HemeOnc Uworld Flashcards
(168 cards)
1
Q
Tx to maintenance sickkle cell
A
vaccination
penicillin(until age 5)
folic acid supp
hydroxyurea
2
Q
Tx for acute sickle cell pain crisis
A
hydration, analgesia, +/- transfusion
3
Q
effect of hydroxyurea
A
increase fetal Hb by stimulating erythropoiesis
4
Q
Kleihauer betke tests
A
to determine amount of rhogam to five after delivery
calculates amount of fetal blood cells in maternal circulation
5
Q
findings in anemia of prematuriety
A
tachycardia, apnea, poor weight gain
low Hb and Hct
low reticulocyte count
normochromic normocytic RBC
6
Q
Tx anemia of prematurity
A
minimize blood draws, iron supp
transfusions
7
Q
what to do if sickle cell patient presenting with Sx of stroke
A
exchange tranfusion
8
Q
where does osteosarcoma present
A
meatphyses
distal femur, proximal tibia, proximal humerus
9
Q
labs in osteosarcoma
A
alk phos and LDH elevated from turnover of damaged osteocytes
10
Q
imaging of osteosarcoma
A
sunburst and codman triangle
11
Q
cotrical lesion on imagin with central nidus lucency
pain worse at night
A
osteoid osteoma
12
Q
clinical signs CLL
A
lymphadenopathy
HSM
mild thrombocytopenia and anemia
age >65
13
Q
how to Dx CLL
A
severe lymphocytosis and smudge cells
flow cytometry
14
Q
clinical features lead poisoning
A
GI- abdominal pain, constipation, anorexia
neurlogic- cognitive deficits, peripheral neuropathy
hematologic- anemia
15
Q
macrocytic anemia in sickle cell
A
likely from folate deficiency
usually normocytic normochromic
16
Q
splenic sequestration
A
vaso occlusive and splenic pooling of RBC in sickle cell
leads to abrupt fall in Hb
17
Q
findings in fanconi anemia
A
aplastic anemia
short stature, microcephaly, abnormal thumbs, hypogonads
hypopigmented skins
cafe au lait spots
large freckles
low set ears and middle ear abnormalities
18
Q
Dx fanconi Anemia
A
chromosomal breaks on genetic analysis
19
Q
when do you do leukoreduction for blood transfusion
A
chronically transfused patients
CMV seronegative at risk
potential transplant rejections
previous febrile nonhemolytic transfusion reaction
20
Q
when do you “wash” a RBC Tx
A
IgA deficiency
C’ dependent autoimmune hemolytic anemia
continued allergic reactions with red cell transfusion despite antihistamine Tx
21
Q
febrile nonhemolytic transfusion reaction
A
most common reaction that occurs within 1-6 hours of transfusion
leukocytes release cytokines
22
Q
patient with IgA deficiency needs transfusion
A
RBC should be washed to remove as much plasma as possible
23
Q
how does Cystic Fibrosis affect platelets
A
malabsorption ADEK vitamins
so affects Vit K dependent
II VII IX and X
24
Q
anterior mediastinal mass
A
thymoma
teratoma
thyroid neoplasm
terrible lymphoma
25
elevated AFP and b-hcg
nonseminomatous germ cell tumor "mixed germ cell tumor"
26
elevated hormones in seminoatous germ cell tumor
B-hcg
27
side effects of steroid abuse
```
gynecosmastia
LVH, inc LDL
aggressive behavior
polycythemia, possible hypercoagulability
can cause hepatotoxicity
```
28
PSA elevated, had prostatectomy
3 years later PSA is high again and has radiation
what type of tx is that considered
salvage
29
hypoplastic anemia, macrocytic, low reticulocyte and congenital anomalies
diamond blackfan
30
how to distinguish macrocytic anemia in Diamond blackfan from megaloblastic anemia
no hypersegmentation of nucleus in Neutrophils in diamond-blackfan
31
electrophoresis in diamond blackfan
elevated fetal Hb levels
32
Tx diamond blackfan
corticosteroids
33
what is wiskott aldrich
X linked disorder with eczema, thrombocytopenia and hypogammaglobulinemia
34
35 year old man acting weird. no HA, sensory or focal weakness
untreated HIV and hep C
fever and pulse 96
mild scleral icteris
normocytic anemia with 8% reticulocytes
low platelets, low WBC and elevated BUN elevated total bili
high alk phos and LFTs
thrombotic thrombocytopenic purpura TTP
HIV is a risk factor
will ahve schistocytes on peripheral smear
35
thrombocytopenia and hemolytic anemia without reason or alternate cause
TTP
36
flow cytometry in TTP
ADAMTS13 protease level is decreased
37
most common cause of iron deficient anemia
chronic blood loss
38
when is TIBC elevated
iron deficiency, everything else is low
39
iron panel in thalassemia
dec MCV
inc Fe
dec TIB
inc ferritin
40
iron panel in fe deficiency
dec Fe
dec MCV
inc TIBC
dec ferritin
41
iron panel in anemia of chronic disease
normal or dec MCV
dec iron
dec TIBC
normal or elevated ferritin
42
Hereditary spherocytosis
autosomal dominant
| defect in ankyrin gene causing abnormal RBC plasma membrane scaffolding proteins. more prone to spleni sequestration
43
clinical presentation HS
northern european
hemolytic anemia
jaundice
splenomegaly
44
lab findings in HS
```
inc mean corpuscular hemoglobin [ ]
spherocytes
negative coombs
inc osmotic fragility with acidified glycerol lysis test
abnormal eosin-5 maleimide binding test
```
45
Tx for HS
folic acid supp
blood transfusions
splenectomy
46
complciations HS
pigment gallstones
| aplastic crises from parvo B19
47
young male with RUQ pain and has cholelithiasis
anemia
PBS show round hyperchromic RBC
HS
48
triad HS
hemolytic anemia
jaundice
splenomegaly
49
complication MM
renal insufficiency from M protein
| will see granular casts
50
woman with back pain in lumbar and thoracic spine
4 mo ago was fine. electrolytes are ok
Hb low
no lymphadenopathy
urine dip is negative but has few granular casts
MM- monoclonal protein
51
hard unilateral non-tender lymph nodes in older patient with smoking Hx
squamous cell carcinoma
52
older patients with smoking Hx and firm solitary lymph nodes in neck area
concern for squamous cell carcinoma
53
what causes hemophilic arthropathy
increased iron/hemosiderin deposition
| causes synovitis and fibrosis
54
signs Sx of waldenstroms macroglobulinemia
```
increased spleen liver and lymph nodes
tiredness
tendency to bleed and bruise
night sweats
HA and dizziness
various visual problems
pain and numbness in extremities
```
55
IgM spike
waldenstroms macroglobulinemia
56
2 important Diagnostic components for waldenstroms macroglobulinemia
IgM spike
| hyperviscosity
57
opthalmoscopy shows dilated, segmented and tortuous retinal veins
hyperviscosity
| waldenstroms macroglobulinemia
58
aplastic anemia
pancytopenia
59
aplastic crisis
in sickle cell worsening anemia
60
lab values for hemolytis
high LDH and hyperbilirubinemia
61
phenytoin effects what
folic acid absorption so can get megaloblastic anemia
62
which drugs affect folic acid
TMP- antagonize it
MTX- antagonize it
phenytoin- impair absorption
63
ethnicity for pernicious anemia
northern european
64
shiny tongue, loss position and vibration, ataxia
Vit B 12 deficiency
65
18 year old african american, 3 day Hx progressive worsening fatigue with exertiona ldyspnea
had URI Tx with amoxicillin
now has large spleen and mild icterus normocytic anemia with 10% reticulocytes
autoimmune hemolytic anemia
66
how to Dx autoimmune hemolytic anemia
warm agglutins- direct coombs + with anti IgG anti-C3
| cold agglutins- livedo reticularis, direct coombs + with anti -C3 or anti IgM
67
Tx for autoimmune hemolytic anemia warm agglutins
corticosteroids
| splenectomy for refractory disease
68
tx for autoimmune hemolytic anemia cold agglutins
avoidance of cold temps
| rituximab +/- fludarabine
69
triggers for autoimmune hemolytic anemia
lymphoproliferative disorders
viral infections
autoimmune conditions
drugs (penicillins)
70
peripheral smear in autoimmune hemolytic anemia
spherocytes, microspherocytes, elliptocytes or increased reticulocytes
71
smudge cells
CLL
72
JAK2
myeloproliferative
| PCV
73
RDW and RBCs in beta thalassemia minor
normal
74
soap bubble appearance on XR
giant cell tumor
75
clinical signs of giant cell tumor
pain, swelling and decreased ROM
76
where do giant cell tumors occur
epiphyseal regions of long bones like distal femur and proximal tibia
77
Tx for giant cell tumor in bone
intralesional curettage with or w/o grafting
78
osteitis fibrosa cystica
von recklinhaused from hyper PTH
brown tumors causing bone pain
salt and pepper appearance
79
tartrate resistant acid phosphotase stain TRAP
hairy cell leukemia
80
CD11c
hairy cell leukemia
81
causes of anemia
decreased production
increased destruction
blood loss
82
heterophile negative but highly clinically suspicious mono
heterophile could be negative early on
83
most common inherited disorder causein hypercoagulability
factor V leiden
84
warfarin skin necrosis
protein C def
85
RDW in iron deficiency
increased!!
86
why do patients with sickle cell urinate alot
inability to concentrate urine
| problem from sickling in the vasa rectae
87
if suspect epidural spinal cord compression
give glucocorticoids immediately
88
subacute back pain with lower extremity motor weakness, hyperreflexia and bladder dysfunction
epidural spinal cord compression
89
coombs test in HS
negative
90
iron deficiency in infant
excessive consumption cows milk
91
pineal gland mass Sx
limited upper gaze
upper eyelid retratction
pupils non reactive to light
reactive to accomodation
92
what happens with pineal gland masses
obstructive hydrocephalus- HA and vomiting
93
66 yr old with back pain and constipation, urinating excessively
no neuro signs
anemia mild elevated ESR
MM
94
how does MM cause hyperCa
bone lysis from humoral factors from the plasma cells
95
hereditary hemochromatosis signs
```
hyperpigmentation
arthralgia and chondrocalicinosis
elevated LFTs
DM hypogonad, hypothyroid
restrictive or dilated cardiomyopathy
increased infections
```
96
hereditary hemochromatosis increased risk for
hepatocellular carcinoma
97
bronze diabetes
hereditary hemochromatosis
98
mutation in hereditary hemochromatosis
HFE gene
99
Tx herediaty hemochromatosis
phlebotomies
100
What is mutation in wiskot aldrich
WAS protein
| cytoskeleton regulation
101
Tx wiskott aldrich
stem cell transplant
102
eczema, hemolytic anemia, arthritis and vasculitis
wiskott aldrich
103
Tdt+ staining
ALL
104
peroxidase +
myeloblast
105
clinical features paroxysmal nocturnal hemoglobinuria
hemolysis- fatigue
cytopenias
venous thrombosis
106
workup for paroxysmal nocturnal hemoglobinuria
CBC: hypoplastic, thrombocytopenia, leukopenia
elevated LDH and low haptoglobin
indirect hyperbili
UA showing hemoglobinuria
107
low haptoglobin
hemolysis
108
flow cytometry in paroxysmal nocturnal hemoglobinuria
absent CD55 and CD59
109
Tx for paroxysmal nocturnal hemoglobinuria
iron and folate supplementation
| eculizumab (inhibits C' activation)
110
Paroxysmal nocturnal hemoglobinuria pathophys
C' activated and MAC forms and results in hemolysis
111
when does paraoxysmal nocturnal hemoglobinuria occur
in 40s
112
anemia from lead poisoning
microcytic
113
type of anemia from thalassemias
microcytic
114
leukomoid reaction
leukocytosis from severe infections
| also have high leukocyte alkaline phosphatase level
115
common complication if have sickle trait
hematuria
116
how does SLE cause pancytopenia
peripheral destruction immune mediated
117
sideroblastic anemia
defective heme synthesis
118
acquired sideroblastic anemia
isoniazid without B6 supp
119
DIC like syndrome in patient with liver failure
| give what
FFP
120
protein gap
>4 difference of total protein and albumin
121
rouleaux formation
MM
122
M protein spike
MM
123
HS labs
normal MCV
inc MCHC
inc indirect bili
inc LDH
124
acidified glycerol lysis test
osmotic fragility
| used for HS
125
eosin 5 maleimide binding test
used for HS
126
hemolytic anemia, jaundice and splenomegaly in newborn
HS
127
coagulopathy labs in antiphospholipid syndrome
increased PTT time
128
anti b2GP1 Ab
antiphospholipid syndrome
129
Tx for anemia chronic disease
Tx underlying disorder
130
post surgery bleeding from puncture sites but not DIC labs
mild anemic. normocytic. normal platelets no infection
increased PTT slightly
Vit K deficiency because they did not feed him
131
howel jowel bodies
absence of spleen or splenic autoinfarction
| blue inclusions with wright stain
132
hemolytic anemia and thrombocytopenia in renal faiure with gramented cells in peripheral smear
TTP-HUS
133
Tx TTP-HUS
plasmapheresis to remove offending autoAb and replete deficient enzyme
134
polycythemia, nausea, dizziness and HA
carboxyhemoglobinemia
135
ABO vs Rh incompatability which is worse
Rh
| ABO is mild
136
Tx hairy cell leukemia
cladribine
137
bone marrow in hairy cell leukemia
dry tap
138
adverse effects cladribine
neurological and kidney damage
139
all patients with presume ITP should be tested for what
HIV and Hep C
140
howell jowel bodies
sickle cell
141
helmet cell bodies
traumatic hemolytic conditions like DIC, HUS and TTO
142
why are patients wih MM prone to infections
ineffective Ab production
| hypogammaglobulinemia
143
what can increase risk for neonatal polycythemia
increased erythropoiesis from hypoxia in utero
- DM
- maternal HTN
- smoking
- IUGR
144
swelling in face and arm worse in morning. new onset exertional dyspnea
weight loss over short period time
afebrile
face is plethoric and dark appearing
SVC
145
most common cause of SVC
malignancy causing obstruction
| lung CA and non hodgkin lymphoma
146
signs of vit K deficiency in newborn
brusiing, bloody stools and intracranial hemorrhage
147
most common brain tumor in children
pilocytic astrocytoma
148
assoc syndromes with wilms tumors
WAGR
beckwith wiedemann
denys drash
149
WAGR
Wilms tumor
Aniridia
GU
Retardation
150
imtainib works how
tyrosine kinase inhbitior
151
retinoic R is attacked in what disease
promyelocytic AML
152
what to do for type 2 HIT
stop heparin and start argatroban
153
signs CML
dramatic leukocytosis >100,000 and absolute basophilia
| lower leukoctye alkaline phosphotase
154
side effects of EPO therapy
worsenin gHTN
HA
flu like syndrome
red cell aplasia
155
Tx for children with ITP
observation
156
homocysteine in patient
| supplement with what
pyridoxine
157
what is recommended to stabilize bony metastatic lesions
bisphosphonates
158
HIT 2 puts you at risk for
arterial thrombosis
159
Tx of acute DVT that is now there for a week and gotten worse due to noncomplainat patient on warfarin
direct factor XA inhibitor like rivaroxaban
160
first line for chemo induced nausea
serotonin R antagonist like ondansteron 5HT3
161
what helps increase appetite for cancer patients
progesterone analoges like megestrol acetate
162
Dx ALL patient with obvious lymph nodes
bone marrow bx
163
pigment gall stones
HA
164
trousseaus syndrome
hypercoagulable
superficial venous thrombosis usually in chest and arms
usually means CA somewhere like pancreas, lung, prostate, stomach colon and acute leukemias
165
risk with pernicious anemia
gastric cancer, atrophic gastriits
166
osler weber rendu
hereditary telangiectasias
auto dominant
with recurrent epistaxis, widespread AV malgormations
167
findings in primary polycythemia vera
low EPO
JAK2 mutation V617F
increased WBC, RBC, platelet
168
deficiency of folic acid or cobalamine results in what
increased homocysteine
