Haematology Flashcards
Sx of anaemia?
fatigue, dyspnoea, faintness
Severe anaemia bloods?
Signs?
< 8g/dL
Tachycardia, cardiac enlargement, increased CO
Transports iron?
Transferrin
Stores iron?
Ferritin, haemosiderin
Pres of iron-def anaemia?
Signs?
Fatigues, faintness, dyspnoa
+/- :
Pallor
Nail changes [koilonychia (spoon), brittle]
Hair loss
Mouth [angular stomatitis, atrophic glossitis]
Cause of iron-def ?
Inadequate intake (h.pylori) Poor absorption (coeliac) Excess loss (GI bleed) Excess iron requirement
Ix in iron-def anaemia?
Hb <13g/dL (men), <12g/dL (women)
MCV = microcytic
Blood smear
Iron studies
Coeliac autoantibodies
Endoscopy
Why may ferritin be high in iron-def?
Acute phase protein so increases in inflam
DDx for iron-def anaemia?
Chronic disease
Sideroblastic anaemia
Mx of iron-def?
Ferrous sulphate
+/- transfusion
SE of ferrous sulphate
Constipation
Black stools
Vomit
When would you suspect sideroblastic anaemia
Microcytic hypochromic anaemia
NOT responding to iron
Cause of sideroblastic anaemia?
Ineffective erythropoesis [cant incorporate iron into Hb]
Congenital - X-linked
Acquired - MDS, myeloma
Ix in sideroblastic anaemia
Hb
MCV
Iron studies
MARROW ASPIRATE
Mx of sideroblastic
Iron chelation
Desferrioxamine
Avoid alcohol / Vit C
B thalassmia?
Inherited microcytic anaemia
Mutation to beta-globin gene
Ineffective erythropeiss
Sx of b thalassemia
Bony changes - skull bossing
Hepatosplenomegaly
ix in b thalassemia
FBC Blood smear Hb analysis LFT Skull xray Abdo USS
Mx of b thalassemia
Genetic counselling
Transfusion
Iron chelation
Splenectomy
BMT
comps of thalassemia
Thrombotic
Iron overload
Transfusion reactions
Causes of haemolysis
Liver macrophages
Spleen
Red cell destruction by immune system
Hereditary causes of haemolytic anaemia?
G6PDD
Hereditary spherocytosis
SC, thalassemia
Test for immune mediated haemolytic anaemia?
Direct antiglobulin +ve (Coombs)
Causes of non-immune mediated haemolytic anaemia?
Infection (malaria) , trauma
DIC, TTP, HUS, HELLP
Paroxysmal nocturnal haemoglobinuria
Dx of paroxysmal nocturnal haemoglobinuria?
Urinary haemosiderin
Ix in haemolytic anaemia?
FBC MCV is increased! (reticulocytes) Unconj bilirubin Urinalysis Blood film Coombs test
Mx of haemolytic anaemia?
Folic acid supplement
Pres of G6PDD?
Prolonged neonatal jaundice
Mx of G6PDD?
Fluids
Folic acid
Transfusion
Anaemia of chronic disease?
Decreased RBC production and survival
Normocytic normochromic
Low reticulocytes, low iron, elevated ferritin
Pres of ickle cell disease?
Cresentic shaped Hb –> disrupt blood flow and break –> painful crisis, organ damage, increased infections
Triggers of sickle cell crisis
CHIDS Cold Hypoxia Infection Dehydration Stress
Areas affected by sickle cell crisis?
Chest - pain, fever, dyspnoea, tachypnoea
Bone infarction - avasc necrosis of femoral head
Acute abdo - mesenteric ischaemia
Dx of sickle cell?
Newborn screening
Mx of sickle cell crisis
Corss match Analgesia O2 IV fluids Abx \+/- blood transfusion
Cause of megaloblastic anaemia?
B12/folate deficiency
Defective DNA synthesis –> leukopenia and thrombocytopenia
Dietary sources of folate?
Green veg
Nuts
Liver
Causes of folate deficiency?
Poor diet
Increased demand (pregnancy, renal disease)
Malabsorp (coeliac)
Drugs, alcohol, MTX
Signs of folate def
Headache
Anaemia
Wt loss
Glossitis, dermatitis
Mx of folate def
Folic acid oral
Pres of B12 def?
Macrocytic anaemia with peripheral neuropathy and neuropsych complaints
Dietary sources of V12
Meat
Fish
Dairy
Which cell is needed for b12 absorption?
Parietal cells produce Intrinsic Factor
Mouth signs of b12 deficiency
Glossitis (painful!),
Angular chelitis
Neuro sx of b12 def
Subacute degen of spinal cord + peripheral neuorpath + dementia + ataxia + paraesthesia
Mx of b12 def
IM hydroxycobalamin
Oral folic acid
+/- blood transfusion
Pernicious anaemia?
Autoimmune atrophic gastritis + autoantibodies to IF
Leukaemia?
Excess of abnormal white cells in peripheral blood
Can be myeloid of lymphoid
Pres of acute leukaemia?
Bone pain, fever, lethargy, night sweats, wt loss
Anaemia, thrombocytopenia, neutropenia
Headache, lymphadenopathy, hepatosplenomegaly
Dx of AML
Tends to be >75yo
Bone marrow blasts > 20%
Ix in AML
FBC
Blood film - blasts + auer rods
Bone marrow biopsy - blasts > 20%
Coag
Tumour lysis syndrome?
Electrolyte + metabolic disturbance due to breakdown of large number cancer cells (common in leukaemia)
Hyperuricaemia, hyperphosphataemia, hyperkalaemia, hypocalcaemia, +/- renal impairment)
Mx of tumour lysis syndrome?
IV fluids
Allopurinol in chemo reigime
+/- Haemodialysis
Chromosomal assos with ALL
Philadelphia - t(9,22)
Pres of ALL
Most common tumour of childhood <5yo. / > 45yo
Assos with Down’s syndrome
Bruising, pancytopaenia
Ix of ALL
FBC Blood film - blasts Coag Bone marrow biopsy CXR
seen on CXR of ALL
mediastinal widening
Comps of ALL
Tumour lysis syndrome
Neutropenic sepsis
Pancytopenia
Chemo SEs
Mx of ALL
Hydration, allopurinol, prophylactic abx/anti-virals/antifungals
Transfusions
Chemo + imatinib
Mx of CML
Imatinib
Chemo
Stem cell transplant
What happens in CLL?
B lymphocytes avoid apoptosis –> invade liver, spleen and bone marrow
> 55yo
Sx of CLL?
> 55yo
Haemolytic anaemia
Can transfrom into lymphoma [Infiltration, B sx] = Richters transformation
Ix in CLL?
FBC
Blood film - smudge cells
CT
Mx of CLL
Chemo
+/- stem cell transplant
Lymphoma vs leukaemia?
Lymphoma = solid Leukaemia = circulating
“lemon vs lemonade”
Most common type of NHL?
Diffuse B-Cell lymphoma
can produce Ig
Pres of NHL?
Lymphadenopathy, dry cough, bone marrow, spleonmegaly
B sx
Ix in NHL?
FBC
LN biopsy
CT/MRI
HL from what cells?
Mature B cells
Pres of HL?
Cervical/supraclav lymphadenopathy (painless)
B sx in 30%
Which cells seen in HL?
Reed-Sternberg
look like an owl
Aetiology of HL?
immunodef
EBV
AI - SLE
Mediastinal adenopathy is common in HL –> how would this present?
Dry cough, dyspnoea, chest pain
SVCO
Multiple myeloma?
Clonal proliferation of plasma cells in bone marrow –> overprod of Ig
Dx of MM?
Serum + urine protein electrophoresis
Pres of MM?
Bone pain
Anaemia
Fatigue, infections, hypercalcamiea, RENAL IMPAIRMENT
Features of MM?
OLD CRAB Old age Calcium raised Renal insufficiency Anaemia Bone disease
What causes renal impairment in MM?
Deposition of Tamm-Horsfall protein in LoH
Mx of MM?
Bisphosphonates
Blood transfusion/EPO
Hydration
Polycythaemia?
Increased proportion of Hb in blood
Pres of PRV?
Hyperviscosity
Pruritis
Ix in PRV?
FBC
JAK2 mutation
Mx of PRV?
Hydroxycarbamide (decreases bone marrow production)
+ aspirin
Manage CV RFs
What regulates platelet production?
Thrombopoeitin (from the liver)
When does ITP occur?
Children post viral illness
Ix in ITP
FBC
LFTs
peripheral smear
BM biopsy
Mx of ITP
Acute –> IVIg, pred, pt transfusion
Chronic –> rituximab + splenectomy
Pres of TTP?
EMERGENCY!
Fever Renal failure Haemolytic anaemia Thrombocytopenia Neurological change
Cause of TTP?
Absence of VWF cleaving protein –> platelet aggregation in microvasculature + haemolysis
Seen on blood smear in TTP?
Microangiopathic blood film with SCHISTOCYTES
Mx of TTP?
Urgent plasma exchange + prednisolone + aspirin
DO NOT GIVE PLATELETS
Drug to target antibody production?
Rituximab
Pres of DIC?
Systemic collapse –> tachycardia, hypotension, oliguria
Bleeding –> bruising, purpura
Mx of DIC?
Platelet transfusion
FFP
Haemophillia inheritance?
X-linked recessive
Pres of haemophillia?
Boys - toddlers
Bleeding into muscles + joints
Pres of VWD?
Teens
Bleeding into mucus membranes and skin
Haemophillia A & B factors?
A = 8 deficient B = 9 deficient
Avoid in haemophillia?
IM injections
Aspirin
NSAIDs
Cause of VWD?
AD chromosome 12
Mx of VWD?
IV desmopressin
Vit K for which factors?
1972
What is prolonged in liver disease?
PT
Staging for lymphoma?
Ann-Arbor
HL vs NHL age?
HL = younger 20-34
Stages of CML
Chronic (asympto - 5y)
Accelerated (anaemia, thrombocytopaenia) - more blast cells in blood
Blast (pancytopaenia - can be fatal) >30% blasts
PHILADELPHIA CHROMOSOME