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Flashcards in HemeOnc Uworld Deck (168):
1

Tx to maintenance sickkle cell

vaccination
penicillin(until age 5)
folic acid supp
hydroxyurea

2

Tx for acute sickle cell pain crisis

hydration, analgesia, +/- transfusion

3

effect of hydroxyurea

increase fetal Hb by stimulating erythropoiesis

4

Kleihauer betke tests

to determine amount of rhogam to five after delivery
calculates amount of fetal blood cells in maternal circulation

5

findings in anemia of prematuriety

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

6

Tx anemia of prematurity

minimize blood draws, iron supp
transfusions

7

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

exchange tranfusion

8

where does osteosarcoma present

meatphyses
distal femur, proximal tibia, proximal humerus

9

labs in osteosarcoma

alk phos and LDH elevated from turnover of damaged osteocytes

10

imaging of osteosarcoma

sunburst and codman triangle

11

cotrical lesion on imagin with central nidus lucency
pain worse at night

osteoid osteoma

12

clinical signs CLL

lymphadenopathy
HSM
mild thrombocytopenia and anemia
age >65

13

how to Dx CLL

severe lymphocytosis and smudge cells
flow cytometry

14

clinical features lead poisoning

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

15

macrocytic anemia in sickle cell

likely from folate deficiency
usually normocytic normochromic

16

splenic sequestration

vaso occlusive and splenic pooling of RBC in sickle cell
leads to abrupt fall in Hb

17

findings in fanconi anemia

aplastic anemia
short stature, microcephaly, abnormal thumbs, hypogonads
hypopigmented skins
cafe au lait spots
large freckles
low set ears and middle ear abnormalities

18

Dx fanconi Anemia

chromosomal breaks on genetic analysis

19

when do you do leukoreduction for blood transfusion

chronically transfused patients
CMV seronegative at risk
potential transplant rejections
previous febrile nonhemolytic transfusion reaction

20

when do you "wash" a RBC Tx

IgA deficiency
C' dependent autoimmune hemolytic anemia
continued allergic reactions with red cell transfusion despite antihistamine Tx

21

febrile nonhemolytic transfusion reaction

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

22

patient with IgA deficiency needs transfusion

RBC should be washed to remove as much plasma as possible

23

how does Cystic Fibrosis affect platelets

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

24

anterior mediastinal mass

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