Haematology Flashcards
incidental finding of thrombocytopenia
normal PT and APTT
petechiae, fatigue
immune thrombocytopenic purpura
Mx of ITP
self limiting, if severe: prednisolone
petechiae, neurological symptoms, anaemia
thrombotic thrombocytopenic purpura
Mx of TTP
plasmapheresis
polycythaemia rubra vera Px
itching after hot shower, redder skin
low erythropoetin
high MCV high haematocrit
polycythaemia mutation
JAK2 mutation
ALL Px
children, bone marrow failure, testicular enlargement, hepatosplenomegaly, increased infections
ALL Ix
Blast cells
AML Px
bone marrow failure, hepatosplenomegaly
AML Ix
Auer rods + blast cells
CML Px
weight loss, fever, massive splenomegaly, gout
CML Ix
Philadelphia chromosome, mature myeloid cells
CLL Px
Asymptomatic, old males
CLL Ix
smudge cells, increased lymphocytes
Complication of CLL
can transform into non-hodgkins lymphoma (Richter transformation)
Symptoms of lymphoma
lympadenopathy (rubbery tender lymph nodes)
fever, weight loss, night sweats
differentiating Hodgkins vs non-Hodgkins
Hodgkins has Reed-Sternberg cells (owls)
Burkitt’s lymphoma: associated with, Ix
Epstein Barr virus
Malaria
HIV
Starry sky appearance on lymph node biopsy
MALT lymphoma Px
affected area: around stomach
associated with H pylori
paraprotein spike, bence jones protein
multiple myeloma
dark urine in the morning, fatigue, dyspnea.
Ix:
paroxysmal nocturnal haemogloninuria
flow cytometry for CD55 and CD59
most common cause of aplastic crisis in pts with sickle cell / hereditary spherocytosis
parvovirus b19 infections
anaemia, bone pain, hypercalcaemia, renal failure
lytic lesions
multiple myeloma
positive coombs test?
autoimmune haemolytic anaemia
iron deficiency anaemia, high ferritin and iron. pappenheimer bodies
sideroblastic anaemia
haemophilia A inheritance
x linked recessive
Dx of haemophilia A
prolonged APTT normal PT
Pathophis of haemophilia A
factor 8 deficiency
Mx fo haemophilia A in mild vs severe bleeds
mild: desmopressin and tranexamic acid
severe: recombinant factor 8
post partum haemorrhage, prolonged APTT and PT, low Hb, low fibrinogen
DIC
investigation for multiple myeloma
urine electrophoresis to look for Bence-Jones protein
hereditary haemochromatosis
iron deposition in the tissues
Px: diabetes mellitus, hyperpigmentation, testicular enlargement, erectile dysfunction, weakness
hereditary haemochromatosis Ix
low TIBC, high ferritin, high transferrin
rouleaux, when does it occur
stacks of RBC
multiple myeloma, waldrens macroglobulinemia
howell jolly bodies
nuclear remnants found in RBC
hyposplenism
schistocytes
jagged fragments of RBC
haemolysis
tear drop cells
abnormality of bone marrow function (myelofibrosis)
nocturnal haemoglobinuria cause
defect in phosphotidylinitol glycan A
Ix findings in anaemia of chronic disease
increased ferritin
decreased TIBC
normal transferrin
features of hodgkins lymphoma
Reed sternberg cells (B cells with multi lobed nuclei)
B symptoms
Cervical/mediastinal lymph nodes
Lymph nodes hurt with alcohol
SVC obstruction, dysnpnoea
chemo for hodgkins lymphoma
ABVD
Diffuse large b cell lymphoma
high grade NHL
layers of Large B cells growing in sheets
CLL can turn into this
Assoc with Hepatitis C
Burkitt lymphoma associated with
EBV
2 variants of Burkitt lymphoma
endemic/african variant. Risk factor is co infection of EBV and malaria
Massive Jaw tumour
Sporadic variant: ileocecal tumour
Staging system for lymphoma
Ann Arbor
1: one node
2: spread on same side of diaphragm
3: both sides of diaphragm
4: extra nodal
Symptoms of ALL
bone marrow failure
infiltration (lymphadenopathy, splenomegaly, orchidomegaly, CNS involvement)
CNS involvement common in which leukaemia?
ALL
lymphocytes cross the blood brain barrier more readily
what is diagnostic of ALL
> 20% blast cells on bone marrow aspirate
3 phases of chemo for ALL and AML
- remission induction
- remission consolidation
- maintenance therapy
what should be added for management for CNS involvement
intrathecal chemotherapy
what is diagnostic of AML
> 20% blast cells and Auer rods
what is t(9:22)
Philadelphia chromosome
associated with CML
indicates poor risk for ALL
what is t(15:17)
Acute Promelanocytic Leukaemia (APML).
The PML gene fuses with the RAR alpha gene (retinoic acid receptor alpha) to make a PML-RAR fusion. This causes arrest of promelanocyte differentiation.
Retinoic acid ATRAs are effective in targeting this gene fusion, and then restoring differentiation.
important gene in CML + management
Fusion gene encoding the BCR-ABL protein, which enhances Tyrosine kinase activity
Imatinib is a tyrosine kinase inhibitor, inhibits phosphorylation
3 Phases of CML development:
- Chronic
- Accelerated
- Blastic (transforms to acute leukaemia)
CLL diagnostic criteria
smear cells
mx of non-diet related B12 deficiency
1mg hydroxycobalamin IM 3x week for 2 weeks
1mg IM every three months
sickle cell disease, anaemia and low reticulocyte count
parvovirus infection
polycythemia or myelodysplasia can lead to what malignancy?
AML
moa of rituximab
monoclonal antibody against CD20
what virus to screen for before starting rituximab
Hep B
causes of drug induced haemolytic anemia
cephalosporins (cefalexin)
quinine
transfusion threshold for ACS
Hb < 80
Transferrin, ferritin and TIBC in IDA
Low transferrin
Low ferritin
High tibc
mnemonic for multiple myeloma
CRABBI
hypercalcemia
renal impairment
anemia
bone pain
bleeding
infections
which thalassemia has high HbA2
beta thalassemia major
interesting thing about CML presentation
no bone marrow failure
what is the philadelphia chromosome
t(9,22)
standard treatment for diffuse large b cell lymphoma
RCHOP