Hematology 3 Flashcards
Adverse risk factors of progression from MGUS to Active multiple myeloma?
Serum M protein > 1.5g/dL
Non-IgG MGUS
abnormal serum FLC ratio < 0.26 or > 1.65
Commonest T cell lymphoma?
Peripheral T lymphoma not otherwise specified (NOT)
Risk factors that worsens Hodgkin lymphoma?
ONLY WBC > 15 high
Anemia, LOW Albumin/Lymphocytes
Male, Age > 45
2 siblings having same haplotype - how many %?
25%
HBS properties?
Glutamate substituted with Svaline on B globin chain- becomes less negative charged, less soluble
- push Oxygen dissociation curve to right - to dump oxygen to tissue
- hence why malaria/Africa - have sickle cell - to improve oxygen delivery to cells
HB that has highest affinity to oxygen?
HBF
vWF half life is?
12 hours
Worse genetic prognosis in AML?
Monosomy 7
Aplastic anemia is;
What modal occurrence?
Common in what population?
progressed to what?
What you can’t see in aplastic anemia?
Bimodal peak
Asian population
AML
No polychromasia
Irradiation of RBC for?
Washed RBC for?
Leukoreduction RBC for?
Irradiation - to reduce GVHD
Washed RBC - to reduce IgA or severe allergic reactions
Leukoreduction - to reduce febrile non-hemolytic transfusion reaction/ HLA alloimmunization
What is reversible in hemochromatosis - following venesection?
Cardiomyopathy, dermal pigmentation
what thrombophilia needs anticoagulation ?
HOMOZYGOUS Factor V leiden
Anti-thrombin deficiency
The rest watchful observation
When to screen for thrombophilia in pregnancy?
- Hx of venous thrombosis - unprovoked/ transient risk factors - OCP/immobilisation/surgery/pregnancy
- Asymp. people but has family hx of thrombophilia/ VTE
Poor prognosis marker for Multiple myeloma?
Raised B2 microglobulin High LDH Low Albumin Deletion 17p Deletion 4:14
AML has what CD expression?
CD13,CD 33, CD34
Acute intermittent porphyria
-mutation gene?
- clinical features?
- blood test shows?
- treatment?
HMBS gene mutation - so no enzyme porphobilinogen deaminase —-> porphobilinogen accumulates in cytoplasm
-can’t proceed for heme synthesis
- Mainly in women
- Abdomen pain + vomiting
- Severe neuropathy - loss of deep tendon reflexes, paralysis
- psychosis/agitation/seizures
-HYPONATREMIA/HYPOMAGNESEMIA
- IV glucose to buy time
- Intravenous Hemin to reduce intensity and shorten attack duration
What does IV Hemin does?
inhibit aminolevulinic acid - high up in the chain of prophyrin synthesis
Cytarabine antidote?
Uridine Triacetate
what rebound effect if stop;
ruxolitinib?
fedratinib?
Ruxolitinib - rebound myelofibrosis
Fedratinib - Wernicke
Warm AIHA treatment?
If relapsed/refractory - what then?
Tx = Steroids/ prednisolone
Relapsed - Rituximab, last resort - splenectomy
Criteria for relapsed warm AIHA?
*-this means need secondary treatment ( rituximab)
Prednisolone 20 mg daily to maintain control of hemolysis
Ongoing hemolysis causing symptoms
HB < 110
Aplastic anaemia - when is it severe?
Therapy for non-severe Aplastic anaemia?
Therapy for severe Aplastic anaemia?
Severe if; Marrow cellularity < 25% \+ 2 of following; <0.5 neutrophils <20 platelets <20 reticulocytes
Non-severe AA - IST ( immunosuppressive therapy)
ATG + Cyclosporine
Severe AA - HSCT
if not young/fit - eltrombopag
Aplastic anaemia - what must be associated disorder must be ruled out as well?
PNH
Apoptosis has 2 main pathways -what is it?
Ligand-activated ( extrinsic) - needs binding to Transmembrane receptor
Mitochondrial ( intrinsic)
