Haem Flashcards
Thelper
CD 3, CD 4, CD 28
Cytotoxic T
CD 3, CD8, CD 28
T reg
CD 3, CD 4, CD 25,
B cell
CD 19, CD 20, CD 40, B7 (for CD 28)
macrophage
CD 14, CD 40, B7 (for CD 28)
NK cell
CD 16, CD 56
CD 23
CLL (negative in Berkett)
CD 105
Hairy Cell
CD13, 33, 34, 117 and MPO
AML
t 9;22
CML- philadelphia
t 15;17
APML- PLM/RAR alpha fusion
t 8;14
Burkitt- MYC oncogene
t 11;14
Mantal cell- BCL 1
t 14;18
follicular- BCL 2
CD 15, CD 30
Hodgkins, Reed-stenberg
Hodgkin poor prognosis
age > 45, stage IV, Hb < 105, lymph < 0.6, male, albumin<40, WCC >15
Hodgkin treatment
ABVD- doxorubicin, bleomycin, vinblastine, dacarbazine
BEACOPP- sub dacarbazine to procarbazine, cyclophos, pred
** secondary cancer, pulmonary, cardiac, infertility
Brentuximab- anti CD 30 conjugate with MMAE tubulin toxin–> peripheral neuropathy
PD-1 - pembro
CD 10/19/20, BCL2, BCL 6
Follicular. B cells
Grade 1-3B
BCL2 overexpression
nodular growth pattern on histology
treat when advance stage symptomatic
Follicular treatment
RCHOP, RCVP (cyclo/vincris/pred)
OBintuzumab + Bendamustine- anti CD 20- better then ritux
Mantle cell
CD 19, CD 20, CD 5, negative CD 23
B symptoms, splenomegaly
Ritux + chemo + ASCT
New - ibrutinib for relapsed ( inhibitor of Bruton’s tyrosine kinase)
Waldenstrom
plasma cells
lymphoplasmacitic lymphoma
high Ig M
CD19, CD20, CD22, CD25, CD27, CD38, CD79a, FMC
MYD 88 or CXCR 4 mutation
peripheral neuropathy, lymph, splien, B symptoms, hyperviscos, cryoglobulinaemia
PLEX, anti CD 20
alkylating agents (chlorambucil, cyclophosphamide,melphalan), purine analogues (cladribine, fludarabine)
Ibrutinib
POEMS
polyneuropathy, organomegaly, endocrinopathy (adrenal, thyroid, pit), monoclonal plasma cell, skin changes
+ bone lesion, castleman
MGUS
non-IgM
monoclonal protein <30g, BM plasma cell <10%, no End organ dam
plasmacytoma
biopsy proven bone/soft tissue plasma cell
BM <10%
no end organ dam
radiation
transform risk 10-15%
MM
IgG 50%, IgA 20%
rouleaux
B 2 microglobulin
CRAB- anaemia most common
hyperviscous
AL amyloid
high risk MM
del 17p, t14;16, t 16;20, 1q,
MM staging
revised ISS
high b2 microglobuin, genetics
LDH high
unfit
bortezomib
proteasome inhibitor
MM 1st line
lenalidomide
immunomodulator Inhibition of TNF-a and IL-6 / VEFG
MM 1st line , maintainance
deletion 5q in MDS
myelosuppression, renal clear. increase clots
daratutumab
anti‐CD38
MM relapsed
interfere with transfusion testing !!
Elotuzumab
anti‐SLAMF7
MM relapsed
pomalidomide
immunoomodulator
MM relapsed
CART
chimeric antigen receptor T cell
target BCMA, FcRH5 in MM
Tisagenleclucel- DLBCL, B-ALL
Axicabtagene- LBCL
infections, B cell aplasia, cytopenia
Hairy cell
CD 19/20, CD11c, CD25 & CD103
Tartrate-Resistant Acid Phosphatase on histology
splenomegaly and pancytopenia
BRAF inhibitor: Vemurafenib
DLBCL
CD 19, 20, 22, 79a
Ki-67= proliferation marker
B symptom, nodes
International prognosis
- age, ECOG, stage, extranodes, LD
DLBCL
CD 19, 20, 22, 79a
Ki-67= proliferation marker
B symptom, nodes
International prognosis
- age, ECOG, stage, extranodes, LD
RCHOP
Burkett
CD 19,20, 22, 79a, CD 10, BCL 6
MYC chrosome 8
t8;14
EBV, AIDs
“starry sky: histicocytes
jaw/facial bone, lymph node, TLS
CNS prophylaxis
HLH
raised ferritin, triglyceride
soluble CD 25 (solluble IL2 receptor alpha)
elevated CXCL9
Dex, etop, cyclo
IVIG, anakinra, ritux
cytokine release syndrome
IL6 sytolkine storm, oedema, organ dysfunction, fever shock, hypoxic
give tocilizumab IL6 and steroids
ICAN
Immune effector cell associated neurotoxicity syndrome
IL-1B into BBB
headache, ehcephalopathy, delirium
steroids, seizure prophylaxis
romiplostin
ITP- activate thrombopoeitin receptor
PNH
PIGA gene- loss CD 55, CD 59, lack glycoprotein glycosyl-phosphatidylinositol
complement mediated haemolysis
Budd chiari
aplastic anaemia
eculizumab- C5 inhbibitor
anticoagulation
stem cell transplant
acute thrombosis affect tests
protein C, protein S, antithrombin, and lupus anticoagulants can have falsely low
eculizumab
a monoclonal antibody directed against terminal protein C5 for PNH
HbF
2 alpha 2 gamma
HbA2
2 alpha 2 delta= beta thallacemia
venetoclax
BCL2 inhibitor
for unfit AML
AML unfavourable
t 6;9, t 11;23, T 9;22, inv 3, del 5q, del 7, del 17, FLT 3, mutated RUNX1, ASXL1, TP 53
FLT 3 is
TK receptor stimulat cell proliferation
AML induction
cytarabine, idarubicin
- cardiomyopathy, mucositis, cerebellar
AML consolidation
cytrabine, idarubicin, etoposide,
midostaurin
FLT 3 muitation positive AML
multitarget protein kinase inhibitor
cytopenia, LFT, rash, HTN
Ruxolitinib
JAK1&2 inhibitor
myelofibrosis
VSV
hydroxyurea
(ribonucleotide reductase) RR inhibitor
reduces intracellular deoxynucleotide triphosphat
What Haemoglinoopathy is protective in sickle cell anaemia -
HbF
G6PD
deficiency of NADPH , X linked
poikilocyte
pencel cell, FE deficiency
tear-drop
myelofibrosis
Schistocytes
FBC fragment- DIC
MDS prognostic
IPSS-R
cytogenetic
blast%
Hb
plt
Azacitidine
DNA hypomethylating agent (exact mechanism not clear)
50% respond high risk MDS
mastocytosis mutation
C-KIT— D816V, specific for systemic mastocytosis
Obinutuzumab + chlorambucil
CLL
anti CD 20 with enhanced antibody depeodnent cytotoxicity
idealisib
PI3 kinase inhibitior–
CLL
increase PJP, MCV
CLL < 65 treatment
fludarabine, cyclophos, ritux
ibrutinib
BTK inhibitor
CLL
AF, HTN, platelet dysfunction
Romiplostim
thrombopoietin recepotr agonist
bind to the TPO receptor (c-MpL) and stimulate megakaryocyte differentiation and proliferation
a recombinant, Fc-peptide fusion protein (peptibody) given subcutaneously,
eltrombopag
thrombopoietin recepotr agonist
bind to the TPO receptor (c-MpL) and stimulate megakaryocyte differentiation and proliferation
orally available drug that binds to the transmembrane region of c-MpL
ITP
aplastic anaemia
Unfractionated heparin
inhibits thrombin, factors Xa, IXa, XIa and XIIa
LMWH
increases the action of antithrombin III–> inhibit factor Xa
antibodies form against complexes of platelet factor 4 (PF4)
HIT
check heparin PF4 assay, heparin antibodies
fondaparinux
anti Xa
contraindicate eGFR <30
half-life 17 hr
bivalirudin
direct thrombin inhibitor
can use in liver, kidney (dose adjust)
continuous infusion
argatroban
direct thrombin inhibitor
continuous intravenous infusion, can use CKD. avoid in liver
Haemophilia A
Factor 8
X link- 30% spontaneous mutation
Emicizumab- mimiv factor 8- bind factor 9/10
desmopressin- increases plasma concentrations of coagulation factor VIII and von Willebrand factor
typical hus
shiga toxin E coli, HIV, pneumococcal
