-
if a lady has rheumatoid arthritis and a large spleen and is anaemic, what is the diagnosis?
felty's syndrome with pancytopenia
-
what are fragment cells (schistocytes) and what are the causes of getting them in the blood film?
- damaged RBC which have resealed their cell membrane
- causes: prosthetic heart valve, renal dialysis, microangiopathic haemolytic anaemia eg in DIC
-
if a boy with lead poisoning is anaemic, what is the diagnosis, explain?
- siderblastic anaemia
- lead inhibits the enzyme involved in HAEM synthesis causing anaemia
-
what are the causes of sideroblastic anaemia?
- congenital: x-linked, rare
- acquired: myelodysplastic disorder, after chemo or radio, alcohol, lead excess, antiTB drugs
- ineffective erythropoiesis leading to increased iron absorption and iron loading in BM, haemosiderosis (iron deposition in endocrine, liver, heart leading to damage)
-
what is the blood film and bone marrow film in siderloblastic anaemia?
- blood: hypochromic RBC
- BM: ring sideroblasts - erythroid precursors with iron deposited in mitochondria in a ring around the nucleus
-
what is the treatment of sideroblastic anaemia?
- treat cause
- pyroxidine
- repeat blood transfusion if severe anaemia
-
what is anisocytosis and when is it seen?
- variation in RBC size
- megaloblastic anaemia, thalassaemia, IDA
-
what is poikilocytosis and when is it seen?
- variation in RBC shape
- IDA, myelofibrosis, thalassaemia
-
On a blood film, what are pencil cells a feature of?
IDA
-
in which situations is the platelet count likely to DROP?
-
what is the normal daily iron requirement for adults and where is it absorbed?
-
what are the characteristic features of myelodysplastic disorders?
increased BM cellularity and dysplasia
-
what are the main differences between myelodysplastic disrobers and myeloproliferative disorders?
- myelodysplastic: spleen is not enlarged, blood count low, no basophilia
- myeloproliferative: spleen enlarged, high blood count, basophilia
-
what percent of AML myeloblasts have Auer rods?
only 25%
-
what are the symptoms of AML?
- marrow failure: anaemia, infection, bleeding
- infiltration: hepato splenomegaly, gum hypertrophy
-
why must APML be identified early?
- theres release of thromboplastin (TF + PL) which can set off DIC
- this can be prevented using all-transretinoic acid so good prognosis
-
what is the translocation of APML?
t(15:17)
-
which age group does AML affect the most?
elderly
-
if there is dietary deficiency of vitamin B12 what is the treatment?
50mcg ORAL hydrocobalamin daily!
-
why do many patients with HIV have B12 deficiency?
abnormal small intestine flora so less absorption
-
how long do our body stores of B12 last?
4 years
-
-
what is b12 needed for?
thymidine synthesis hence DNA
-
what are the causes of B12 deficiency?
- dietary - vegans
- malabsorption: stomach = pernicious anaemia, gastrectomy, terminal ileum = ileal resection, Crohns, bacterial overgrowth (HIV), tropical strue (tapeworm)
- congenital abnormalities in metabolism
-
why do you get lemon tinge skin in B12 deficiency
- combo of jaundice from haemolysis
- pallor from anaemia
-
what is subacute combined degeneration of the spinal cord?
- subacute: insidious onset with peripheral neuropathy due to low B12
- combined: i.e. symmetrical dorsal column loss (sensory and LMN signs) and symmetrical corticospinal tract loss (motor and UMN signs)
-
what is the triad seen in subacute combined degeneration of the spinal cord?
- extensor plantars (UMN)
- absent knee jerks (LMN)
- absent ankle jerks (LMN)
-
what are the causes of megaloblastic anaemia?
- B12 deficiency
- folate deficiency
-
in RA with enlarged spleen, what is the syndrome and what is the cause of anaemia?
- felty's syndrome
- pancytopenia with hypocellular bone marrow
-
what are 3 causes of avascular necrosis e.g. of femoral head?
- perthes disease
- sickle cell
- steroid use
-
What are burr cells and when are they seen?
- irregularly shaped cells
- uraemia - kidney
-
what are howell jolly bodies?
- DNA nuclear remnants in RBCs
- normally removed by spleen
-
when are howell jolly bodies seen in the circulation?
- post splenectomy
- hyposplenism - e.g. sickle cell disease, coeliac disease, congenital, IBD, myeloproliferative disease, amyloid
-
What are the causes of a normocytic anaemia?
- anaemia of chronic disease
- renal failure
- pregnancy
- haemolysis
- hypothyroidism
- bone marrow failure - aplastic anaemia
- acute blood loss
-
what are the causes of non megaloblastic macrocytic anaemia?
- cytotoxics - hydroxyurea
- hypothyroidism
- haemolysis - reticulocytosis
- alcohol
- myelodysplasia
- marrow infiltration
- antifolate drugs - e.g. phenytoin
-
what are the causes of warm AIHA?
- autoimmune conditions: SLE
- CLL, lymphomas, methyldopa
-
what is the treatment of warm AIHA?
- remove cause
- steroids
- splenectomy
- immunosuppression
-
how is warm AIHA diagnosed?
direct antiglobulin test (Coombs')
-
what does the blood film of AIHA look like?
spherocytes as macrophages remove part of RBC membrane
-
what are the causes of cold AIHA?
- infection: mycoplasma pneumoniae
- EBV
-
what are the features of cold AIHA?
Raynauds phenomenon
-
what is the treatment of cold AIHA?
-
which drugs can cause haemolytic anaemia?
- penicillin: binds to RBC membrane and so an antibody to the drug will destroy the RBC
- quinine: production of immune complexes
-
what is the treatment for APML?
ATRA to reduce DIC, gets through differentiation block
-
what is the differential diagnosis for parotid gland enlargement?
- ALL infiltration
- sarcoidosis
- mumps
-
What are the most common reasons for a hypercoagulable state? inherited or acquired?
acquired: trauma, pregnancy
-
what are the inherited causes of a thrombophilia state?
- factor V leaden: disrupts action of protein C!
- antithrombin deficiency
- protein C and S deficiency
-
what are the acquired cause of thrombophilia?
- 1. essential thrombocythaemia: lots of platelets (both ATE and VTE)
- 2. anti-phospholipid syndrome: anti cardiolipin and lupus anticaog (both ATE and VTE)
- 3. homocystinaemia
- 4. polycythaemia: sluggish flow
-
how can you get a macrocytic anaemia in coeliac disease?
folate deficiency
-
Give some examples of when you would see thrombocytopenic purport
- immune mediated thrombocytopenia: infectious mononucleosis, gold therapy, SLE
- purport in chronic alcoholism: BM suppression so less platelets made
-
what are the purpura seen in Henoch-Schonlein purpua due to? and where in the body are they typically found?
- small vessel vasculitis
- non blanching
- buttocks and extensor surfaces
-
who does Henoch-Schonlein purpua normally affect?
young women
-
where are the features of Henoch-Schonlein purpua?
- glomerulonephritis
- joint pain
- acute abdomen
-
where is folate absorbed?
duodénum and jéjunum
-
what is the difference between folate stores and B12 stores?
- folate: short - 2-3wks
- B12: long - many months (up to 4 yrs.)
-
what are the causes of folate deficiency?
- poor diet: low green veg
- malab: coeliac
- drugs: phenytoin, sodium valproate, methotrexate, trimethoprim
-
what happens if give folate supplements without checking B12 levels?
subacute combined degeneration of the spinal cord
-
when do you see Heinz bodies? how formed?
- G6PD deficiency (xlinked african and med men)
- formed from oxidised denatured Hb during oxidative crises
-
what triggers the attacks in G6PD deficiency?
- oxidative stress eg sepsis
- drugs: primaquine (used to treat malaria), sulphonamides, aspirin
- fava beans (med)
-
what does the blood film of G6PD deficiency look like?
- bite cells: following removal of Heinz
- Heinz bodies: formed from oxidised denatured Hb
- nucleated RBC
-
which infections are sickle cell patients specifically susceptible to and why?
- encapsulated organisms eg Strep pneumo, H. influenzae and N.Meningitidis
- these are normally removed by spleen
- but in sickle - splenic infarction and some have splenectomy.
-
name 2 different types of anaemia when you see spherocytes on blood film
- hereditary spherocytosis: auto doom, most common inherited haemolytic anaemia in N.europe
- AIHA
-
how is the diagnosis of hereditary spherocytosis made?
osmotic fragilité tests: increased RBC osmotic fragility in hypotonic solutions
-
what are 5 features of hereditary spherocytosis?
- 1. haemolytic crises with bad jaundice
- 2. aplastic anaemia
- 3. folate deficiency due to increased BM requirement
- 4. pigment gall stone
- 5. leg ulcers
-
which chromosome is B globin on?
11
-
what are the features of B thal trait?
- asymp or mild microcytic anaemia
- often confused with IDA
-
what are the features of B thal intermedia?
- moderate anaemia
- maybe splenomegaly
-
what are the features of B thal major? (cooleys)
- severe anaemia
- HbA absent
- extramedullary haematopoiesis - frontal bossing,
- life long transfusions needed
- haemochromatosis - DM, hypopit, cardiac, liver toxicity
-
what are the features of skull XR in B thal major?
hair on end skull XR
-
which chromosome is a globin on?
16
-
what are the features of a thalassaemia with 1 gene deletion?
clinically normal
-
what are the features of a thalassaemia with 2 gene deletion?
- low MCV
- asymptomatic carrier state
-
what are the features of a thalassaemia with 3 gene deletion?
- alpha thal major
- HbH disease
- Excess B chains causes formation of B4 tetratmers (HbH)
- moderate anaemia
- hepatosplenomegaly
- leg ulcers
- jaundice
-
what are the features of a thalassaemia with 4 gene deletion?
- HbBarts - physiologically useless
- death in utero - hydrops fettles
-
which leukaemia is particularly associated with DIC?
APML (prevent with ATRA)
|
|
