Medicine - Haematology Flashcards
define anaemia
low Hb
TAILS: causes of microcytic anaemia?
NAME?
3As and 2Hs: causes of normocytic anaemia?
NAME?
how can macrocytic anaemia be classified?
- megaloblastic| - normoblastic
causes of megaloblastic anaemia?
- B12 def| - folate def
causes of normoblastic, macrocytic anaemia?
NAME?
symptoms of anaemia?
NAME?
which conditions may worsen with anaemia?
NAME?
which 3 symptoms of anaemia are specific to iron deficiency anaemia?
NAME?
signs O/E of anaemia?
NAME?
which signs O/E are specific to iron deficiency anaemia?
NAME?
which type of anaemia is jaundice specific to?
haemolytic anaemia
which type of anaemia do bone deformities indicate?
thalassaemia
findings O/E of anaemia due to CKD?
NAME?
bloods done to investigate for anaemia?
NAME?
non-blood test investigations done in anaemia?
- oesophageal-gastroduodenoscopy (OGD)| - bone marrow biopsy
causes of iron deficiency?
NAME?
where in the gut does iron get absorbed? what is the soluble form of iron?
- duodenum and jejunum| - ferrous (Fe 2+)
how is iron converted from the insoluble Fe3+ to the soluble Fe2+?
using stomach acid
how can PPIs cause an iron deficiency?
- they reduce gastric acid secretion- stops iron being converted to the soluble form of Fe 2+- therefore not absorbed
most common cause of iron deficiency in adults not menstruating?
blood loss in the GI tract
most common cause of iron deficiency in children?
dietary insufficiency
how can transferrin saturation be calculated?
serum iron / TIBC
what can cause a raised ferritin?
infection
how are TIBC and transferrin affected by iron deficiency?
both increase
how are TIBC and transferrin affected by iron overload?
both decrease
what could cause iron studies to give an iron overload impression?
- iron supplements| - acute liver damage
3 management options for iron deficiency anaemia?
- blood transfusion2. iron infusion3. PO ferrous sulfate
how should Hb rise with oral iron supplements?
10g/L per week
what are the 2 causes of B12 deficiency?
- insufficient dietary uptake| - pernicious anaemia
what is needed to absorb B12? where is this secreted?
- intrinsic factor| - parietal cells of the stomach
where in the gut does B12 get absorbed?
terminal ileum
neuro signs of B12 deficiency?
NAME?
pathophysiology of pernicious anaemia?
NAME?
how is pernicious anaemia diagnosed?
test for the following antibodies:- intrinsic factor antibody (1st line)- gastric parietal cell antibody
management of insufficient dietary B12?
PO cyanocobalamin supplements
management of pernicious anaemia? hint: can’t be oral because won’t be absorbed
IM hydroxycobalamin
why is it important to treat a B12 def before a folate def (in pts with both)?
giving folic acid to pt with B12 def can cause subacute combined degeneration of the spinal cord
how can causes of haemolytic anaemia be classified?
inherited vs acquired
inherited forms of haemolytic anaemia?
NAME?
acquired forms of haemolytic anaemia?
NAME?
signs O/E of haemolytic anaemia? (hint: all due to RBC destruction)
NAME?
investigations for haemolytic anaemia?
NAME?
what is seen on the blood film in haemolytic anaemia?
schistocytes (fragments of RBCs)
when does the direct Coombs test give a positive result?
autoimmune haemolytic anaemia
mode of inheritance of hereditary spherocytosis?
autosomal dominant
presentation of hereditary spherocytosis?
NAME?
which organism can bring on an aplastic crisis in hereditary spherocytosis patients?
parvovirus
how is hereditary spherocytosis diagnosed?
NAME?
findings on blood film in hereditary spherocytosis?
spherocytes
finding on FBC in hereditary spherocytosis? hint: it’s a rogue one
MCHC is raised
treatment of hereditary spherocytosis?
- folate supplements| - splenectomy
key difference between hereditary spherocytosis and hereditary elliptocytosis?
- in the second one, RBCs are ellipse shaped| - otherwise identical
mode of inheritance for G6PD deficiency?
NAME?
G6PD deficiency is more common in patients of which descent?
Mediterranean / African
what can trigger a G6PD deficiency crisis?
NAME?
drugs which cause G6PD deficiency crisis?
NAME?
how is G6PD deficiency diagnosed?
G6PD enzyme assay
signs O/E of G6PD deficiency crisis?
NAME?
what is seen on the blood film of someone in G6PD deficiency crisis?
Heinz bodies
how can autoimmune haemolytic anaemia (AIHA) be classified?
- warm type: haemolysis at normal or higher temperatures| - cold type: haemolysis at colder temps
causes of warm AIHA?
idiopathic
causes of cold AIHA?
NAME?
management of AIHA?
NAME?
what is alloimmune haemolytic anaemia? give 2 types
NAME?
what are the chains making up normal adult Hb?
2 alpha + 2 beta globin chains
pathophysiology of thalassaemia?
NAME?
potential signs and symptoms of thalassaemia?
NAME?
investigations in thalassaemia?
NAME?
how can thalassaemia (and treatment) result in iron overload?
- increased Fe absorption in response to anaemia| - recurrent blood transfusion therapy
what needs to be monitored in thalassaemia patients? why?
- serum ferritin| - to check for iron overload
presentation of iron overload in thalassaemia? hint: similar to haemachromatosis
NAME?
where is the affected gene in alpha thalassaemia?
chromosome 16
where is the affected gene in beta thalassaemia?
chromosome 11
how is iron overload in thalassaemia managed?
- limit blood transfusions| - iron chelation
management of alpha thalassaemia?
NAME?
what are the types of beta thalassaemia?
- thalassaemia minor - “ “ intermedia - “ “ major
presentation of thalassaemia minor?
- mild microcytic anaemia| - only monitoring needed, no treatment
presentation of thalassaemia minor? management?
- mild microcytic anaemia| - only monitoring needed, no treatment
presentation of thalassaemia major?
NAME?
pathophysiology of sickle cell anaemia?
- abnormal beta globin chain gene on chromosome 11 - this causes HbS to form instead of HbA - HbS causes RBCs to be sickle-shaped
what is the relation between sickle cell trait and malaria?
having sickle cell trait reduces the severity of malaria infection
how is sickle cell disease tested for in newborns?
part of the day 5 heel-prick test (Guthrie)
complications of sickle cell anaemia?
NAME?
general management principles of sickle cell anaemia?
NAME?
potential triggers of a sickle cell crisis?
NAME?
management of a sickle cell crisis?
all supportive:- low threshold for admission- treat underlying cause (e.g. ABx, keep warm, hydrated)- paracetamol / ibuprofen - penile aspiration if priapism
pathophysiology of a vaso-occlusive crisis?
- sickle-shaped RBCs clog up capillaries| - causes distal ischaemia
presentation of vaso-occlusive crisis?
NAME?
pathophysiology of splenic sequestration crisis? how does it present?
NAME?
management of splenic sequestration crisis?
NAME?
what is an aplastic crisis in sickle cell disease?
temporary cessation of erythropoiesis
typical trigger of an aplastic crisis?
parvovirus B19 infection
management of aplastic crisis?
- blood transfusions if needed| - otherwise self-resolving within a week
how is acute chest syndrome of sickle cell disease diagnosed?
both must be present:- fever / resp symptoms - new infiltrates seen on CXR
infective causes of acute chest syndrome?
- bronchiolitis| - pneumonia
non-infective causes of acute chest syndrome?
- pulmonary vaso-occlusion| - fat emboli
management of acute chest syndrome?
NAME?
age groups typically affected by ALL?
<5 years and >45 years
age group typically affected by CLL?
> 55 years
age group typically affected by CML?
> 65 years
age group typically affected by AML?
> 75 years
presentation of leukaemia? hint: pancytopenia
NAME?
differentials for a non-blanching rash?
- leukaemia- meningococcal septicaemia- vasculitis- HSP (lower limbs, buttocks)- ITP- NAI (children / vulnerable adults)
initial investigation for leukaemia?
urgent FBC (<48h) for all with suspected leukaemia
investigations for leukaemia?
NAME?
what might the blood film show in leukaemia?
- abnormal cells| - inclusions
where is a bone marrow biopsy usually taken from?
iliac crest
which cell type over-proliferates in ALL and CLL?
usually B-lymphocytes
which genetic condition is ALL associated with?
downs syndrome
blood film findings in ALL?
blast cells
characteristic chromosomal change seen in CML in adults?
philadelphia chromosome
what type of anaemia could CLL give?
warm autoimmune haemolytic anaemia
which other cancer could CLL transform into?
- high-grade lymphoma| - called richter’s transformation
blood films findings in CLL?
“smudge” / “smear” cells (WBCs which ruptured in the process of preparing the film)
3 phases of CML?
NAME?
3 phases of CML?
- chronic phase (asymptomatic, 5 years)- accelerate phase - blast phase
what is seen in the blast cells on a blood film in AML?
auer rods
which cancer could a myeloproliferative disorder (e.g. PRV) transform into?
AML
management of leukaemia?
NAME?
complications of chemotherapy for leukaemia?
NAME?
pathophysiology of tumour lysis syndrome?
NAME?
management of tumour lysis syndrome?
allopurinol and rasburicase to reduce uric acid levels
how can lymphoma be categorised?
- hodgkin’s| - non-hodgkin’s (all the other types)
peak ages affected by hodgkin’s lymphoma? hint: bimodal
aged 20 and then aged 75
risk factors for hodgkin’s lymphoma?
NAME?
presentation of hodgkin’s lymphoma?
NAME?
what are B symptoms? list them
NAME?
investigations in hodgkin’s lymphoma
NAME?
lymph node biopsy findings in hodgkin’s lymphoma?
reed-sternberg cells (large B cells with multiple nuclei)
what is ann arbor staging used for? what does it take into account?
NAME?
management of hodgkin’s lymphoma?
- chemotherapy| - radiotherapy
prognosis of hodgkin’s lymphoma?
- chemo and radiotherapy are curative- they both carry own risk of secondary malignancy- B = BAD (B-symptoms have worse prognosis)
types of non-hodgkin’s lymphoma?
NAME?
key association with MALT lymphoma?
H. pylori infection
which other conditions are associated with burkitt lymphoma? hint: all infections
NAME?
risk factors for non-hodgkin’s lymphoma?
- HIV- EBV - H. pylori (MALT)- hep B / C- exposure to pesticides- FHx
management of non-hodgkin’s lymphoma?
NAME?
pathophysiology of myeloma? what is multiple myeloma?
NAME?
skin sign of pernicious anaemia?
gives “lemon tinged” skin
most common immunoglobulin to be raised in myeloma?
IgG
urinalysis finding in myeloma?
bence jones protein
which bones are most commonly affected in myeloma?
NAME?
effects of myeloma on bone turnover? result of this?
NAME?
how does myeloma cause renal impairment? hint: multifactorial
- lots of Igs block tubular flow- high Ca- dehydration - bisphosphonate therapy … all cause impairment
how is plasma viscosity affected in myeloma?
- increases| - due to increased Igs
complications relating to increased plasma viscosity in myeloma?
NAME?
what type of anaemia arises from myeloma?
normocytic, normochromic
how does myeloma cause anaemia?
bone marrow being resorbed
presentation of myeloma?
NAME?
risk factors for myeloma?
NAME?
what must be considered in anyone over 60 with bone pain? which investigation is key?
- myeloma| - urgent FBC required
findings on bloods for myeloma?
NAME?
BLIPE: initial investigations for multiple myeloma?
- Bence jones protein (urine electrophoresis)rest are all in blood: - Light-chain assay - Immunoglobulins - Protein Electrophoresis
diagnostic investigation for multiple myeloma?
bone marrow biopsy
pereferred investigation to stage multiple myeloma?
whole body MRI
signs on X-ray in multiple myeloma?
- punched out lesions - lytic lesions - “rain-drop skull” appearance
management of myeloma?
NAME?
management of bone disease secondary to myeloma?
NAME?
complications associated with myeloma?
NAME?
what are the 3 types of myeloproliferative disorder?
NAME?
gene mutations associated with myeloproliferative disorders?
NAME?
presentation of myeloproliferative disorders?
NAME?
feautres of polycythaemia vera?
- plethoric conjunctiva and face- “ruddy” complexion- splenomegaly- pruritis, worse after hot bath - HTN
findings on FBC in polycythaemia vera?
isolated rise in Hb
findings on FBC in essential thrombocythaemia?
raised platelets
findings on FBC in myelofibrosis?
more variable: - anaemia (low Hb)- high OR low WCC- high OR low platelets
findings on blood film in myelofibrosis?
NAME?
diagnostic investigation and finding for myeloproliferative disorders?
- bone marrow biopsy| - comes back “dry”
management of myelofibrosis?
- if mild, none needed- allogeneic stem cell transplant could be curative- chemotherapy- supportive (for anaemia / splenomegaly / portal HTN)
management of polycythaemia vera?
NAME?
management of essential thrombocythaemia?
- aspirin| - chemotherapy
findings on FBC in myelodysplastic syndrome?
NAME?
risk factors for myelodysplastic syndrome?
NAME?
which malignancy could myelodysplastic syndrome transform into?
AML
presentation of myelodysplastic syndrome?
NAME?
how can a diagnosis of myelodysplastic syndrome be confirmed?
on bone marrow aspiration and biopsy
management of myelodysplastic syndrome?
NAME?
causes of thrombocytopenia relating to reduced platelet production?
NAME?
causes of thrombocytopenia relating to increased platelet destruction?
NAME?
drugs which can cause thrombocytopenia?
NAME?
presentation of thrombocytopenia?
NAME?
differentials for prolonged bleeding?
NAME?
pathophysiology of ITP?
antibodies made against platelets
management of ITP?
NAME?