Heme/Onc SBSI Flashcards
complications of hemophilia
arthropathy, spontaneous intracerebral, renal, retroperitoneal, and GI hemorrhages
tx of severe hemophilia
immediate transfusion of missing factor or cryoprecipitate
initial test for hemophilia
mixing study, PTT corrects after mixing
what is prolonged in hemophilia?
PTT
what does cryoprecipitate consist of?
mainly Factor VIII and fibrinogen
tx of mild hemophilia
desmopressin, releases factor VIII from endothelial cells
MC inherited bleeding disorder?
von willebrand disease
child with prolonged or recurrent mucosal bleeding and bleeding after dental or sx (epistaxis or menorrhagia)
positive family hx
von willebrand disease
initial test for von willebrand disease
increased BT and increased PTT
PT and plts are normal
most accurate test for vWD
ristocetin cofactor assay of pt plasma
mild to moderate vWD tx
desmopressin
what will worsen vWD?
ASA, NSAIDs, plt function inhibitors
acute neutropenia, what bugs are more likely?
S. aureus, Pseudomonas, E. coli, proteus, and klebsiella
best initial test for neutropenia
CBC with smear
after looking at drugs they are on
if decreased platelets and anemia with neutropenia, what should you get
bone marrow biopsy and/or aspirate
neutropenic fever, tx?
emergency
broad spec abx, cefepime
tx suspected fungal as well
what can be given to shorten duration of neutropenia?
G-CSF
What cytokines overproduced can cause eosinophilia?
IL-3, IL-5, GM-CSF
or by chemokines
dx with eosinophilia starts with
CBC with diff
CSF showing eosinophilia is suggestive of
drug reaction or infxn with coccidio or helminth
hematuria with eosinophilia
schistosomiasis
tx of new onset cardiac findings, eosinophilia or drug rxn
steroids
transplantation between identical twins
syngenic transplantation
complication of allogenic bone marrow transplantation in which donated T cells attack host tissues, especially skin, liver, and GI tract
graft v host
skin changes, cholestatic liver dysfunction, obstructive lung disease, or GI problems after transplant
graft v host
transplantation, 5 days to 3 months, increase GGT, alk phos, LDH, BUN, or Cr
acute rejection
vascular thrombi or tissue ischemia after transplant
hyperacute rejection
young, white pt, <45 yo with personal and family hx of thrombosis
Factor V Leiden
hyperhomocysteinemia
MTHFR gene mutation
test for factor V Leiden
activated protein C resistance test
test for HIT
platelet factor 4 antibody or serotonin release assay
tx for factor V Leiden
warfarin for 6 months, avoid OCPs
tx of HIT
d/c heparin. start direct thrombin inhibitor (fondaparinux, argatroban, bivalirudin)
followed by warfarin
tx antiphospholipid antibody syndrome
give heparin and warfarin
what is contraindicated in ITP and TTP
platelets
deficiency of vWF-cleaving enzyme (ADAMTS-13)
TTP
suspect TTP if 3/5 of these are present
Fever
Anemia
thrombocytopenia
renal issue
neuro symptoms
tx for TTP
plasma exchange
lab major difference TTP and HUS
HUS has much higher Cr
IgG antibodies formed against platelets, and then complex is destroyed by the spleen
ITP
abrupt onset of hemorrhagic complications following a viral illness with sudden, self-limiting purpura
ITP
tx of ITP, plts >30k and no bleeding
no tx
tx ITP, plt <30k or clinically significant bleeding
corticosteroids or IVIG
tx ITP with failed prior tx
splenectomy + rituximab + TPO receptor agonist
bone marrow production of platelets is increased with increased megakaryocytes in the marrow
ITP
glossitis, conjunctival pallor, cheilosis, koilonychia
fe deficiency anemia
initial test of Fe deficiency anemia will show
decreased MCV, decreased MCH, decreased MCHC
decreased ferritin
increased RDW
increased TIBC
decreased serum Fe
tx of Fe deficiency anemia
replace Fe orally until normal and for at least 4-6 months
IM Fe if oral not working
IV Fe for people with malabsorption issues
what will be labs in anemia of chronic disease?
ferritin increased
decreased serum Fe
decreased TIBC
tx of anemia associated with ESRD
epo
hx of chloramphenicol, INH, alcohol use, exposure to lead, collagen vascular disease and some myelodysplastic syndromes can cause
sideroblastic anemia
tx of sideroblastic anemia?
remove offending agent, transfuse as necessary, may also consider pyroxidine
neruopathy and subacute combined degeneration of the spinal cord
vit B12 deficiency
inability to generate glutathione reductase
G6PD deficiency
CD55 and 59 inhibited attachment
PNH
tx for PNH
prednisone is best initial therapy
allogenic bone marrow transplant is curative
eculizumab for hemolysis and thrombosis
extravascular hemolytic anemia with splenomegaly and jaundice
hereditary spherocytosis
best test for spherocytosis
eosin- 5- maleimide flow cytometry
or acidified glycerol lysis test
tx for hereditary spherocytosis
splenectomy and chronic folic acid replacement
pancytopenia on labs, pallor, fatigue, weakness, tendency to infxn, petechiae, bruising and bleeding
aplastic anemia
drugs that can cause aplastic anemia
PTU, methimazole, carbamazepine
only form of hereditary aplastic anemia
Fanconi anemia
severe case of aplastic anemia but can’t do stem cell transplant, tx?
cyclosporine (or tacrolimus), antithymocyte globulin (ATG), eltrombopag
PCV can convert to
AML in small portion of pts
why would PCV cause pruritis after hot bath?
increased histamine release
what happens to EPO in PCV?
EPO is decreased
tx for polycythemias
phlebotomy
ASA if they have thrombosis
allopurinol/rasburicase prevents an increase in uric acid
hydroxyurea reduces cell counts
antibody response against donor plasma proteins causing urticaria, pruritis, wheezing, fever
allergic reaction
cytokine formation during storage of blood
host antibodies against the donor HLA antigens and WBCs
Type II hypersensitivity reaction
febrile nonhemolytic reaction
fever, HA, chills, flushing, rigors, and malaise 1-6 hours after transfusion
febrile nonhemolytic reaction
tx of febrile nonhemolytic transfusion reaction
stop transfusion, give acetaminophen
leukoreduction of donor blood
DIC picture s/p transfusion
hemolytic transfusion reaction
type II hypersensitivity
AML and ALL will have signs of
pancytopenia and RAPID onset and progression
DIC can be seen in what leukemia?
APML
Auer rods will be found in
AML
WBC is typically low in AML/ALL, if it very high, this is a risk of
leukostasis -> DIC
tx of AML or ALL
chemo, or BMT if not responsive
how might you prevent Tumor Lysis syndrome
hydration and rasburicase to decrease uric acid level as renal protection
MC childhood malignancy
ALL
Down syndrome is associated with what leukemia?
ALL
t(15:17)
APML
tx for APL
all trans retinoic acid
what do you use to reverse factor Xa inhibitors?
andexanet alfa
reverse warfarin with
activated prothrombin complex concentrate
how do you tx tPa toxicity
aminocaproic acid
malignant clonal proliferation of functionally incompetent lymphocytes
CLL
cells that have a large nucleus, immature chromatin, a prominent nucleolus, scant cytoplasm and few or no cytoplasmic granules is
a blast cell
seen in acute leukemias
blast cell that is myeloperoxidase positive
myeloblast
Terminal deoxynucleotidyltransferase (TdT) positive
lymphoblast
antidote/reversal to dabigatran
idarucizumab
malignancies that go with tuberous sclerosis
astrocytoma and cardiac rhabdomyoma
clonal proliferation of functionally incompetent lymphocytes that accumulate in the BM, peripheral blood, LN, spleen, and liver
CLL
fatigue, malaise, infxn, LAD, hepatomegaly, and splenomegaly in an older individual
CLL
initial tx of CLL
fludarabine and chlormabucil
palliative and withheld until symptomatic
flow cytometry in CLL shows
CD5 marker on B cells
splenomegaly, LUQ pain, early satiety, fatigue, malaise
CML
basophilia
CML
labs elevated in CML
WBC >100k, granulocytes in all stages of maturity
LDH, uric acid, and B12
CML chromosome abnl
philadelphia chr. t(9:22)
tx of CML
tyrosine kinase inhibitors (imatinib)
tx of blast crisis in CML
2nd gen tyrosine kinase inhibitors
(dasatinib, nilotinib)
and hemopoietic stem cell transplantation
well differentiated B lymphocyte disorder
hairy cell leukemia
initial tx of hairy cell leukemia
cladribine
labs of tumor lysis syndrome
hyperK, hyperuricemia, hyperphosphatemia, hypocalcemia
painless wax and wane of adenopathy
follicular lymphoma (B cell)
MC NHL in adults, single rapidly growing mass, high cure rate with R CHOP
diffuse large B cell lymphoma
elderly male, CD5+ cells, NHL
mantle cell lymphoma
high grade neoplasm that can progress to ALL, cutaneous lesions
caused by HTLV and a/w IVDA
Adult T cell lymphoma
cutaneous eczema like lesions and pruritis with ‘cerebriform’ lymphoid cellsmy
mycosis fungioides and can lead to Sezary syndrome
pt has long standing inflammatory disease/ or infection and is presenting with kidney, liver, and GI involvement
amyloidosis
if amyloidosis progresses rapidly, tx with
corticosteroids, melphalan
chemo type when standard chemo fails
salvage
chemo type that is given in addition to standard therapy
adjuvant
chemo type that is given before the standard therapy
neoadjuvant
chemo type that is the initial dose of treatment
induction
type of chemo given after induction therapy to reduce the tumor burden
consolidation
type of chemo you give after induction and consolidation
maintenance
