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Step2CK > HemeOnc Uworld > Flashcards

HemeOnc Uworld Flashcards

1
Q

Tx to maintenance sickkle cell

A

vaccination
penicillin(until age 5)
folic acid supp
hydroxyurea

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2
Q

Tx for acute sickle cell pain crisis

A

hydration, analgesia, +/- transfusion

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3
Q

effect of hydroxyurea

A

increase fetal Hb by stimulating erythropoiesis

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4
Q

Kleihauer betke tests

A

to determine amount of rhogam to five after delivery

calculates amount of fetal blood cells in maternal circulation

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5
Q

findings in anemia of prematuriety

A

tachycardia, apnea, poor weight gain
low Hb and Hct
low reticulocyte count
normochromic normocytic RBC

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6
Q

Tx anemia of prematurity

A

minimize blood draws, iron supp

transfusions

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7
Q

what to do if sickle cell patient presenting with Sx of stroke

A

exchange tranfusion

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8
Q

where does osteosarcoma present

A

meatphyses

distal femur, proximal tibia, proximal humerus

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9
Q

labs in osteosarcoma

A

alk phos and LDH elevated from turnover of damaged osteocytes

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10
Q

imaging of osteosarcoma

A

sunburst and codman triangle

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11
Q

cotrical lesion on imagin with central nidus lucency

pain worse at night

A

osteoid osteoma

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12
Q

clinical signs CLL

A

lymphadenopathy
HSM
mild thrombocytopenia and anemia
age >65

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13
Q

how to Dx CLL

A

severe lymphocytosis and smudge cells

flow cytometry

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14
Q

clinical features lead poisoning

A

GI- abdominal pain, constipation, anorexia
neurlogic- cognitive deficits, peripheral neuropathy
hematologic- anemia

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15
Q

macrocytic anemia in sickle cell

A

likely from folate deficiency

usually normocytic normochromic

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16
Q

splenic sequestration

A

vaso occlusive and splenic pooling of RBC in sickle cell

leads to abrupt fall in Hb

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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

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18
Q

Dx fanconi Anemia

A

chromosomal breaks on genetic analysis

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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

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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

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21
Q

febrile nonhemolytic transfusion reaction

A

most common reaction that occurs within 1-6 hours of transfusion
leukocytes release cytokines

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22
Q

patient with IgA deficiency needs transfusion

A

RBC should be washed to remove as much plasma as possible

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23
Q

how does Cystic Fibrosis affect platelets

A

malabsorption ADEK vitamins
so affects Vit K dependent
II VII IX and X

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24
Q

anterior mediastinal mass

A

thymoma
teratoma
thyroid neoplasm
terrible lymphoma

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25
Q

elevated AFP and b-hcg

A

nonseminomatous germ cell tumor “mixed germ cell tumor”

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26
Q

elevated hormones in seminoatous germ cell tumor

A

B-hcg

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27
Q

side effects of steroid abuse

A 
gynecosmastia
LVH, inc LDL
aggressive behavior
polycythemia, possible hypercoagulability
can cause hepatotoxicity
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28
Q

PSA elevated, had prostatectomy
3 years later PSA is high again and has radiation
what type of tx is that considered

A

salvage

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29
Q

hypoplastic anemia, macrocytic, low reticulocyte and congenital anomalies

A

diamond blackfan

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30
Q

how to distinguish macrocytic anemia in Diamond blackfan from megaloblastic anemia

A

no hypersegmentation of nucleus in Neutrophils in diamond-blackfan

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31
Q

electrophoresis in diamond blackfan

A

elevated fetal Hb levels

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32
Q

Tx diamond blackfan

A

corticosteroids

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33
Q

what is wiskott aldrich

A

X linked disorder with eczema, thrombocytopenia and hypogammaglobulinemia

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34
Q

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

A

thrombotic thrombocytopenic purpura TTP
HIV is a risk factor
will ahve schistocytes on peripheral smear

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35
Q

thrombocytopenia and hemolytic anemia without reason or alternate cause

A

TTP

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36
Q

flow cytometry in TTP

A

ADAMTS13 protease level is decreased

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37
Q

most common cause of iron deficient anemia

A

chronic blood loss

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38
Q

when is TIBC elevated

A

iron deficiency, everything else is low

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39
Q

iron panel in thalassemia

A

dec MCV
inc Fe
dec TIB
inc ferritin

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40
Q

iron panel in fe deficiency

A

dec Fe
dec MCV
inc TIBC
dec ferritin

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41
Q

iron panel in anemia of chronic disease

A

normal or dec MCV
dec iron
dec TIBC
normal or elevated ferritin

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42
Q

Hereditary spherocytosis

A

autosomal dominant

defect in ankyrin gene causing abnormal RBC plasma membrane scaffolding proteins. more prone to spleni sequestration

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43
Q

clinical presentation HS

A

northern european
hemolytic anemia
jaundice
splenomegaly

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44
Q

lab findings in HS

A 
inc mean corpuscular hemoglobin [ ]
spherocytes
negative coombs
inc osmotic fragility with acidified glycerol lysis test
abnormal eosin-5 maleimide binding test
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45
Q

Tx for HS

A

folic acid supp
blood transfusions
splenectomy

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46
Q

complciations HS

A

pigment gallstones

aplastic crises from parvo B19

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47
Q

young male with RUQ pain and has cholelithiasis
anemia
PBS show round hyperchromic RBC

A

HS

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48
Q

triad HS

A

hemolytic anemia
jaundice
splenomegaly

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49
Q

complication MM

A

renal insufficiency from M protein

will see granular casts

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50
Q

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

A

MM- monoclonal protein

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51
Q

hard unilateral non-tender lymph nodes in older patient with smoking Hx

A

squamous cell carcinoma

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52
Q

older patients with smoking Hx and firm solitary lymph nodes in neck area

A

concern for squamous cell carcinoma

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53
Q

what causes hemophilic arthropathy

A

increased iron/hemosiderin deposition

causes synovitis and fibrosis

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54
Q

signs Sx of waldenstroms macroglobulinemia

A 
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
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55
Q

IgM spike

A

waldenstroms macroglobulinemia

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56
Q

2 important Diagnostic components for waldenstroms macroglobulinemia

A

IgM spike

hyperviscosity

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57
Q

opthalmoscopy shows dilated, segmented and tortuous retinal veins

A

hyperviscosity

waldenstroms macroglobulinemia

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58
Q

aplastic anemia

A

pancytopenia

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59
Q

aplastic crisis

A

in sickle cell worsening anemia

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60
Q

lab values for hemolytis

A

high LDH and hyperbilirubinemia

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61
Q

phenytoin effects what

A

folic acid absorption so can get megaloblastic anemia

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62
Q

which drugs affect folic acid

A

TMP- antagonize it
MTX- antagonize it
phenytoin- impair absorption

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63
Q

ethnicity for pernicious anemia

A

northern european

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64
Q

shiny tongue, loss position and vibration, ataxia

A

Vit B 12 deficiency

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65
Q

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

A

autoimmune hemolytic anemia

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66
Q

how to Dx autoimmune hemolytic anemia

A

warm agglutins- direct coombs + with anti IgG anti-C3

cold agglutins- livedo reticularis, direct coombs + with anti -C3 or anti IgM

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67
Q

Tx for autoimmune hemolytic anemia warm agglutins

A

corticosteroids

splenectomy for refractory disease

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68
Q

tx for autoimmune hemolytic anemia cold agglutins

A

avoidance of cold temps

rituximab +/- fludarabine

69
Q

triggers for autoimmune hemolytic anemia

A

lymphoproliferative disorders
viral infections
autoimmune conditions
drugs (penicillins)

70
Q

peripheral smear in autoimmune hemolytic anemia

A

spherocytes, microspherocytes, elliptocytes or increased reticulocytes

71
Q

smudge cells

72
Q

JAK2

A

myeloproliferative

PCV

73
Q

RDW and RBCs in beta thalassemia minor

74
Q

soap bubble appearance on XR

A

giant cell tumor

75
Q

clinical signs of giant cell tumor

A

pain, swelling and decreased ROM

76
Q

where do giant cell tumors occur

A

epiphyseal regions of long bones like distal femur and proximal tibia

77
Q

Tx for giant cell tumor in bone

A

intralesional curettage with or w/o grafting

78
Q

osteitis fibrosa cystica

A

von recklinhaused from hyper PTH
brown tumors causing bone pain
salt and pepper appearance

79
Q

tartrate resistant acid phosphotase stain TRAP

A

hairy cell leukemia

80
Q

CD11c

A

hairy cell leukemia

81
Q

causes of anemia

A

decreased production
increased destruction
blood loss

82
Q

heterophile negative but highly clinically suspicious mono

A

heterophile could be negative early on

83
Q

most common inherited disorder causein hypercoagulability

A

factor V leiden

84
Q

warfarin skin necrosis

A

protein C def

85
Q

RDW in iron deficiency

A

increased!!

86
Q

why do patients with sickle cell urinate alot

A

inability to concentrate urine

problem from sickling in the vasa rectae

87
Q

if suspect epidural spinal cord compression

A

give glucocorticoids immediately

88
Q

subacute back pain with lower extremity motor weakness, hyperreflexia and bladder dysfunction

A

epidural spinal cord compression

89
Q

coombs test in HS

90
Q

iron deficiency in infant

A

excessive consumption cows milk

91
Q

pineal gland mass Sx

A

limited upper gaze
upper eyelid retratction
pupils non reactive to light
reactive to accomodation

92
Q

what happens with pineal gland masses

A

obstructive hydrocephalus- HA and vomiting

93
Q

66 yr old with back pain and constipation, urinating excessively
no neuro signs
anemia mild elevated ESR

94
Q

how does MM cause hyperCa

A

bone lysis from humoral factors from the plasma cells

95
Q

hereditary hemochromatosis signs

A 
hyperpigmentation
arthralgia and chondrocalicinosis
elevated LFTs
DM hypogonad, hypothyroid
restrictive or dilated cardiomyopathy
increased infections
96
Q

hereditary hemochromatosis increased risk for

A

hepatocellular carcinoma

97
Q

bronze diabetes

A

hereditary hemochromatosis

98
Q

mutation in hereditary hemochromatosis

99
Q

Tx herediaty hemochromatosis

A

phlebotomies

100
Q

What is mutation in wiskot aldrich

A

WAS protein

cytoskeleton regulation

101
Q

Tx wiskott aldrich

A

stem cell transplant

102
Q

eczema, hemolytic anemia, arthritis and vasculitis

A

wiskott aldrich

103
Q

Tdt+ staining

104
Q

peroxidase +

A

myeloblast

105
Q

clinical features paroxysmal nocturnal hemoglobinuria

A

hemolysis- fatigue
cytopenias
venous thrombosis

106
Q

workup for paroxysmal nocturnal hemoglobinuria

A

CBC: hypoplastic, thrombocytopenia, leukopenia
elevated LDH and low haptoglobin
indirect hyperbili
UA showing hemoglobinuria

107
Q

low haptoglobin

108
Q

flow cytometry in paroxysmal nocturnal hemoglobinuria

A

absent CD55 and CD59

109
Q

Tx for paroxysmal nocturnal hemoglobinuria

A

iron and folate supplementation

eculizumab (inhibits C’ activation)

110
Q

Paroxysmal nocturnal hemoglobinuria pathophys

A

C’ activated and MAC forms and results in hemolysis

111
Q

when does paraoxysmal nocturnal hemoglobinuria occur

112
Q

anemia from lead poisoning

A

microcytic

113
Q

type of anemia from thalassemias

A

microcytic

114
Q

leukomoid reaction

A

leukocytosis from severe infections

also have high leukocyte alkaline phosphatase level

115
Q

common complication if have sickle trait

116
Q

how does SLE cause pancytopenia

A

peripheral destruction immune mediated

117
Q

sideroblastic anemia

A

defective heme synthesis

118
Q

acquired sideroblastic anemia

A

isoniazid without B6 supp

119
Q

DIC like syndrome in patient with liver failure

give what

120
Q

protein gap

A

> 4 difference of total protein and albumin

121
Q

rouleaux formation

122
Q

M protein spike

123
Q

HS labs

A

normal MCV
inc MCHC
inc indirect bili
inc LDH

124
Q

acidified glycerol lysis test

A

osmotic fragility

used for HS

125
Q

eosin 5 maleimide binding test

A

used for HS

126
Q

hemolytic anemia, jaundice and splenomegaly in newborn

127
Q

coagulopathy labs in antiphospholipid syndrome

A

increased PTT time

128
Q

anti b2GP1 Ab

A

antiphospholipid syndrome

129
Q

Tx for anemia chronic disease

A

Tx underlying disorder

130
Q

post surgery bleeding from puncture sites but not DIC labs
mild anemic. normocytic. normal platelets no infection
increased PTT slightly

A

Vit K deficiency because they did not feed him

131
Q

howel jowel bodies

A

absence of spleen or splenic autoinfarction

blue inclusions with wright stain

132
Q

hemolytic anemia and thrombocytopenia in renal faiure with gramented cells in peripheral smear

133
Q

Tx TTP-HUS

A

plasmapheresis to remove offending autoAb and replete deficient enzyme

134
Q

polycythemia, nausea, dizziness and HA

A

carboxyhemoglobinemia

135
Q

ABO vs Rh incompatability which is worse

A

Rh

ABO is mild

136
Q

Tx hairy cell leukemia

A

cladribine

137
Q

bone marrow in hairy cell leukemia

138
Q

adverse effects cladribine

A

neurological and kidney damage

139
Q

all patients with presume ITP should be tested for what

A

HIV and Hep C

140
Q

howell jowel bodies

A

sickle cell

141
Q

helmet cell bodies

A

traumatic hemolytic conditions like DIC, HUS and TTO

142
Q

why are patients wih MM prone to infections

A

ineffective Ab production

hypogammaglobulinemia

143
Q

what can increase risk for neonatal polycythemia

A

increased erythropoiesis from hypoxia in utero

  • DM
  • maternal HTN
  • smoking
  • IUGR
144
Q

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

145
Q

most common cause of SVC

A

malignancy causing obstruction

lung CA and non hodgkin lymphoma

146
Q

signs of vit K deficiency in newborn

A

brusiing, bloody stools and intracranial hemorrhage

147
Q

most common brain tumor in children

A

pilocytic astrocytoma

148
Q

assoc syndromes with wilms tumors

A

WAGR
beckwith wiedemann
denys drash

149
Q

WAGR

A

Wilms tumor
Aniridia
GU
Retardation

150
Q

imtainib works how

A

tyrosine kinase inhbitior

151
Q

retinoic R is attacked in what disease

A

promyelocytic AML

152
Q

what to do for type 2 HIT

A

stop heparin and start argatroban

153
Q

signs CML

A

dramatic leukocytosis >100,000 and absolute basophilia

lower leukoctye alkaline phosphotase

154
Q

side effects of EPO therapy

A

worsenin gHTN
HA
flu like syndrome
red cell aplasia

155
Q

Tx for children with ITP

A

observation

156
Q

homocysteine in patient

supplement with what

A

pyridoxine

157
Q

what is recommended to stabilize bony metastatic lesions

A

bisphosphonates

158
Q

HIT 2 puts you at risk for

A

arterial thrombosis

159
Q

Tx of acute DVT that is now there for a week and gotten worse due to noncomplainat patient on warfarin

A

direct factor XA inhibitor like rivaroxaban

160
Q

first line for chemo induced nausea

A

serotonin R antagonist like ondansteron 5HT3

161
Q

what helps increase appetite for cancer patients

A

progesterone analoges like megestrol acetate

162
Q

Dx ALL patient with obvious lymph nodes

A

bone marrow bx

163
Q

pigment gall stones

164
Q

trousseaus syndrome

A

hypercoagulable
superficial venous thrombosis usually in chest and arms
usually means CA somewhere like pancreas, lung, prostate, stomach colon and acute leukemias

165
Q

risk with pernicious anemia

A

gastric cancer, atrophic gastriits

166
Q

osler weber rendu

A

hereditary telangiectasias
auto dominant
with recurrent epistaxis, widespread AV malgormations

167
Q

findings in primary polycythemia vera

A

low EPO
JAK2 mutation V617F
increased WBC, RBC, platelet

168
Q

deficiency of folic acid or cobalamine results in what

A

increased homocysteine

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