Hematology 4 Flashcards
How dietary iron enters absorptive cell from gut lumen?
DMT-1 channel transporter
What EXPORT iron from cells into circulation?
where is this exporter found and suppressed by?
Export iron = FPN1
FPN1 located - basolateral membrane of enterocytes/macrophages
FPN1 suppressed by hepcidin!
What converts ferrous (storage iron) to ferric?
What converts ferric into ferrous?
Ferroxidase- then ferric can bind to transferrin
Ferritin - sequestered ferrous in ferritin core
Non-haem iron is what form of iron and from where?
Ferric iron from vegetables - not well absorbed in gut
Lenalidomide cleared by what and given with what drug?
Lenalidomide side effect?
Renally cleared
given with prednisolone - for helping with fatigue
Peripheral neuropathy
Cytopenias
Difference of IgM-MGUS and non-IgM MGUS?
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
Now added SLim to CRAB for MM, what does it stands for?
S - Sixty %
Li - light chains > 100
M - MRI shows 1 or more focal lesion + > 5 mm in lesion
Multiple myeloma
1st line treatment/Mainstay treatment?
2nd line treatment?
All oral treatment?
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
B12 and folate deficiency causes what in the cell cycle?
Prolonged S phase and maturation arrest
how to differentiate non-Philadelphia myeloproliferative neoplasms?
- PV?
- ET?
- primary myelofibrosis?
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
When acute exchange transfusion in sickle cell?
Exchange transfusion - benefit of using apheresis machine?
Acute Chest syndrome
Stroke
No need iron chelation therapy - iron neutral
Peripheral blood SCT
higher rates of?
Lower rates of?
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
Commonest vein to have thrombosis in upper extremities?
Subclavian vein
Paget-schroetter syndrome?
epidemiology of this syndrome?
Effort induced thrombosis in upper extremities (Subclavian vein)
a/w Malignancy
Younger, leaner, higher mortality
Activated Protein C (+S) inactivates what?
F5 + 8
What activates protein C?
Thrombin + Thrombomodulin ( becomes anti-coagulant)
Waldenstrom macroglubolinemia has 2 major categories - whats that?
IgM paraprotein related symptoms = hypervicosity
Lymphoplasmacytic infiltration symptoms = Lymphadenopathy/splenomegaly/B symptoms
WM emergency treatment if hypervicosity?
Plasma exchange to remove IgM
IVIG if hemolytic anaemia
what is Hegde syndrome? Triad of 3As
Triad of 3As
Aortic stenosis
Anaemia Acquired - can cause VWF Type 2 due to shearing thru Aortic valve - losing large multimers
Angiodysplasia of intestine
Hereditary spherocytosis a/w what stones?
Gallstones!
CML in blood shows what?
CML = Muhibah - all the shit
Leucoerythroblastic - like AML
Basophils
Eosinophils
Neutrophils
Multiple myeloma genetic mutation
17p deletion
T4.14
ESR measures what?
Rate of Fall of erythrocytes (sedimentation) in a sample of blood
-quicker in MM due to stacked proteins/paraproteins
Myeloma cast nephropathy - how it occurs?
what drug is effective against it?
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
After autologous SCT - when to start prophylaxis?
Drugs prophylaxis? and against what?
6 months post grafting - continue for 6 months
Mainly HZV and PJP ( usually 6 months post graft)
- Bactrim
- Valaciclovir
Commonest lymphoma?
Does FNAC has role in lymphoma invx?
Non-Hodgkin lymphoma
FNAC - no role
In Follicular lymphoma - why Bendamustine and Obintuzumab cannot be use together in elderly?
Choice of tx drugs in Follicular lymphoma?
When to treat FL?
Toxicity - high infectious risk!
Obintuzumab + whatever chemo = good PFS
Stage 3A onwards = centrocytes and centroblasts presence
MALT/DLBCL found where in gut?
Indolent FL found where in gut?
Mantle cell lymphoma found where?
MALT = Gastric /stomach
Indolent = duodenum
Mantel cell lymphoma = cutaneous and transverse/sigmoid colon
Waldestrom Macroglobulinemia has what mutation and presence of what signifies poor prognosis?
MYD88 mutation
CXCR4 mutation = poor prognosis
Mantle Cell Lymphoma has CD 23 what?
CD23 -VE
Hairy cell leukemia has CD what?
CD25/CD100
