Hematology 4

Question 1

How dietary iron enters absorptive cell from gut lumen?

Answer 1

DMT-1 channel transporter

Question 2

What EXPORT iron from cells into circulation?

where is this exporter found and suppressed by?

Answer 2

Export iron = FPN1

FPN1 located - basolateral membrane of enterocytes/macrophages

FPN1 suppressed by hepcidin!

Question 3

What converts ferrous (storage iron) to ferric?

What converts ferric into ferrous?

Answer 3

Ferroxidase- then ferric can bind to transferrin

Ferritin - sequestered ferrous in ferritin core

Question 4

Non-haem iron is what form of iron and from where?

Answer 4

Ferric iron from vegetables - not well absorbed in gut

Question 5

Lenalidomide cleared by what and given with what drug?

Lenalidomide side effect?

Answer 5

Renally cleared
given with prednisolone - for helping with fatigue

Peripheral neuropathy
Cytopenias

Question 6

Difference of IgM-MGUS and non-IgM MGUS?

Answer 6

IgM MGUS - CD20+ lymphoplasmacytic - NO switch recombination
-Progress to WM, AL amyloidosis, Lymphoma

Non-IgM MGUS - MAture plasma cells ( undergone recombination)
-progress to MM, AL amyloidosis

Question 7

Now added SLim to CRAB for MM, what does it stands for?

Answer 7

S - Sixty %
Li - light chains > 100
M - MRI shows 1 or more focal lesion + > 5 mm in lesion

Question 8

Multiple myeloma
1st line treatment/Mainstay treatment?
2nd line treatment?
All oral treatment?

Answer 8

1st line - LBD
L-lenalidomides/ domides
B- Bortezomib ( proteasome inhibitors) or Ixazomib
D - Dexamethasone

2nd line - if refractory to LBD
Panobinostat ( HDAC - histone deacetylase inhibitor)
-HDAC - express cancer-associated protein through histone modification

Oral treatment - Ixazomib
L I D
Lenalidomides
Ixazomib
Dexamethasone

Question 9

B12 and folate deficiency causes what in the cell cycle?

Answer 9

Prolonged S phase and maturation arrest

Question 10

how to differentiate non-Philadelphia myeloproliferative neoplasms?

• PV?
• ET?
• primary myelofibrosis?

Answer 10

PV bone marrow trilineage growth - granulocytes, myelocytes, megakaryocytes
+ JAK mutation

ET - megakaryocytes WITH RETICULIN FIBERS
+ JAK/CALR/MPL

Primary myelofibrosis - megakaryocytes WITHOUT RETICULIN, tear drops cells

Question 11

When acute exchange transfusion in sickle cell?

Exchange transfusion - benefit of using apheresis machine?

Answer 11

Acute Chest syndrome
Stroke

No need iron chelation therapy - iron neutral

Question 12

Peripheral blood SCT
higher rates of?

Lower rates of?

Answer 12

Higher rates of CHRONIC GVHD
Higher rates of OS and Leukemia-free-survival
Higher engraftment

Lower risk of leukemia relapse

*PBSCT and BMT = no difference in rates of Acute GVHD

Question 13

Commonest vein to have thrombosis in upper extremities?

Answer 13

Subclavian vein

Question 14

Paget-schroetter syndrome?

epidemiology of this syndrome?

Answer 14

Effort induced thrombosis in upper extremities (Subclavian vein)
a/w Malignancy

Younger, leaner, higher mortality

Question 15

Activated Protein C (+S) inactivates what?

Answer 15

F5 + 8

Question 16

What activates protein C?

Answer 16

Thrombin + Thrombomodulin ( becomes anti-coagulant)

Question 17

Waldenstrom macroglubolinemia has 2 major categories - whats that?

Answer 17

IgM paraprotein related symptoms = hypervicosity

Lymphoplasmacytic infiltration symptoms = Lymphadenopathy/splenomegaly/B symptoms

Question 18

WM emergency treatment if hypervicosity?

Answer 18

Plasma exchange to remove IgM

IVIG if hemolytic anaemia

Question 19

what is Hegde syndrome? Triad of 3As

Answer 19

Triad of 3As
Aortic stenosis

Anaemia Acquired - can cause VWF Type 2 due to shearing thru Aortic valve - losing large multimers

Angiodysplasia of intestine

Question 20

Hereditary spherocytosis a/w what stones?

Answer 20

Gallstones!

Question 21

CML in blood shows what?

Answer 21

CML = Muhibah - all the shit

Leucoerythroblastic - like AML
Basophils
Eosinophils
Neutrophils

Question 22

Multiple myeloma genetic mutation

Answer 22

17p deletion

T4.14

Question 23

ESR measures what?

Answer 23

Rate of Fall of erythrocytes (sedimentation) in a sample of blood
-quicker in MM due to stacked proteins/paraproteins

Question 24

Myeloma cast nephropathy - how it occurs?

what drug is effective against it?

Answer 24

light chains + Tamm-Horsfall proteins = casts and then block tubules

• these complexes due get broken via lysis in the end but cause inflammatory cascade

Drug - Bortezomib

Question 25

After autologous SCT - when to start prophylaxis?

Drugs prophylaxis? and against what?

Answer 25

6 months post grafting - continue for 6 months

Mainly HZV and PJP ( usually 6 months post graft)

• Bactrim
• Valaciclovir

Question 26

Commonest lymphoma?

Does FNAC has role in lymphoma invx?

Answer 26

Non-Hodgkin lymphoma

FNAC - no role

Question 27

In Follicular lymphoma - why Bendamustine and Obintuzumab cannot be use together in elderly?

Choice of tx drugs in Follicular lymphoma?

When to treat FL?

Answer 27

Toxicity - high infectious risk!

Obintuzumab + whatever chemo = good PFS

Stage 3A onwards = centrocytes and centroblasts presence

Question 28

MALT/DLBCL found where in gut?

Indolent FL found where in gut?

Mantle cell lymphoma found where?

Answer 28

MALT = Gastric /stomach

Indolent = duodenum

Mantel cell lymphoma = cutaneous and transverse/sigmoid colon

Question 29

Waldestrom Macroglobulinemia has what mutation and presence of what signifies poor prognosis?

Answer 29

MYD88 mutation

CXCR4 mutation = poor prognosis

Question 30

Mantle Cell Lymphoma has CD 23 what?

Answer 30

CD23 -VE

Question 31

Hairy cell leukemia has CD what?

Answer 31

CD25/CD100