Haem Flashcards
only type of microcytic anaemia with increased reticulocyte count
3 gene deletion alpha thalassemia
only type of microcytic anaemia with increased reticulocyte count
3 gene deletion alpha thalassemia
new fact about sideroblastic anemia
it causes BOTH micro and MACROcytic anaemia
when to transfuse in anaemia
- symptomatic
- Hct very low
- elderly
- heart disease
Hct very low
what transfusion to use in person with IgA deficiency
give IgA deficient FFP
each unit of PRBC….
increases Hct by 3 points per unit, or 1g/dl of Hg
what is NOT the answer to haemophilia A or B
FFP
cryoprecipitate….
NEVER used first for anything
whole blood….
NEVER correct, since divided into PRBC or FFP
transfuse in asymptomatic young person with anaemia
NO NEED TO TRANSFUSE
levels of hepcidin in anaemia of chronic disease
LOW levels
PC= asymptomatic anaemia with NORMAL Fe studies
thalassemia
target cells most commonly found in….
thalassemia
ferritin in Fe deficiency anaemia
= LOW;
but……1 out of 3 patients: normal or increased
new thing elevated in Fe deficiency anaemia
platelets
new fact about sideroblastic anemia
it causes BOTH micro and MACROcytic anaemia
when to transfuse in anaemia
- symptomatic
- Hct very low
- elderly
- heart disease
Hct very low
less than 25-30
what transfusion to use in person with IgA deficiency
give IgA deficient FFP
each unit of PRBC….
increases Hct by 3 points per unit, or 1g/dl of Hg
what is NOT the answer to haemophilia A or B
FFP
cryoprecipitate….
NEVER used first for anything
whole blood….
NEVER correct, since divided into PRBC or FFP
transfuse in asymptomatic young person with anaemia
NO NEED TO TRANSFUSE
levels of hepcidin in anaemia of chronic disease
LOW levels
PC= asymptomatic anaemia with NORMAL Fe studies
thalassemia
target cells most commonly found in….
thalassemia
ferritin in Fe deficiency anaemia
= LOW;
but……1 out of 3 patients: normal or increased
new thing elevated in Fe deficiency anaemia
platelets
most accurate test for Fe deficiency anaemia (But rarely done….)
bone marrow biopsy
most accurate test in thalassemia
electrophoresis
alpha thalassemia diagnosis
DNA analysis
only anemia a/w end stage renal failure responds to…
EPO replacement
tx of sideroblastic anaemia
B6
Cooley anemia
= beta thalassemia major
diagnosis of MACROcytic anaemia
peripheral blood smear (because looking for hyperhsegmented neutrophils)
increased methylmalonic acid
B12 deficiency
B12 deficiency
= high LDH + high bilirubin + low reticulocytes
—-> ineffective erythropoiesis
schilling test
NEVER the correct answer
pernicious anaemia dx confirmation
Anti-if Ab, Anti-parietal cell Ab
B12 and folate deficiency cause what two things…
- MACROcytic anaemia
2. PANCYTOPENIA!!!!!!!!!!!!!!!!!!!!!
improvement of neuro symptoms with B12 deficiency
- minor
- short duration
complication of B12 or folate replcaement
HYPOkalaemia, because so quickly new blood cells are made–> marrow packages up all the potassium
best initial test for sickle cell
blood smear
most accurate test for sickle cell
electrophoresis
2 things that lower mortality in sickle cell
- hydroxyurea prevention
2. antibiotics with fever
morulae inside neutrophils
ehrlichia infections
what to do…. sickle cell patient with fever
ANTIBIOTICS ASAP– do not wait for testing
exchange transfusion severe VOC:
- acute chest syndrome
- priapism
- stroke
- visual disturbance– retinal infarct
parvovirus B19 most accurate test
PCR > IgM level
best initial tx of parvovirus B19
IVIG
bone marrow in parvovirus B19
rarely done, but shows…
GIANT PRONORMOBLASTS
treatment of warm agglutinin disease
- steroids
- splenectomy
- IVIG
- rituximab
alternate treatments of warm agglutinin disease, to diminish need for steroids:
- cyclophosphamide
- cyclosporine
- azathioprine
- mycophenolate mofetil
tx of cold agglutinin
- stay warm
- rituximab and sometimes plasmapheresis
- cyclophosmaide, cyclosporine, other immunosuppressive agents, stop Ab production
steroids and splenectomy in cold agglutinin
NEVERRRRRRR NOOOOOOO WRONG ANSWER
cryoglobulins a/w
Hep C
joint pain
glomerulonephritis
DELAY plasmapharesis tx of HUS/TTP….
transfuse FFP
transfusion of platelets in HUS/TTP
NOOOOOOOOOOOOO
worsens the disease
most accurate test in PNH
FLOW CYTOMETRY– CD55,CD59
treatment of PNH
- prednisone– best initial tx for haemolysisi
- BMT-allogenic= CURE
- Eculizumab: for haemolysis and thrombosis
- folic acid and replacement with transfusions as needed
most common sites of thrombosis in PNH
- hepatic vein thrombosis
2. mesentetric vein thrombosis
tx of aplastic anaemia
- blood transfusion (anemia), antibiotics (infection), platelets (bleeding)
- BMT allogenic– young
- too old for BMT
tx if too old for BMT in aplastic anaemia
- antithymocyte globulin= ATG
2. cyclosporine (or Tarcolimus), since T cell mediated response
2 things that present with pruritus after bath in harm onc
- PCV
2. CML
MCV in PCV
LOW
after spleen removal in PCV, what happens to platelets
transient INCREASE in platelets
new point of thing elevated in PCV
B12 INCREASE (unsure reason why)
most accurate test PCV
JAK2 mutation
ruzolitinib
inhibits JAK2
JAK2 in ET
50%
ET secondary in
- infection
- malignancy
- Fe deficiency
best initial tx of ET
HYDROXYUREA
Tx erythromelalgia
ASPIRIN
tx of myelofibrosis
thalidomide and lenalidomide
best initial test for acute leukaemia
BLOOD SMEAR– BLASTS
best indicator of prognosis in acute leukaemia is
CYTOGENETICS
what tx must you add for ALL
intrathecal MTX
most accurate test in CML
FISH or PCR– Bcr:Abl
best initial therapy for CML
Imatinib
Dasatinib
Nilotinib
= TK inhibitors
most effective cure of CML
BMT– NEVER this first therapy
of all the myeloproliferative disorders– which is most likely to transform into acute leukemia
CML
tx of leukostasis reaction
LEUKAPHERESIS
what deletion is associated with myelodysplastic syndrome
5q deletion
what 2 complications occur in MDS before development of acute leukaemia
bleeding and infection
diagnosis of MDS:
- CBC: increased mcv, nucleated red cells, less than 20% blasts
- hypercellular marrow
- ringed sideroblasts
- severity– blast percentage dependent
5/ 5q deletion= better prognosis
5q deletion tx
LENALIDOMIDE
ringed sideroblasts not in sideroblastic anemia
myelodysplastic syndrome
what drug decreases transfusion dependence, and ultimately increases survival in MDS
AZACITIDINE
richter phenomenon
5% CLL–> acute leukemia
stage 3 CLL
anaemia
stage 4 CLL
thrombocytopenia
stage 0 CLL
elevated WBC
stage 1 CLL
LAD
stage 2 CLL
HSM
tx for stage 0-2 CLL
NO TX
tx for stage 3 and 4 CLL
- fludrabine
- cyclophosphamide
- rituximab
when fludrabine fails in CLL
alemtuzumab= anti-CD-52
refractory cases CLL
cyclophos
mild cases of CLL
chlorambucil (elderly)
severe infection CLL
IVIG
autoiummune thrombocytopenia or hemolysis in CLL
prednisone
what prophylaxis in CLL
PCP
which is less dangerous: thrombocytopenia and anemia from autoimmune effect, or from marrow infiltration with CLL cells?
autoimmune effect– treated with prednisone (NOT the same as stage 3/4)
tx of hairy cell leukemia
cladribine or pentostatin
non-HL best initial test
excisional biopsy– NOT NEEDLE ASIRATION
tx of non-HL stage Ia, IIa
local radiation and small course CTX
tx of non-HL stage III, IV, B symptoms
CHOP + rituximab Cyclophosphamide Hydroxydaunorubicin Oncovin== vincristine Prednisone
presentation of advanced non-hl
80 to 90%
tx of HL
ABVD Adriamycin Bleomycin Vinblastine Dacarbazine
complications off radiation
solid tumors: breast/thyroid/lung
increased chance of PREMATURE CORONARY ARTERY DISEASE
relapses after radiaiton….
tx= CTX
relapses after CTX…..
tx= INCREASE CTX
what scan before adriamycin
MUGA or nuclear ventriculogram
SPEP in MM
IgG= 60% IgA= 25%
bence jones protein found on….
urine electrophoresis
most common causes of death in MM
INFECTION
RENAL FAILURE
what type of anion gap in MM
DECREASED anion gap (since IgG= positive, increase Cl-, HCO3-)
single most accurate test for MM
bone marrow biopsy: greater than 10% plasma cells
of those with M spike, how many have myeloma
1% have myeloma
99% DO NOT have myeloma
best initial therapy in waldenstroms
PLASMAPHARESIS
SIZE and ITP
spleen= normal platelets= LARGE
no bleeding, count> 30,000 ITP
NO tx
mild bleeding, count
glucocorticoids
severe bleeding (GI or CNS), count
IVIG, anti-D
recurrent episodes, steroid dependence ITP
splenectomy
ineffective splenectomy or steroids in ITP
- romiplostim or eltrombopag
- rituximab
- azathioprine
- cyclosporine
- mycophenolate
romiplostim
synthetic thrombopoietin
eltrombopag
synthetic thrombopoietin
tx of HIT
STOP all heparin containing products
- administer direct thrombin inhibitors: argatroban, lepirudin, bivalrudin, fondaparinux
scenario where get one clot, but require lifelong warfarin
APLS
arterial or venous clots in APLS
VENOUS
confirmed dx of HIT
ELISA– platelet factor 4 antibodies
best initial test for APLS
mixing study
transfuse platelets in those with HIT?
NOOOOOOO makes it worse
