Haemotology Flashcards
What is Hairy cell leukaemia?
A rare malignant proliferation disorder of B cells
features of Hairy cell leukaemia
pancytopenia, splenomegaly, skin vasculitis in 1/3 patients, and tartrate resistant acid phosphotase (TRAP) stain positive
what is diagnosis if tartrate resistant acid phosphotase (TRAP) stain positive and pancytopenic?
hairy cell leukaemia (b cell malignant prolif)
Patient with high platelets and burning sensation in hands
Essential thrombocytosis… JAK2 mutation is found in around 50% of patients
It’s a myeloproliferative disorders which overlaps with chronic myeloid leukaemia, polycythaemia rubra vera and myelofibrosis.
Essential thrombocytosis
Myeloproliferative disorder which overlaps with CML, polycythemia rubra Vera, myelofibrosis
Treatment of essential thrombocytosis
hydroxyurea (hydroxycarbamide)
Or interferon-alpha in younger pt
low dose aspirin may be used
Philadelphia chromosome
Present in 95% of patients with chronic myeloid leukemia
Sometimes found in acute lymphoblastic leukaemia and is bad prognostic
Sickle cell genetics
Autosomal recessive
Features of Acute promyelocytic leukaemia
Presents younger than other AML (25yr)
And presents with DIC
good prognosis
T(15:17), Auer rods (seen with myeloperoxidase stain)
Most common type of Hodgkin’s lymphoma
Nodular Sclerosing
Good prognosis
Associated with lacunar cells
Features of lead poisoning
Abdominal pain, unusual taste in mouth, parasthesia of extremities, blue lines on gum in 20%, fatigue, constipation
Microcytic anaemia,
Features of thrombotic thrombocytopenic purpura (TTP)
Thrombotic thrombocytopenic purpura (TTP) is classically characterised as a pentad of: thrombocytopenia, microvascular haemolysis, fluctuating neurological signs, renal impairment and fever…
treat with plasma exchange
Liver biopsy of haemochromatosis
Perl’s stain
Genetic mutations in haemochromatosis
C282Y and H63D
Testing family members: HFE mutation on chromosome 6
General screen with transferrin saturation
What bloods determine if venesection is needed for haemochromatosis
Transferrin saturation and ferritin
What histology is associated with DIC
DIC is associated with schistocytes due to microangiopathic haemolytic anaemia
Medication to treat neutropenia
Filgrastim
- Recombinant human granulocyte-colony stimulating factor (rhG-CSF) stimulates the production of neutrophils
17 year old bleeding excessively after tooth extraction, most likely factor deficiency
Haemophilia A accounts for 90% of haemophilias and is due to a deficiency of factor VIII
haemophilia B (Christmas disease) there is a lack of factor IX
Diagnosis if schistocytes present on blood film
Aka helmet cells
Intravascular haemolysis (TTP) Mechanical heart valve Disseminated intravascular coagulation
Diagnosis of Haemochromatosis
Transferrin saturation…then confirm with molecular genetic testing for the C282Y and H63D mutations
autosomal recessive disorder of iron absorption and metabolism resulting in iron accumulation
HFE gene chromosome 6
most common inheritied bleeding disorder
Von Willebrand’s
Prolonged bleeding
APTT may be prolonged
Immune thrombocytopenia. Antibodies directed to…
Glycoprotein II b / III a complex
Treatment if DVT and cancer
Even though it’s provoked, you still carry on for 6months of warfarin
Hyperviscosity , weight loss, hepatosplenomegaly… IgM monoclonal paraproteinemia
Waldenstroms macro globulinaemis
Antibodies for warm Vs cool coombs test in haemolytic anaemias
IgM cold
IgG warm
Haemolytic anaenia normal wcc
Lymphoma has normal WCC. Can be warm (IgG) or cold (IgM)
Leukaemia is raised
which blood product has highest risk of bacterial contamination
platelets
Features of myeloma
C - hypercalcaemia
R - renal failure
A - anaemia (from thrombocytopenia)
B - bone features /lytic lesions
Blood tests for antiphospholipid syndrome
paradoxical rise in the APTT and reduced platelets
which immunoglobulin can cross placenta?
IgG
predominantly IgA in breast milk