Heme/Onc Flashcards
residual ribosomal RNA
bluish color on Wright Giemsa
reticulocytes
contents dense granules
in thrombocyte
ADP and Ca
contents alpha granules leukocytes
vWF, fibronogen, fibronectin
granules in neutrophil
leukocyte alkaline phosphatase
collagenase
lysozyme and lactoferrin
neutrophil chemotactic
c5a, IL8, kallikrein, LTB4, platelet activating factor
activation macrophages
IFN gamma
initiation of septic shock
lipid A from LPS binds CD14 on macs
causes of eosinophilia
neoplasia asthma allergic processes chronic adrenal insufficiency parasites
products eosinophils
histamine, major basic protein, eosinophil peroxidase, eosinophil cationic protein
basophilia
CML
prevention mast cell degranulation
cromolyn
costim for T cells
CD28
fetal erythropoiesis
yolk sac 3-8 weeks liver 6-birth spleen 10-28 weeks bone marrow Young liver synthesizes blood
higher O2 binding fetal Hb
less avid binding of 2,3BPG
ABO hemolytic disease
O mother with A or B fetus
mild jaundice in neonate within 24 hrs of birth
Hb on electrophoresis
A fat santa Claus
A travels furthest
VWF and coag
protects and carries factor 8
factor V leiden
mutation produces factor V resistant to inhibition by protein C
antithrombin inhibition
2,7,9,10,11,12
protein C
inactivates 5a and 8a
induction Gp2b/3a
ADP binding P2Y12
Ristocetin
activates vWF to bind Gp1b
failure occurs in vWF disease and Bernard-Soulier syndrome
causes acanthocyte
liver disease, abetalipoproteinemia
burr cell
end stage renal disease, liver disease, pyruvate kinase deficiency
more uniform than acanthocyte
causes target cell
HbC, asplenia, liver disease, thalassemia
deletions alpha thalassemia
cis worse-Asian
trans-African
Hb Barts
found in 4 deletions
incompatible with life
HbH
found with 3 deletions
excess beta globulin formation B4
cause of beta thalassemia
mutations in splice sites and promoter sequences
labs b thal minor
increase A2
risk for b thal major
B19
labs b thal major
HbF
lead inhibition
ferrochelatase and ALAD
symptoms lead poisoning
lead lines
wrist and foot drop
treatment lead poisoning
EDTA and dimercaprol
succimer in children
genetic sideroblastic anemia
defect in ALAS
could also be B6 deficit
B12 vs folate
increase both lab findings and neuro in B12
tracts B12 def
lateral corticospinal tract, spinocerebellar, dorsal column
orotic aciduria
inability to convert orotic acid to UMP due to defective UMP synthase
AR
treatment orotic aciduria
uridine monophosphate
diff orotic aciduria and ornithine transcarbamylase
no hyperammonemia in orotic aciduria
cause Diamond-Blackfan anemia
defect in erythroid progenitor cells
increase %HbF
labs intravascular hemolysis
decrease haptoglobin
hemoglobinuria, hemosiderinuria and urobilinogen in urine
etiology anemia of chronic disease
increase hepcidin inhibits iron transport
give EPO in CKD
cause Fanconi anemia
DNA repair defect causing bone marrow failure
short stature, increase tumors/leukemias, cafe-au-lait spots, thumb/radial defects
pyruvate kinase deficiency
AR
rigid RBCs, increase 23BPG and decreased affinity for O2
leads to hemolytic anemia in newborn
cause PNH
GPI anchor for DAF
leads to increased leukemias
labs PNH
CD55/59-
complications sickle
painful crises, acute chest syndrome, avascular necrosis, renal papillary necrosis and microhematuria (medullary infarcts)
smear HbC
crystals inside RBCs, target cells
from lysine mutation in beta globin
warm agglutination
SLE and CLL
IgG mediated
also alpha-methyldopa
cold agglutination
CLL, mycoplasma, mono
IgM mediated
Direct Coombs
anti-Ig Ab to patient RBC
Indirect Coombs
normal RBC to patient serum
lab diff iron def and chronic disease anemia
decreased transferrin in chronic disease
increase ferritin in chronic disease
defective enzyme acute intermittent porphyria
porphobilinogen deaminase leads to accumulation of ALA and porphobilinogen
AD
symptoms acute intermittent porphyria
painful abdomen port wine colored urine polyneuropathy psychological distubrances precipitated by drugs-p450 inducers, alcohol, starvation
treatment acute intermittent porphyria
glucose and heme
defective enzyme porphyria cutanea tarda
uroporphyrinogen decarboxylase AD
leads to increase uroporphyrin
symptoms porphyria cutanea tarda
blistering cutaneous photosensitivity and hyperpigmentation
exacerbated by alcohol
mechanism iron poisoning
cell death due to peroxidation of membrane lipids
coagulation studies
factor inhibitors will not correct
hemophilia C
AR
defective 11
defect bernard-soulier
defective platelet plug formation
decrease Gp1b
defect Glanzmann thrombasthenia
defect in Gp2b3a
blood smear shows no platelet to platelet clumping
labs ITP
large megs
Ab to Gp2b3a
labs TTP
increase LDH
normal coag
increase BT
causes DIC
sepsis (gram-), trauma, obstetric complications, acute pancreatitis, malignancy, nephrotic syndrome
anti-thrombin deficiency
diminishes increase in PTT following heparin admin
can be acquired from renal failure-lost in urine leading to decreased inhibition of 2a/10a
protein C or S def
decreased ability to inactivate 5a/8a
protnrhombin mutation
in 3’ UTR leads to increased production
leads to venous clots
contents cryoprecipitate
fibrinogen, factor 8,13, vWF, fibronectin
give for factor deficiencies
blood transfusion risks
iron overload
hypocalcemia (due to citrate binding Ca)
hyperkalemia
associations non-hodgkin lymphoma
HIV and autoimmune
cell markers hodgkin lymphoma
15, 30
genetics diffuse large B cell lymphoma
bcl2, bcl6
presentation follicular lymphoma
waxing and waning
genetics and association marginal zone lymphoma
11;18
associated with chronic inflammation-Sjogren and chronic gastritis
primary central nervous system lymphoma
associated with HIV/AIDS
pathogenesis involves EBV
needs to be distinguished from toxo
ault T cell lymphoma
caused by HTLV (IV drug association)
cutaneous lesions, lytic bone lesions and hypercalcemia
M spike
Waldenstrom macroglobulinemia
Pseudo-Pegler-Huet anomaly
neutrophils with bilobed nuclei
seen after chemo
ALL
children, Down syndrome
TdT and CD10+
11;21
spread ALL
CNS and testes
CLL
20,23,5+
smudge cells
Richter transformation
CLL into aggressive lymphoma-diffuse large B cell lymphoma
Hairy cell leukemia
mature B cell tumor
marrow fibrosis, massive splenomegaly
stains TRAP
treatment hairy cell leukemia
cladribine, pentostatin
transformation CML
to AML or ALL low LAP (diff from leukemoid reaction)
itching after hot shower
polycythemia vera
decrease EPO
presentation essential thrombocythemia
massive megs and platelets, symptoms of bleeding and thrombosis
cause relative polycythemia
decrease plasma volume from dehydration and burns
presentation langerhans cell histiocytosis
lytic bone lesions and skin rash
recurrent otitis media
cell markers langerhans cell histiocytes
S100 (mesodermal) and CD1a
HIT
IgG ab against heparin platelet factor 4
reverse dabigatran
idarucizumab
adverse cytarabine
pancytopenia
MOA 5FU
bioactivated by 5-FdUMP and complexes with thymidylate synthase and folic acid
inhibits thymidylate synthase
leucovorin
increases effects 5FU
busulfan vs bleomycin
minimal BM suppression with bleo
MOA cyclophosphamide
crosslink at guanine N7
requires activation by liver
MOA mesna
binds toxic metabolites
bevacizumab
inhibits VEGF
erlotinib
inhibits EGFR tyrosine kinase for non-small cell lung cancer
cetuximab
ab against EGFR
used for colorectal, head and neck cancer
can elevated LFTs
adverse rituximab
increase risk of PML
bortezomib, carfilzomib
protease inhibitors
arrest G2-M
can be used for MM and mantle cell lymphoma
can reactivate herpes, cause peripheral neuropathy
raloxifene
no risk of endometrial carcinoma
vemurafenib
inhibits BRAF in V600E melanoma
rasburicase
recombinant uricase that catalyzes uric acid to allantoin
prevention and treatment of tumor lysis syndrome
