heme/onc Flashcards

1
Q

this is the most severe type of hemolytic reaction and is caused by ABO isoagglutinin

A

acute hemolytic

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2
Q

s/s of hemolytic transfusion rxn

A

fever, flank pain, red urine, rigors, tachy, DIC

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3
Q

dx of hemolytic transfusion rxn

A

+ coombs test

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4
Q

tx of hemolytic transfusion rxn

A

stop transfusion
IV fluids and mannitol
monitor for DIC

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5
Q

delayed hemolytic rxn cause

A

minor RBC antigen discrepancies

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6
Q

this is the MC transfusion rxn

A

febrile non-hemolytic transfusion rxn

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7
Q

s/s of febrile non-hemolytic transfusion rxn

A

fever, chills, rigors within 12 hrs

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8
Q

tx of febrile non hemolytic transfusion rxn

A

acetaminophen and benadryl
severe- IV corticosteroids

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9
Q

s/s of allergic transfusion rxns

A

bronchospasm, urticaria, pruritus, anaphylaxis, shock

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10
Q

tx of allergic transfusion rxn

A

stop
epi and glucocorticoids

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11
Q

s/s of graft vs host transfusion rxn

A

fever, rash, diarrhea, lymphadenopathy, hepatitis

SEVERE pancytopenia
usually fatal

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12
Q

prevention of graft vs host transfusion rxns

A

radiation of cellular components

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13
Q

TRALI s/s

A

non-cardiogenic pulmonary edema

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14
Q

TACO s/s

A

cardiogenic pulmonary edema

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15
Q

prevention of TRALI

A

male only plasma donors

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16
Q

dx of TACO

A

elevated BNP

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17
Q

tx of TACO

A

stop transfusion
diuretics and inotropes

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18
Q

what is essential thrombocythemia/ thrombocytosis?

A

increased platelets, but do not adhere properly

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19
Q

s/s of thrombocytosis

A

thrombosis in uncommon sites
erythromelaglia and calor
hemorrhage

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20
Q

dx of thrombocytosis

A

elevated platelet count
large platelets and megakaryocytes
genetic testing- JAK2, MPL, CALR

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21
Q

tx of thrombocytosis

A

oral hydroxyurea and aspirin

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22
Q

what is polycythemia vera?

A

overproduction of all 3 cell lines

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23
Q

list 2 things polycythemia vera is associated with

A

JAK2 mutation
PUD

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24
Q

s/s of polycythemia vera

A

pruritus
bone pain
decreased vision (retinal veins enlarge)
tinnitus
palpable spleen

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25
dx of polycythemia vera
increased Hct confirm- JAK2 mutation
26
tx of polycythemia vera
phlebotomy
27
what is hemochromatosis?
increased iron
28
s/s of hemochromatosis
hypogonadism cirrhosis, HF arthritis bronze skin and DM1
29
iron labs of hemochromatosis
increased iron and ferritin decreased TIBC
30
Tx of hemochromatosis
symptomatic--> phlebotomy asymptomatic--> diet
31
what is myelodysplasia?
hematopoietic stem cell malignancy--> cytopenia and increased blasts
32
s/s of MDS
asymptomatic s/s of pancytopenia (infection, pallor, fatigue, bleeding)
33
dx of MDS
defining- genetic testing - hypercellular, left shift -ringed sideroblasts - dwarf megakaryocytes <20% blasts
34
tx of MDS
bone marrow stem cell transplant is only cure
35
what is plasma cell/ multiple myeloma?
proliferation of plasma cells--> paraprotein production and bone destruction
36
s/s of plasma cell myeloma
C- high calcium R- renal issues A- anemia B- bone issues
37
dx of plasma cell myeloma
paraprotein (M protein) Rouleaux formation Bence Jones- protein in urine
38
what is the cause of non-hodgkin's lymphoma?
chromosomal translocation--> oncogene overexpression
39
s/s of non-hodgkin's lymphoma
enlarged lymph nodes (peripheral or central) weight loss >10%, fever, night sweats Burkitt lymphoma- abd pain/ fullness
40
dx of non-hodgkin's lymphoma
tissue or lymph node biopsy
41
tx of non-hodgkin's lymphoma
indolent w/ 1 or 2- cure intent aggressive- RCHOP
42
pathological hallmark of hodgkin's lymphoma
Reed-Sternberg cells (owl eyes)
43
s/s of hodgkin's lymphoma
painless mass (common in neck) contiguous spreading
44
dx of hodgkin's lymphoma
lymph node biopsy--> Reed Sternberg cells
45
tx of hodgkin's lymphoma
ABVD (chemo)
46
s/s of ALL
pancytopenia hepatosplenomegaly lymphadenopathy
47
dx of ALL
mediastinal mass on CXR bone marrow- >20% lymphoblasts
48
tx of ALL
chemo daunorubicin, vincristine, prednisone, asparaginase
49
s/s of CML
fatigue, night sweats, fever splenomegaly--> fullness blurred vision, priapism
50
Dx of CML
philadelphia chromosome (9 and 22, bcr/ abl gene) left shift hypercellular marrow
51
tx of CML
tyrosine kinase inhibitors (imatinib)
52
AML presentation
pancytopenia
53
AML dx
auer rod
54
tx of AML
anthracycline + cytarabine
55
2 conditions that may progress to AML include...
MDS CML
56
what cell type is affected by CLL
B lymphocytes
57
s/s of CLL
pancytopenia, lymphadenopathy, splenomegaly
58
dx of CLL
isolated lymphocytosis small lymphocytes smudge cells
59
tx of indolent/ early CLL
indolent/ early-- observe
60
list 3 indications for tx of CLL
progressive fatigue lymphadenopathy anemia of thrombocytopenia
61
list tx of CLL if indications are present
BTK inhibitors w/ anti CD20 allogenic transplant
62
s/s of iron deficiency anemia
glossitis, koilonychia, pica, plummer vinson syndrome (esophageal webs)
63
lab studies in iron deficiency anemia
hypochromic microcytic low iron and ferritin, increased TIBC
64
tx of iron deficiency anemia
iron supplementation - oral ferrous sulfate w/ vit C
65
labs of anemia of chronic disease
micro to normo cytic decreased TIBC, normal or increased ferritin
66
dx of anemia of chronic disease
bone marrow biopsy with iron stain
67
tx of anemia of chronic disease
tx chronic condition + EPO + iron supplements
68
what is aplastic anemia?
pancytopenia due to bone marrow failure
69
s/s of aplastic anemia
pancytopenia - anemia - infections - bleeding
70
labs of aplastic anemia
pancytopenia and reticulocytopenia
71
dx of aplastic anemia
hypocellular bone marrow biopsy
72
tx of aplastic anemia
mild- erythropoietic or myeloid GFs severe- - <40: bone marrow transplant - >40: immunosuppressiona dn hematopoietic agent
73
what is sideroblastic anemia?
decreased Hb synthesis due to impaired ability to incorporate iron into Hb
74
s/s of sideroblastic anemia
anemia (pallor, SOB, fatigue)
75
labs of sideroblastic anemia
increased iron and ferritin, decreased TIBC low retic count decreased B6 ringed siderob lasts dimorphic cell population
76
tx of sideroblastic anemia
oral pyridoxine (B6)
77
s/s of vitamin B12 (cobalamin) def
paresthesias, balance, proprioception, glossitis, anorexia, diarrhea, dementia s/s of anemia
78
labs of B12 def
low B12 elevated MMA and homocysteine low retic count macrocytic RBCs hypersegmented neutrophils
79
tx of B12 def
IM B12 injection
80
s/s of folate def (B9)
no neuro symptoms glossitis, anorexia, diarrhea, pallor, tachy
81
labs of folate def
decreased folate elevated homocysteine and normal MMA
82
tx of folate def
oral folic acid
83
s/s of hemolytic anemia
jaundice, icterus, dark urine splenomegaly anemia s/s
84
labs of hemolytic anemia
increased retic count increased LDH and bilirubin
85
list 3 subtypes of hemolytic anemia
autoimmune hereditary spherocytosis G6PD
86
+ spherocytes and + coombs test is what type of hemolytic anemia
autoimmune hemolytic anemia
87
+ spherocytes and - coombs test, hyperchromic microcytosis + osmotic fragility test
hereditary spherocytosis
88
bite cells and Heinz bodies suggest what type of hemolytic anemia
G6PD
89
list 3 triggers for G6PD hemolytic anemia
infection meds fava beans
90
tx of G6PD hemolytic anemia
avoid oxidant meds/ triggers
91
tx of hereditary spherocytosis
folic acid splenectomy
92
tx of autoimmune hemolytic anemia
steroids
93
HUS triad
thrombocytopenia hemolytic anemia renal dysfunction
94
dx of HUS
thrombocytopenia with normal coag studies and increased bleeding time normal ADAMTS13 schistocytes, reticulocytosis, increased LDH and bilirubin increased BUN and creatinine
95
tx of HUS
supportive
96
what causes TTP?
destruction of ADAMTS13
97
pentad of TTP
thrombocytopenia hemolytic anemia neuro fever kidney failure
98
dx of TTP
schistocytes, increased LDH, bilirubin decreased ADAMTS13 and antibodies
99
tx of TTP
plasmaphoresis glucocorticoids immunosuppression
100
cause of ITP
autoimmune destruction of platelets
101
s/s of ITP
asymptomatic purpura, petechiae, gum bleeding severe bleeding NO splenomegaly
102
dx of ITP
isolated thrombocytopenia increased megakaryocytes
103
tx of ITP
kids- observation platelets low- glucocorticoids, IVIG, anti-D
104
factor V Leiden disorder cause
mutated factor V--> resistant to breakdown via protein C hypercoaguability
105
s/s of factor V Leiden disorder
VTE- DVT, PE
106
dx of factor V leiden
activated protein C resistance assay if +--> confirm with DNA testing
107
tx of factor V Leiden
asymptomatic- not treated moderate- prophylaxis for procedures high risk- warfarain indefinitely
108
what type of genetic disorder is hemophilia?
x linked recessive A- VIII B- IX C- XI
109
s/s of hemophilia
deep tissue bleeding/ hemarthrosis
110
dx of hemophilia
increased PTT decreased factor
111
tx of hemophilia
factor infusion A- DDAVP and emicizumab
112
what causes Von Willebrand's disease and what type of genetic disease is it?
ineffective platelet adhesion autosomal dominant
113
s/s of vWD
prolonged bleeding
114
Dx of vWD
increased PTT and bleeding time normal platelets and PT diagnostic- decreased vWF antigen ristocetin induced platelet aggregation (RIPA)
115
tx of vWD
DDAVP severe- vWF containing product
116
timeline for heparin induced thrombocytopenia
5-14 days after exposure
117
heparin induced thrombocytopenia s/s
thrombocytopenia and thrombosis often asymptomatic
118
dx of heparin induced thrombocytopenia
initial- PF4 heparin antibody ELISA confirm- functional assay
119
tx of heparin induced thrombocytopenia
discontinue heparin and start different anticoag
120
cause of DIC
coagulation and fibrinolysis
121
s/s of DIC
bleeding- IV and catheter sites, ecchymosis, purpura thrombosis- gangrene, cyanosis
122
labs for DIC
decreased platelets, increased bleeding time, PT and PTT decreased fibrinogen increased D dimer schistocytes
123
tx of DIC
tx underlying cause severe- platelets, FFP