heme/onc Flashcards
this is the most severe type of hemolytic reaction and is caused by ABO isoagglutinin
acute hemolytic
s/s of hemolytic transfusion rxn
fever, flank pain, red urine, rigors, tachy, DIC
dx of hemolytic transfusion rxn
+ coombs test
tx of hemolytic transfusion rxn
stop transfusion
IV fluids and mannitol
monitor for DIC
delayed hemolytic rxn cause
minor RBC antigen discrepancies
this is the MC transfusion rxn
febrile non-hemolytic transfusion rxn
s/s of febrile non-hemolytic transfusion rxn
fever, chills, rigors within 12 hrs
tx of febrile non hemolytic transfusion rxn
acetaminophen and benadryl
severe- IV corticosteroids
s/s of allergic transfusion rxns
bronchospasm, urticaria, pruritus, anaphylaxis, shock
tx of allergic transfusion rxn
stop
epi and glucocorticoids
s/s of graft vs host transfusion rxn
fever, rash, diarrhea, lymphadenopathy, hepatitis
SEVERE pancytopenia
usually fatal
prevention of graft vs host transfusion rxns
radiation of cellular components
TRALI s/s
non-cardiogenic pulmonary edema
TACO s/s
cardiogenic pulmonary edema
prevention of TRALI
male only plasma donors
dx of TACO
elevated BNP
tx of TACO
stop transfusion
diuretics and inotropes
what is essential thrombocythemia/ thrombocytosis?
increased platelets, but do not adhere properly
s/s of thrombocytosis
thrombosis in uncommon sites
erythromelaglia and calor
hemorrhage
dx of thrombocytosis
elevated platelet count
large platelets and megakaryocytes
genetic testing- JAK2, MPL, CALR
tx of thrombocytosis
oral hydroxyurea and aspirin
what is polycythemia vera?
overproduction of all 3 cell lines
list 2 things polycythemia vera is associated with
JAK2 mutation
PUD
s/s of polycythemia vera
pruritus
bone pain
decreased vision (retinal veins enlarge)
tinnitus
palpable spleen
dx of polycythemia vera
increased Hct
confirm- JAK2 mutation
tx of polycythemia vera
phlebotomy
what is hemochromatosis?
increased iron
s/s of hemochromatosis
hypogonadism
cirrhosis, HF
arthritis
bronze skin and DM1
iron labs of hemochromatosis
increased iron and ferritin
decreased TIBC
Tx of hemochromatosis
symptomatic–> phlebotomy
asymptomatic–> diet
what is myelodysplasia?
hematopoietic stem cell malignancy–> cytopenia and increased blasts
s/s of MDS
asymptomatic
s/s of pancytopenia (infection, pallor, fatigue, bleeding)
dx of MDS
defining- genetic testing
- hypercellular, left shift
-ringed sideroblasts
- dwarf megakaryocytes
<20% blasts
tx of MDS
bone marrow stem cell transplant is only cure
what is plasma cell/ multiple myeloma?
proliferation of plasma cells–> paraprotein production and bone destruction
s/s of plasma cell myeloma
C- high calcium
R- renal issues
A- anemia
B- bone issues
dx of plasma cell myeloma
paraprotein (M protein)
Rouleaux formation
Bence Jones- protein in urine
what is the cause of non-hodgkin’s lymphoma?
chromosomal translocation–> oncogene overexpression
s/s of non-hodgkin’s lymphoma
enlarged lymph nodes (peripheral or central)
weight loss >10%, fever, night sweats
Burkitt lymphoma- abd pain/ fullness
dx of non-hodgkin’s lymphoma
tissue or lymph node biopsy
tx of non-hodgkin’s lymphoma
indolent w/ 1 or 2- cure intent
aggressive- RCHOP
pathological hallmark of hodgkin’s lymphoma
Reed-Sternberg cells (owl eyes)
s/s of hodgkin’s lymphoma
painless mass (common in neck)
contiguous spreading
dx of hodgkin’s lymphoma
lymph node biopsy–> Reed Sternberg cells
tx of hodgkin’s lymphoma
ABVD (chemo)
s/s of ALL
pancytopenia
hepatosplenomegaly
lymphadenopathy
dx of ALL
mediastinal mass on CXR
bone marrow- >20% lymphoblasts
tx of ALL
chemo
daunorubicin, vincristine, prednisone, asparaginase
s/s of CML
fatigue, night sweats, fever
splenomegaly–> fullness
blurred vision, priapism
Dx of CML
philadelphia chromosome (9 and 22, bcr/ abl gene)
left shift
hypercellular marrow
tx of CML
tyrosine kinase inhibitors (imatinib)
AML presentation
pancytopenia
AML dx
auer rod
tx of AML
anthracycline + cytarabine
2 conditions that may progress to AML include…
MDS
CML
what cell type is affected by CLL
B lymphocytes
s/s of CLL
pancytopenia, lymphadenopathy, splenomegaly
dx of CLL
isolated lymphocytosis
small lymphocytes
smudge cells
tx of indolent/ early CLL
indolent/ early– observe
list 3 indications for tx of CLL
progressive fatigue
lymphadenopathy
anemia of thrombocytopenia
list tx of CLL if indications are present
BTK inhibitors w/ anti CD20
allogenic transplant
s/s of iron deficiency anemia
glossitis, koilonychia, pica, plummer vinson syndrome (esophageal webs)
lab studies in iron deficiency anemia
hypochromic microcytic
low iron and ferritin, increased TIBC
tx of iron deficiency anemia
iron supplementation
- oral ferrous sulfate w/ vit C
labs of anemia of chronic disease
micro to normo cytic
decreased TIBC, normal or increased ferritin
dx of anemia of chronic disease
bone marrow biopsy with iron stain
tx of anemia of chronic disease
tx chronic condition + EPO + iron supplements
what is aplastic anemia?
pancytopenia due to bone marrow failure
s/s of aplastic anemia
pancytopenia
- anemia
- infections
- bleeding
labs of aplastic anemia
pancytopenia and reticulocytopenia
dx of aplastic anemia
hypocellular bone marrow biopsy
tx of aplastic anemia
mild- erythropoietic or myeloid GFs
severe-
- <40: bone marrow transplant
- >40: immunosuppressiona dn hematopoietic agent
what is sideroblastic anemia?
decreased Hb synthesis due to impaired ability to incorporate iron into Hb
s/s of sideroblastic anemia
anemia (pallor, SOB, fatigue)
labs of sideroblastic anemia
increased iron and ferritin, decreased TIBC
low retic count
decreased B6
ringed siderob lasts
dimorphic cell population
tx of sideroblastic anemia
oral pyridoxine (B6)
s/s of vitamin B12 (cobalamin) def
paresthesias, balance, proprioception, glossitis, anorexia, diarrhea, dementia
s/s of anemia
labs of B12 def
low B12
elevated MMA and homocysteine
low retic count
macrocytic RBCs
hypersegmented neutrophils
tx of B12 def
IM B12 injection
s/s of folate def (B9)
no neuro symptoms
glossitis, anorexia, diarrhea, pallor, tachy
labs of folate def
decreased folate
elevated homocysteine and normal MMA
tx of folate def
oral folic acid
s/s of hemolytic anemia
jaundice, icterus, dark urine
splenomegaly
anemia s/s
labs of hemolytic anemia
increased retic count
increased LDH and bilirubin
list 3 subtypes of hemolytic anemia
autoimmune
hereditary spherocytosis
G6PD
+ spherocytes and + coombs test is what type of hemolytic anemia
autoimmune hemolytic anemia
+ spherocytes and - coombs test, hyperchromic microcytosis
+ osmotic fragility test
hereditary spherocytosis
bite cells and Heinz bodies suggest what type of hemolytic anemia
G6PD
list 3 triggers for G6PD hemolytic anemia
infection
meds
fava beans
tx of G6PD hemolytic anemia
avoid oxidant meds/ triggers
tx of hereditary spherocytosis
folic acid
splenectomy
tx of autoimmune hemolytic anemia
steroids
HUS triad
thrombocytopenia
hemolytic anemia
renal dysfunction
dx of HUS
thrombocytopenia with normal coag studies and increased bleeding time
normal ADAMTS13
schistocytes, reticulocytosis, increased LDH and bilirubin
increased BUN and creatinine
tx of HUS
supportive
what causes TTP?
destruction of ADAMTS13
pentad of TTP
thrombocytopenia
hemolytic anemia
neuro
fever
kidney failure
dx of TTP
schistocytes, increased LDH, bilirubin
decreased ADAMTS13 and antibodies
tx of TTP
plasmaphoresis
glucocorticoids
immunosuppression
cause of ITP
autoimmune destruction of platelets
s/s of ITP
asymptomatic
purpura, petechiae, gum bleeding
severe bleeding
NO splenomegaly
dx of ITP
isolated thrombocytopenia
increased megakaryocytes
tx of ITP
kids- observation
platelets low- glucocorticoids, IVIG, anti-D
factor V Leiden disorder cause
mutated factor V–> resistant to breakdown via protein C
hypercoaguability
s/s of factor V Leiden disorder
VTE- DVT, PE
dx of factor V leiden
activated protein C resistance assay
if +–> confirm with DNA testing
tx of factor V Leiden
asymptomatic- not treated
moderate- prophylaxis for procedures
high risk- warfarain indefinitely
what type of genetic disorder is hemophilia?
x linked recessive
A- VIII
B- IX
C- XI
s/s of hemophilia
deep tissue bleeding/ hemarthrosis
dx of hemophilia
increased PTT
decreased factor
tx of hemophilia
factor infusion
A- DDAVP and emicizumab
what causes Von Willebrand’s disease and what type of genetic disease is it?
ineffective platelet adhesion
autosomal dominant
s/s of vWD
prolonged bleeding
Dx of vWD
increased PTT and bleeding time
normal platelets and PT
diagnostic- decreased vWF antigen
ristocetin induced platelet aggregation (RIPA)
tx of vWD
DDAVP
severe- vWF containing product
timeline for heparin induced thrombocytopenia
5-14 days after exposure
heparin induced thrombocytopenia s/s
thrombocytopenia and thrombosis
often asymptomatic
dx of heparin induced thrombocytopenia
initial- PF4 heparin antibody ELISA
confirm- functional assay
tx of heparin induced thrombocytopenia
discontinue heparin and start different anticoag
cause of DIC
coagulation and fibrinolysis
s/s of DIC
bleeding- IV and catheter sites, ecchymosis, purpura
thrombosis- gangrene, cyanosis
labs for DIC
decreased platelets, increased bleeding time, PT and PTT
decreased fibrinogen
increased D dimer
schistocytes
tx of DIC
tx underlying cause
severe- platelets, FFP
