Hematology 3 Flashcards

1
Q

Adverse risk factors of progression from MGUS to Active multiple myeloma?

A

Serum M protein > 1.5g/dL
Non-IgG MGUS
abnormal serum FLC ratio < 0.26 or > 1.65

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2
Q

Commonest T cell lymphoma?

A

Peripheral T lymphoma not otherwise specified (NOT)

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3
Q

Risk factors that worsens Hodgkin lymphoma?

A

ONLY WBC > 15 high

Anemia, LOW Albumin/Lymphocytes
Male, Age > 45

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4
Q

2 siblings having same haplotype - how many %?

A

25%

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5
Q

HBS properties?

A

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
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6
Q

HB that has highest affinity to oxygen?

A

HBF

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7
Q

vWF half life is?

A

12 hours

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8
Q

Worse genetic prognosis in AML?

A

Monosomy 7

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9
Q

Aplastic anemia is;
What modal occurrence?
Common in what population?
progressed to what?

What you can’t see in aplastic anemia?

A

Bimodal peak
Asian population
AML

No polychromasia

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10
Q

Irradiation of RBC for?

Washed RBC for?

Leukoreduction RBC for?

A

Irradiation - to reduce GVHD

Washed RBC - to reduce IgA or severe allergic reactions

Leukoreduction - to reduce febrile non-hemolytic transfusion reaction/ HLA alloimmunization

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11
Q

What is reversible in hemochromatosis - following venesection?

A

Cardiomyopathy, dermal pigmentation

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12
Q

what thrombophilia needs anticoagulation ?

A

HOMOZYGOUS Factor V leiden
Anti-thrombin deficiency

The rest watchful observation

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13
Q

When to screen for thrombophilia in pregnancy?

A
  1. Hx of venous thrombosis - unprovoked/ transient risk factors - OCP/immobilisation/surgery/pregnancy
  2. Asymp. people but has family hx of thrombophilia/ VTE
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14
Q

Poor prognosis marker for Multiple myeloma?

A
Raised B2 microglobulin
High LDH
Low Albumin
Deletion 17p
Deletion 4:14
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15
Q

AML has what CD expression?

A

CD13,CD 33, CD34

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16
Q

Acute intermittent porphyria
-mutation gene?

  • clinical features?
  • blood test shows?
  • treatment?
A

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
17
Q

What does IV Hemin does?

A

inhibit aminolevulinic acid - high up in the chain of prophyrin synthesis

18
Q

Cytarabine antidote?

A

Uridine Triacetate

19
Q

what rebound effect if stop;
ruxolitinib?
fedratinib?

A

Ruxolitinib - rebound myelofibrosis

Fedratinib - Wernicke

20
Q

Warm AIHA treatment?

If relapsed/refractory - what then?

A

Tx = Steroids/ prednisolone

Relapsed - Rituximab, last resort - splenectomy

21
Q

Criteria for relapsed warm AIHA?

*-this means need secondary treatment ( rituximab)

A

Prednisolone 20 mg daily to maintain control of hemolysis

Ongoing hemolysis causing symptoms

HB < 110

22
Q

Aplastic anaemia - when is it severe?

Therapy for non-severe Aplastic anaemia?

Therapy for severe Aplastic anaemia?

A
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

23
Q

Aplastic anaemia - what must be associated disorder must be ruled out as well?

A

PNH

24
Q

Apoptosis has 2 main pathways -what is it?

A

Ligand-activated ( extrinsic) - needs binding to Transmembrane receptor

Mitochondrial ( intrinsic)

25
Q

both apoptotic pathways produces what?

A

Caspases - cleave intracellular protein and causes cell death

26
Q

Which surgery high peri-procedural thrombotic risk?

A
  1. Heart valve replacement
  2. Carotid endarterectomy
  3. Major vascular surgeries
27
Q

Which surgery high bleeding risk?

A
  1. Large colonic polyp removal
  2. Urological procedures
  3. ICD/PPM insertion
  4. ERCP + sphincterotomy/dilatation of strictures
  5. Procedures where Minor bleeding in brain/spine
  6. PEG insertion
  7. EUS + FNA
  8. Major surgery - organ highly vascularised
28
Q

p53 mutation in CLL - means?

A

Very poor prognosis - aggressive disease

Ibrutinib as treatment!

29
Q

What organism causing Central line infection - no need removal of line?

A

coagulase negative staphylococcus

30
Q

Argatroban is what drug class?

when you use it?

A

Synthetic direct thrombin inhibitor

Use in severe renal impairment

31
Q

In HITS, treatment?

A

switch to non-heparin anticoagulant + Continue till platelet > 150

Not for platelets transfusion unless clinically bleeding

32
Q

Why Hodgkin lymphoma can use pembrolizumab/nivolumab?

A

Reed Sternberg cells in Hodgkin lymphoma escapes immune system via expression PD-L1

upregulation : EBV infection

33
Q

Key findings of ITP?

A

Isolated thrombocytopenia ( PLT >50 , no need treatment

Insidious onset - easy bruising/petechiae/gingival bleeding