Vasopressin-occlusive
- most common
- mesenteric ischemia, avascular necrosis
Splenic sequestration - sudden enlargement of splee, pooling of RBCs
= low hb , high reticulocytes
If recurrent - splenectomy
Aplastic crises
PVB19 commonly
Low hb + low reticulocytes
Haemolytic - rare
Beta thalassemia major
Features
Autosomal recessive
Regular blood transfusion
Hepatosplenomegaly
Frontal bossing
Frequent transfusion > iron overload > endocrinopathy - DM
Treatment of beta thalassemia major
Life long transfusion - maintain hb >9.5
Iron chelation - deferoxamine (Desferal) = SC x2/week
Score used to determine need for anticoagulants in patients who have Atrial fib
CHAD2DS2-VASCULAR score CHF (LVEF <40%) = 1 HTN =1 Age >=75 = 2 DM = 1 Stroke/TIA or sys embolism = 2 Vascular disease = 1 Age 65-74 = 1 Female = 1
All patients Score >/= 2 —- warfarin/ DOAC
Men w/ score >= 1 — consider DOAC or warfarin
DOAC meds + advantage
Disadvantages
Apixaban
Rivaroxaban
Edoxaban
Dabigatran
No INR monitoring , faster onset of action (2-4 hrs), reduce risk of ICH
Dis - no antidote , requires strict compliance
Venous thrombosis above and below inguinal ligament - sites
Above - iliac vein thrombosis
Below - femoral vein thrombosis
Heinz bodies and Bute cells seen in
G6PD
Treatment of superficial thrombophlebitis
NSAIDs to relive pain/soreness
Well’s criteria
Active cancer/in last 6 mo = 1 Paralysis/immobilisation = 1 Bedridden >3 days / major surgery in last 12 weeks = 1 Localised tenderness in venous system = 1 Entire leg swollen = 1 Calf swelling >3cm of asymptomatic leg =1 Pitting oedema of symptomatic leg= 1 Non varicose veins = 1 Previous Dave = 1 Alt dx is at least as likely as DVT = -2 Score 2 or more = DVT like 1 or less - unlikely
Preferred time for US in DVT
In 4 hours
- however usually not possibl esp start treatment dose of DOAC and arrange US with in 24 hrs
B12 vs folic acid deficiency
B12 - peripheral paresthesia
Folate - does not eat vegetables but eats meat
No veggies or meat - B12
Microwaveable canned food w/ no veggies - folate
B12 - fish meat dairy / folate - vegetables
B12 store may last up to 4 years
Milk decreases absorption of ____
Iron
Hodgkins lymphome
Age group
Diagnostic feature
<25 or >55
Binucleated cells with prominent nucleoli - Reed Sternberg cells
Non Hodgkin’s lymphoma
What is seen on LN biopsy
Monomorphic small irregular b lymphocytes
