Hematology and Oncology Flashcards
which platelet granules contain ADP and calcium
dense granules
which platelet granules contain vWF, fibrinogen, and fibronectin
alpha granules
which neutrophil granules contain LAP, collagenase, lysozyme, and lacteferrin
specific granules
which neutrophil granules contain proteinases, acid phosphatase, MPO, and beta-glucuronidase
azurophilic granules
what receptor on macrophages binds LPS lipid A
CD14
MOA cromolyn sodium
prevents mast cell degranulation, for asthma prophylaxis
components of Hb A
2 alpha, 2 beta
components of Hb A2
2 alpha, 2 delta
elevated in beta thal minor
components of fetal hemoglobin
2 alpha, 2 gamma
elevated in beta thal major
components of Hb H
4 betas, seen in 3 alpha deletions
components of Hb Barts
4 gamma, seen in 4 alpha deletions which causes fatal hydrops fetalis
which hemoglobin moves the farthest on electrophoresis, and which are behind
A goes the farthest, then F, then S, then C
which factors are inactivated by warfarin
II, VII, IX, X, C, S
protein C cleaves
V, VIII
acanthocyte (spur cell) assoc dz
liver disease, abetalipoproteinemia
degmacyte (bite cell) dz assoc
G6PD
echinocyte (burr cell) dz assoc
ESRD, liver disease, pyruvate kinase deficiency
note projections are more uniform and smaller than those of the spur cell
spherocyte dz assoc
hereditary spherocytosis, drug- and infection-induced hemolytic anemia
target cell dz assoc
HbC disease, asplenia, liver disease, thalassemia
“‘HALT,’ said the hunter to his target”
Heinz body dz assoc
G6PD deficiency. precursor the the bite cell
Howell-Jolly bodies dz assoc
aslpenia
microcytic anemias
late iron deficiency, late ACD, thalassemias, lead poisoning, sideroblastic anemia
normocytic, nonhemolytic anemias
early iron deficiency, early ACD, aplastic anemia, CKD
normocytic, intrinsic hemolytic anemias
RBC membrane defects (e.g. HS), enzyme defects (e.g. G6PD and PK), HbC disease, sickle cell, PNH
normocytic extrinsic hemolytic anemias
autoimmune, microangiopathic, macroangiopathic, infections
megaloblastic anemias
folate deficiency, B12 deficiency, orotic aciduria
macrocytic, non-megaloblastic anemias
liver disease, alcoholism, Diamond-Blackfan anemia
Burton lines
lead lines on gingivae
deficiency sideroblastic anemia
XR in delta-ALA synthase, which is first step and RLS (occurs in mitochondria)
treatment sideroblastic anemia
pyridoxine/B6, a cofactor for delta-ALA synthase
which drugs can cause folate deficiency
methotrexate, TMP, and phenytoin
defect in orotic aciduria
UMP synthase deficiency
if with hyperammonemia, it is ornithin transcarbamylase deficiency
treatment orotic aciduria
UMP to bypass UMP synthase deficiency
defect in paroxysmal noturnal hemoglobinuria
defect in CD55 (decay-accelerating factor) and/or CD 59 (MAC-inhibitory protein)
triad of sx in PNH
coombs-negative hemolytic anemia, pancytopenia, venous thrombosis
tx PNH
eculizumab
which dz cause cold agglutinin
mycoplasma and mononucleosis
threshold neutropenia
less than 1500
threshold lymphopenia
less than 1500 (in kids less than 3000)
threshold eosinopenia
less than 30
results of steroids on blood levels of eosinophils, lymphocytes, and neutrophils
neutrophilia (high)
low eos and lymphs
name the intermediates in heme synthesis
glycine + succinyl-CoA > delta ALA > porphobilinogen > hydroxymethylbilane > uroporphyrinogen III > coproporphyrinogen III > protoporphyrin > heme
enzyme def and location for sideroblastic anemia
delta ALA synthase in mitochondria
note this is an XR disease
enzyme def and location for lead poisoning
1) d-ALA dehydratase in cytoplasm
2) ferrocheletase in mitochondria
enzyme def and location for acute intermittent porphyria
porphobilinogen deaminase in cytoplasm
enzyme def and location for porphyria cutanea tarda
uroporphyrinogen decarboxylase in cytoplasm
what accumulates in lead poisoning
protoporphyrins and delta-ALA
what accumulates in AIP
porphobilinogen, delta-ALA, coporphobilinogen (in the urine. != coproporphyrinogen III)
what accumulates in porphyria cutanea tarda
uroporphyrin
blistering cutaneous photosensitivity is a sx of which porphyria
PCT
abdominal pain, polyneuropathy, port-wine colored urine and psychological distrubances is a sx of which porphyria
AIP
inheritance for each hemophilia
A XR 8
B XR 9
C AR 11
MOA desmopressin for blood disorders and use
increases vWF release from platelets. good for vWD and hemophilia A
note: not effective for hemophilia B
acquired cause of antithrombin III deficiency
proteinuira
cytology for reed sternberg cells
CD15+, CD30+
translocation and gene Burkitt
8;14 c-myc, heavy ig
translocation and gene follicular lymphoma
14;18 heavy ig, bcl-2
translocation and gene mantle cell lymphoma
11;14 cyclin d1, heavy Ig
translocation and gene CML
9;22. bcr-abl (abl is on 9) also seen in some ALL
translocation and gene APL
15;17 PML-RARA
translocation ALL with better prognosis
12;21
T cell type mycosis fungoides
CD4
cytology ALL
TDT+ (pre-T and pre-B), CD10+ (pre-B)
cytology CLL
CD20+, CD5+
which leukemia/lymphoma is TRAP+
hair cell leukemia
CML has (high/low) LAP
very low
benign reactive neutrophlia has (high/low) LAP
high
translocation and gene Ewing sarcoma
11;22 EWS-FLI 1
presentations langerhans cell histiocytosis
lytic bone lesions and skin rash
or, recurrent otitis media with mastoid mass
cytology langerhans cell histiocytosis
S100+, CD1a+
Birbeck granules
tennis racket-shaped granules seen on EM in langerhans cell histiocytosis
what do you use to monitor warfarin
PT
what do you use to monitor heparin
PTT
class of drugs: enoxaparin, dalteparin
LMWH
class of drugs: bivalirudin
direct thrombin inhibitor
class of drugs: cilostazol, dipyridamole
PDE III inhibitors
MOA PDE III inhibitors
cause increase in platelet cAMP, inhibiting aggregation and causing vasodilation. used in angina prophylaxis
MOA eptifibatide, tirofiban
GP-IIb/IIIa inhibitors
cell cycle independent chemo
platinum agents (e.g. cisplatin) and alkylating agents (busulfan, cyclophos, ifosfamide, nitrosoureas)
MOA cladribine
purine analog antimetabolite. causes nephrotoxicity and neurotoxicity
MOA cytarabine (arabinofuranosyl cytidine)
pyrimidne analog antimetabolite
effect of leucovorin on 5FU AEs
worsens myelosupression
effect of leucovorin on methotrexate AEs
reverses myelosuppression
etoposide, teniposide are for topo [I or II]
II
irinotecan, topotecan are for topo [I or II]
I
MOA erlotinib
EGFR TKI for non-small cell lung carcinoma
MOA cetuximab
mAb against EGFR
used for stage IV CRC (wildtype KRAS)
administer this compound to reduce doxorubicin cardiotox
dexrazoxane
use this to mitigate cisplatin-induced neprhotoxicity
amifostine, a scavenger of free radicals