HAEM 3 Flashcards
what is acute leukaemia
ix
abnormal proliferation and lack of maturation
excess of blasts >20% in either peripheral blood or bone marrow
decrease or normal haemopoietic reserve
ALL
cx
increased production of lymphocytes
children 2-5yo. boys>girls
haemorrhage, thrombosis, tumour lysis syndrome (hyperuricalmia and renal failure)
AML
overproduction of immature myeloid white blood cells. subgroups DIC, gum infiltration
denovo or secondary to myeloproliferative disease
auer rods
blood transfusions, ABs, chemo, stem cell transplant
CLL
commonest leukaemia. older patients. clonal expansion of small lymphocytes. 95% b cell origin
asymp. recurrent infection. anaemia. splenomegaly. hepatomegaly. LD. pallor
smudge cells
CML
malignancy of granulocytes - increased production of myeloid precursor with their differentiation ability still intact
9 and 22 translocation tyrosine kinase production
Imatinib
myeloproliferative disease BCRABI pos
neg
CML
polycythaemia, ET, myelofibrosis
what is polycythaemia
secondary
pseudopolycythemia
primary overproduction of RBCs.
chronic hypoxia, smoking, erythro secreting tumour, OSA
dehydration, diuretics, obesity
ix for polycythaemia disease
patients in secondary
treatment
cx
HCT >0.5 in m and >0.48 in F or increased RC mass >25%
FBC/BF, ferritin, JAK2 mutation
won’t have splenomegaly
venesect to HCT <0.45
aspirin to reduce thrombosis
hydroxycarbomide
myelofibrosis, AML
ET is what at high levels can lead to symptoms ix treatment
uncontrolled production of abnormal PLT. VW disease
gout, headache, weight loss, fatigue, sweats, splenomegaly, bleeding, microvascular occlusion
exclude reactive thrombosytosis: blood loss, inflam, malig, iron defic, CML
JAK2 in 50%, CALR in rest, MPL mutation . characteristic marrow appearance
aspirin. hydrocycarbimide, anagrelide, interferon beta last line
myelofibrsosis is what
idiopathic causes
secondary causes
symp
blood film:
other ix
leukoerythroblastic film causes
treatment
clonal proliferation of haemopoietic stem cells -> BM fibrosis
marrow failure, marrow fibrosis, extra medullary, haemopoiesis, leurkothrombin
post PR/ET
bleeding, anaemia, infection, splenomegaly
tear drop RBC and leukoerythroblastic
dry aspirate vibrate on trephine biopsy. JAK2 or CARL mutation
reative (sepsis), marrow infiltration (cancer), myelofibrsosis
transfusion, ABs, allogenic transplant, splenectomy, JAK inhibitor
decreased production for pancytopenia
inherited Fanconis
acquired
secondary BMF
inherited BM failure
Fanconi’s
unable to correct understanding cross links
short, skin abnormalities, radial ray abnormalities, hypogenitilia, endocrine, GI, CVS, haem, renal
84% BM failure by age 20
53% leukaemia by 40yo
acquired primary BF aplastic anaemia
myelodysplastic dysplasia
acute leukaemia
AI against haemopoietic stem cells
hyper cellular mass, ineffective homepoiesis, 20-30% -> AML
prevention of normal haemopoiesis
secondary BMF
drugs: chemo, chloramphenicol
alcohol, b12/folate defi
infiltrative: malignancy, lymphoma, viral (HIV), storage disease
increased destruction pancytopenia
hypersplenism
portan htn, CCF, felty’s, splenic lymphoma
treatment - splenectomy
clinical features of pan
anaemia, fatigue, SOB, CDV compromise, neutropenia, infections, bleed, symp depending on cause
ix for pan
FBC, film, b12/folate, LFTs, AB tests, chromosome fragility test (Fanconi’s), hypo cellular - aplastic, hyper: MDS, b12 folate, hypersplenism
treatment for pan
general treatment
malig: chemo
congenital: transplant
idiopathic aplastic anaemia: immunosup
supportive, transfusion, ABs
hodgkins lymphoma symptoms
ix
painless cervical LD, cough, hepatosplenomegaly, pruritus, alcohol related pain, B symptoms
reed sterbnergs cells. blows. imaging. histology
hodgkins stages
treatment
I confused to a single lymph node II 2 or over LN groups and on one side of the diaphragm III both sides IV extra lymphatic e.g. bone marrow A absence of B symptoms B presence of them
local - radio
extensive - chemo
burkitts NHL
b high grade
8: 14 (cymyc)
african: maxillay/mandibular EBV
sporadic: abdomen, HIV
starry sky on micro
mantle cell
similar to low grade hidgkins
v aggressive and incurable with chemo
other types NHL
marginal zone
follicular
diffuse large cell (commonest worldwide)
lymphoplasmocytic
