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Flashcards in HemeOnc Step Up Deck (303):
1

what happens to O2 curve in alkalosis

decreased body temp, increased HbF shfit to left

2

what happens to O2 curve in acidosis

increased body temp
high altitude
exercise shift curve to right

3

what is a left shift fo O2 curve

increased Hb affinity

4

what is a right shift O2 curve

decreased affinity for O2. easier off loading

5

immediate Tx for CO poisoning

100% O2 mask

6

signs CO poisoning

mental status changes, cherry red lips, hypoxia despite normal pulse ox readings

7

causes of microcytic acnemia

iron deficiency
lead poisoning
chronic disease
sideroblastic
thalassemias

8

causes of normocytic anemia

hemolytic
chronic disease
hypovolemia

9

causes of macrocytic anemia

folate def
vit B12 def
liver disease
alcohol abuse

10

signs of hemolytic anemia

brown urine
HSM
jaundice

11

labs in hemolytic anemia

decrease Hb and Hct
increased reticulocyte count from turnover
increased bilirubin (indirect) from lysis
increased LDH
normal MCV!!!!
decreased serum haptoglobin

12

most common form anemia

iron deficiency

13

Coombs direct test

Coombs reagant mixed with RBC. if agglutinates then IgG and c' are present (warm and cold agglutinins.

14

Coombs indirect test

patient serum mixed with type O RBCs
then mixed with coombs reagant
if agglutinate-- anti-RBC Ab in serum
Rh alloimmunization

15

schistocytes

seen in hemolytic anemia
RBC fragments

16

angular cheilitis

irritaiton of lips and corners of mouth

17

blood smear shows burr ceslls and schistocytes

Drug induced hemolytic anemia

18

wha drugs cause hemolytic anemia

penicillin, methyldopa
quinidine

19

warm reacting Ab are what

IgG

20

cold reacting Ab are what

IgM

21

Tx immune hemolytic anemia

corticosteroids

22

Coombs test is + when

drug induced hemolytic anemia and immune

23

finding in hereditary spherocytosis

HSM

24

Tx hereditary spherocytosis

splenectomy

25

what induced G6PD hemolytic anemia

ingestion fava beans high dose ASA, sulfa drugs, dapsone, quinine, quinidine, primaquine, nitrofurantoin

26

bite cells and heinz bodies

G6PD def

27

labs in Fe def anemia

decreased Hb and Hct
decreased MCV
decreased or normal reticulocytes
decreased ferritin
decreased Fe
increased transferrin- TIBC
low Fe:TIBC ratio

28

labs in anemia of chronic disease

decreased Fe
normal or increased ferritin
decreased TIBC(transferrin)
hypochromic microcytic or normocytic

29

labs in lead poisoning anemia

inc Fe or normal
inc or normal ferritin
normal or decreased TIBC
stippled microcytic RBC

30

labs in sideroblastic anemia

inc Fe
inc ferritin
dec TIBC (transferrin)
ringed sideroblasts in bone marrow

31

labs in thalassemias

inc or normal Fe
inc or normal ferritin
normal TIBC
increased Fe:TIBC ratio
target cells in alpha
basophilic stippling in beta

32

signs of lead poisoning

mental developmental delayse, gingival lead lines, peripheral neuropathy (motor)
pallor weakness, abdominal pain

33

basophilic stippling

lead poisoning

34

Tx for lead poisoning

EDTA or simercaptosuccinic acid DMSA for chelation

35

severe lead toxication Tx

dimercaprol

36

most common cause megaloblastic anema

folate def

37

what drugs inhibit folate metabolism

MTX, trimethoprim and phenytoin

38

signs of folate def

diarrhea, sore tongue, palor, tachy, tachypnea, inc pulse P, not neuro Sx

39

labs in folate def

dec Hb and HCt
increased MCV
dec folate
decreased reticulocytes

40

hypersegmented neutrophils

folate def and B12 def

41

populations with inadequate folate

alcoholics and elderly

42

pernicious anemia

autoimmune anemia from no IF so cannot absorb B12

43

infection with pernicious anemia

diphyllobothrium latum

44

Sx B12 def

DOE, memory loss, tachy, inc PP, symmetric paresthesias, loss vibration, ataxia, dementia

45

labs in B12 def

macrocytic
dec Hb Hct
increased MCV
dec B12

46

which diet causes B12 def

strict vegetarian

47

which megaloblastic anemia deficiency occurs first

folate because have lots of B12 stores in body

48

associated findings in anemia of chronic disease

iron trapping in macrophages
decreased EPO
increased hepcidin (inhibits iron absorption)

49

labs in anemia chronic disease

mild dec Hb and Hct
normal or dec MCV
dec Fe
dec transferrin!!! TIBC
normal or increased ferritin

50

aplastic anemia in sickle cell patient

parvoB19

51

what is aplastic anemia

pancytopenia in bone marrow failure

52

what drugs cause aplastic anemia

chloramphenicol, sulfonamides, phenytoin, chemo Rx

53

Signs Sx aplastic anemia

fatigue weakness, persistent infections, poor clotting, easy bruising, pallor. petechiae, tachy, tachypnea, systolic murmur. inc PP

54

labs in aplastic anemia

dec Hb HCt dec WBC dec platelets
BM shows hypocellularity and fatty infiltrate!!!

55

Tx for long term survival aplastic anemia

BM transplant

56

what causes sideroblastic anemia

defective heme synthesis

57

iron panel in sideroblastic anemia

dec Hb Hct
increased ferritin
increased Fe
decreased TIBC(transferrin)

58

blood smear in sideroblastic anemia

multiple sizes of RBCs
ringed sideroblasts-- RBC surrounded by iron granules that stain blue

59

Tx sideroblastic anemia

B6 in hereditary
give EPO in acquired cases

phlebotomy for iron overload
chelation with deferoxamine

60

sideroblastic anemia puts one at risk for

Acute leukemia

61

what causes thalassemias

Hb defects in alpha or beta globin

62

alpha thalassemia common in what ethnicities

asian or african

63

alpha thalassemia minima

1 abnormal alpha allele
asymptomatic

64

alpha thalassemia minor

reduced alpha globin
mild anemia
microcytic anemia and target cells

65

Hemoglobin H disease

3 abnormal alpha alleles
chronic hemolytic anmia, splenomegaly, microcytic RBcs, HbH in blood, decreased lifespan

66

What causes hydrops fetalis

4 abnormal alpha chains
Hb Barts (no alpha)

67

beta thalassemia minor

1 abnormal beta allele
mild anemia, increased Hb A2

68

beta thalassemia major

2 abnormal beta alleles
no beta globin production
asymptomatic until decline fetal Ab
growth retardation, develoipmental delays, bony abnormalities, HSM, anemia, increase A2 and F
microcytic anemia
die without tranfusions

69

what ethnicity has beta thalassemias

mediterranean

70

labs in thalassemias

decreased MCV
increased reticulotyes
in Hb Bart (cannot release O2)
increased HbA2 or F in beta thalssemia

71

Dx thalassemia

Hb electrophoresis

72

which thalassemia has RBCs of variable size and shapes with target cells

beta

73

find micorcytic anemia on blood smear
next step

rule out thalassemia before giving Fe because can cause overload

74

complications thalassemias

chronic iron overload from transfusions
damages heart and liver

75

sickle cell is defect in what

beta chain of Hb causing HbS- poorly soluble when deoxygenated
defective chain

76

what causes sickle shape

acidosis, hypoxia and dehydration

77

labs in sickle cell

dec Hct
increased reticulocyte
increased PMN
decreased haptoglobin
increased indirect bili

78

Hb in sickle cell

HbS, no HbA
may have Hb F

79

imaging findings sickle cell

codfish vertebrae
lung infltrates in acute chest syndrome

80

blood smear sickle cell

target cells, nucleated RBCs, deoxygenation of blood produces sickle cells

81

when do HbF levels decrease

after 6 mo old

82

Tx sickle cell

hydration
supp O2
analgesics during crisis
hydroxyurea

83

how does hydroxyurea help in sickle cell

increases HbF

84

what vaccine do you give for sickle cell patients

pneumococcal and meningococcal

85

sickle cell patients are susceptible to what

salmonella osteomyelitis
strep pneumo
H flu
N meningitis
Klebsiella
(encapsulated)

86

what is acute chest syndrome

acute pneumonia, pulm infarction and embolus
in sickle cell

87

what organs are most susceptible to ischemia in sickle cell patients

kidney heart and retina

88

what can cause iatrogenic lymphopenia

after chemo
radiation
diseases with increased cortisol

89

what diseases have eosinophilia

addison
neoplasm
asthma
allergic drug reactions
collagen vascular diseases
transplant rejections
parasitic infections

90

what drugs cause neutropenia

clozapine, antithyroid medicaitons, sulfasalazine, methimazole, TMP-SMX, chemo and aplastic anemia

91

what can help with neutropenia

granulocyte colony stimulating factor, corticosteroids

92

Type I HS

anaphylactic IgE
release of mast cell contents

93

Tx type I HS

antihistamines, leukotriene inhibitors, bronchodilators, corticosteroids

94

Type II HS

IgM and IgG reacting with complement cascade

95

examples of type II HS

drug induced or immune hemolytic anemia
hemolytic disease of the newborn

96

Tx type II HS

anti inflammatories or immunosupressive agents

97

type III HS

IgM and IgG IC that deposit into tissue and start C' cascade

98

types of HS III

arthus reaction
serum sickness
glomerulonephritis

99

Tx type III HS

anti inflammatories

100

type IV HS

T cells and macropahges which secrete lymphokines that induce macrophages to destroy tissue

101

types of HS IV

transplant rejection
allergic contact (poison ivy)
dermatitis
PPD testing

102

Tx type IV HS

corticosteroids or immunosuppressants

103

Tx for anaphylaxis

subcut epinephrine
intubation
antihistamines
bronchodilators
recumbent positioning
IV hydration
vasopressors for hypotension

104

what calculates platelet function

bleeding time

105

what induces the vasoconstriction and platelet plug at sites of vascular injury

ADP

106

coagulation cascade makes what

fibrin clot

107

PTT reflects what

intrinsic pathway

108

PT reflects what

extrinsic pathyway
induced by tissue factor

109

if have increased PT or INR then suspect what

decrease function Factors II VII IX and X

110

heparins effect

enhances antithrombin III

111

problem if start warfarin before LMWH

short period hypercoagulability from inhibition proteins C and S

112

what is considered thrombocyopenia

113

labs for thrombocytopenia

increased BT

114

which clotting factors do not decreased with liver failure

vWF and factor VIII

115

inheritance vWD

auto dominant

116

labs in vWD

increased PTT
increased BT
decreased factor VIII
decreased vWF
decreased ristocetin cofactor activity

117

Tx for vWD

desmopressin for minor bleeding
vWF concentrate and factor VIII concentrate before surgery

118

how does ASA work

inhibits cyclooxygenase and supress thromboxane A2

119

what are thiopyridines and how do they work

clopidogrel and ticlopidine
block ADP R to suppress fibrinogen binding to injury and platelet adhesion

120

What are the GIIb/IIIa inhibitors

abciximab
tirofiban
epitifibatide

121

how do GIIb/IIIa inhibitors work

inhibit platelet aggregatino

122

What are the adenosine reuptake inhibitors and how do they work

dipyridamole
inhibit activity adenosine deaminase and phosphodiesterase to inhibit aggregation

123

how does heparin work

binds antithrombin to increase activity and prevent clot formation

124

adverse effects heparin

hemorrhage
HS
thrombocytopenia
narrow therapeutic window

125

how does LMWH work

binds to factor Xa to prevent clot formation

126

What are the direct thrombin inhibitors

lepirudin
argatroban

127

direct thrombin inhibitors suppress acitvity of what factors

V IX and XIII and aggregation

128

what are the direct Xa inhibitors

apixaban
rivaroxaban

129

how do the direct Xa inhibitors work

no activity against thrombin
highly selective inhibition factor Xa which is in the common pathway

130

how does warfarin work

antagonize K dependent carboxylation of II VII IX and X

131

labs in Vit K def

increased PT and increased INR

132

Tx vit K def

oral or IM vit K and FFP

133

inheritance hemophilia

X linked recessive

134

hemophilia A

factor VIII

135

hemophilia B

factor IX

136

labs in hemophilia

increased PTT
normal PT
normal BT
decareased VIII or IX

137

Tx for hemophilias

replacement of factors
desmopressin can help in hemophilia A
transufsions needed

138

thrombocytopenia with splenomegaly and normal Bone marrow

splenic platlet sequestration

139

Dx HIT

diffuse thrombus formation
sudden decrease platlets
+ serotonin release assay
+ Heparin induced platelet aggregation assay

140

What is Immune thrombocytopenia ITP

autoimmune B cell directed production of antiplatelet Ab

141

platelet count in ITP

142

Tx for ITP

adults need corticosteroids
delayed splenectomy
IV Ig, plasmapheresis or recombinant VIIa

143

What causes TTP-HUS

diffuse platelet aggregation due to AutoAb against preventative enzyme
assoc with endothelial injury and Ecoli infection

144

Dx TTP-HUS

hemolytic anemia, acute renal failure, thrombocytopenia without severe bleeding
sometimes fever

145

Tx TTP-HUS

corticosteroids, plasmapheresis, FFP

146

Tx antiphospholipid syndrome

anticoagulation with heparin warfarin and hydroxychloroquine

147

Dx HELLP

during pregnancy
HTN
increased LFTs
decreased Hb
schistocytes

148

labs in DIC

dec platelets
increased PT and PTT
decreased fibrinogen

149

blood smear DIC

schistocytes with few platelets

150

Tx DIC

underlying disorder
platelets
FFP
cryoprecipitate
heparin

151

Dx for Systemic inflammatory response synrome

2:
-temp >38.3 or 90
-RR >20 or PaCO212000 or 10% bands

152

community acquired sepsis pathogens

strep
taph
E coli
Klebsiella
Pseudomonas
N meningitis

153

Nosocomial pathogens causing sepsis

Staph, gram neg bacilli anaerobes, pseudomonas and candida

154

sepsis in IV drug user

staph

155

what mosquito caries malaria

anopheles

156

Sx malaria

chills, diaphoresis, HA, myalgias, fatigue, nausea, abdominal pain, vomiting, diarrhea, periodic fever, splenomegaly

157

Dx malaria

PCR

158

stain used in blood smear malaria

giemsa

159

Tx malaria

chloroquine, primaquine, quinine
atavoquone and megloquine used in the R cases of P falciparum

160

labs in mono

+ heterophile (monospot)
+ EBV serology
elevated LFTs
increased WBCs
hemolytic anemia
thrombocytopenia

161

blood smear in mono

increased lymphocytes

162

Tx mono

supportive

163

complications mono

splenic rupture
rare aplastic anemia
DIC, tTP
fulminant liver failure

164

what type of virus is HIV 1 and 2

RNA retro

165

risk factors HIV in US

homosexual, bisexual
IV drug user
blood transfusions before 1980s
high risk sexual behavior

166

acute Sx hIV

flu like with myalgias nausea and vomiting, diarrhea
sore throat weight loss
mucosal ulcers fever lymphadenopathy

167

late Sx HIV

opportunistic infections and AIDS illnesses
weight loss night sweats and dementia

168

needle stick with HIV + patient next step

prophylactic tenofovir and emtricitabine and raltegravir

169

Testing post needle stick for HIV

immediately, 6 weeks, 3 mo and 6 mo

170

opportunistic infection CD4

herpes zoster or simplex
parasitic diarrhea from isospora, strongyloides and crytptosporidium

171

Tx parasitic diarrhea in HIV patientq

antiretroviral and metronidazole
TMP SMX and paromomycin

172

opportunistic infection CD4

kaposi sarcoma
coccidiomycosis

173

Tx kaposi sarcoma

topical alitretinoin, chemo, laser therapy and radiation

174

Tx coccidiomycosis

fluconazole, itraconazole, amphotericin B

175

opportunistic infections CD4

AIDS dementia
bacterial pneumonia
candida esophagitis
cervical CA
PCP pneumonia
TB

176

Dx of AIDs dementia

declining mental status
generalized neuro Sx
elevated B2 microglobulin in CSF
cerebral atrophy on CT or MRI

177

dysphagia and odynophagia in HIV patient

candida esophagitis

178

Tx candida esophagitis

cephalosporins, beta lactams or macrolides

179

Dx PCP

bilateral infiltrates on CXR
icreased LDH
sputum gram stain

180

Tx PCP

TMP SMX
corticosteroids

181

Dx TB in HIV

acid fast bacilli
cavitary defects
hilar adenopathy
+ PPD

182

Tx TB

RIPE

183

opportunistic infections CD4

histoplasmosis

184

Tx histoplasmosis

amphotericin B or itraconazole

185

opportunistic infections CD4

cerebral toxo
lymphoma
Progressive multifocal leukoencephalopathy
wasting syndrome

186

Dx toxo in HIV

+ toxo IgG Ab
ring enhacing lesion on CT or MRI

187

Tx cerebral toxo

pyrimethamine
sulfadiazine
clindamycin

188

Tx lymphoma (CNS or non hodgkin)

chemo radiation

189

Dx lymphoma in HIV

CT or MRI shows lesion
Bx confirms

190

focal neurologic Sx in HIV

cerebral toxo or lymphoma

191

signs of progressive multifocal leukoencephalopathy

ataxia, motor deficits, mental status changes

192

Dx PML

positive PCR for JC virus

193

opportunistic infections CD4

cryptococcul meningitis
CMV
myocobacterium

194

Dx cryptococcal meningitis

elevated P on LP
yeast on India ink stain of CSF
+ cryptococcal Ag in CSF or serum

195

Tx cryptococcal meningitis

amphotericin B, fluconazole

196

Sx CMV in HIV

vision loss and esophagitis and diarrhea

197

Dx CMV

viral titer, yellow infiltrates with hemorrhage on fundoscopic exam

198

Tx CMV in HIV

ganciclovir, foscarnet, valganciclovir

199

signs MAC

fatigue, weight loss, fever, diarrhea, abdominal pain, lymphadenopathy, HSM

200

Dx MAC

blood cultures

201

Tx MAC

clarithromycin, azithromycin, ethambutol, rifabutin, rifampin

202

labs for HIV

ELISA if + repeat
then western blot because high specificity

203

viral load indicates what in HIV

rate of degree progression
useful in detection of acute infection

204

HAART

2 NRTI
+ Protease inhibitor or NNRTI
low dose ritonavir can be added to increase Protease inhibitor

205

Antibiotic prophylaxis in HIV

TMP SMX when CD4

206

what are the NRTIS

abacavir
emtricitabine
lamivudine
zidovudine
tenofovir

207

What are the NNRTIs

efavirenz
etravirine
rilpivirine

208

what are the protease inhibitors

navirs

209

what are the integrase inhibitors

gravirs

210

what is the fusino inhibitor in HIV meds

enfuvirtide

211

what is the CCR5 antagonist in HIV Rx

maraviroc

212

how do NRTIs work

inhibit producitno viral genome and preven incorporation of viral dNA into host genome

213

adverse effects NRTIs

lactic acidosis
lipodystrophy
pancreatitis
HS ractions

214

adverse zidovudine

bone marrow toxicity

215

how do NNRTIs work

inhibit reverse transcriptase activity to prevent replication

216

adverse effects NNRTIs

rash

217

adverse efavirenz

neropsychiatric effects and is teratogenic

218

how do protease inhibitors work

interfere with viral replication to cause nonfunctional viruses

219

adverse effects protesase inhibitors

hyperglycemia
hyper TG
GI toxicity

220

side effect of atazanavir

hyperbili

221

how do integrase inhibitors work

inhibit final step of integration viral DNA into host

222

adverse effects integrase inhibitors

neutropenia, pancreatitis, hepatotoxicity
hyperglycemia

223

how do fusion inhibitors work

bind to gp41 inhibiting viral ability to fuse to CD4 membrane and enter cell

224

how do CCR5 antagonist work

inhibit CCR5 coreceptor on virus blocking entry to host cell

225

Tx pregnant HIV + woman

Tx to keep viral load low during pregnancy
zidovudine during labor
and again after birth at 6 weeks

226

can HIV + breast feed

no

227

65 year old with burning in hands and feet and intense itching after showering
large retinal veins and splenomegaly

polycythemia vera

228

labs in PCV

inc Hb Hct inc RBC mass inc WBC normal platelets
dec EPO
hyperceullular marrow

229

Tx PCV

serial phlebotomy
antihistamines
ASA
hydroxyura (BM suppression)

230

complications PCV

thombus formation, leukemia (acute or chonic myelogenous) stroke

231

msot common cause of inc RBC production

chronic hypoxia

232

MM

proliferation plasma cells

233

monoclonal gammopathy of undetermined significance puts one at risk for

MM

234

what is produced in MM

abnormal M protein from IgG and IgA heavy chaings and kappa gamma light chains (bence jones proteins)

235

Signs Sx MM

back pain, radicular pain, weakness, fatigue, weight loss, constipation, fractures, frequent infections, pallor and bone tenderness

236

labs in MM

dec Hb HCt WBC
inc BUn and Cr
Inc Ca
high M protein and bence jones proteins
increased plasma cells on BM Bx

237

imaging MM

punched out lesions on long bones and skull

238

Tx MM

radiation, cehmo, bone marrow transplant
repair fractures Tx infections

239

complications MM

renal failure, recurrent infections, hyperca, spinal cord compression
poor prognosis 2-3 year survival

240

most common CA in children

ALL

241

ALL

children 2-5 years old
whites>blacks
proliferation of lymphoid line- B cell precursors

242

labs in ALL

dec Hb Hct decreased platelets WBCs
increased uric acid, increased LDH

243

bone marrow Bx in ALL

abundant blasts
9:22 BCR-ABL phildelphia chromosome

244

signs Sx ALL

bone pain, infections, fatigue, DOE, easy bruising, fever, pallor, purpura, HSM and lymphadenopathy

245

Tx ALL

chemo and BM transplant

246

complications ALL

adulta have bad prognosis. kids have better
presence of philadelphia chromosome= worse prognosis

247

AML

both children and adults

248

Signs Sx AML

fatigue, easy bruising, DOE, frequent infections, arthralgias, fever, pallor, HSM, muscosal bleeding, ocular hemorrhages

249

labs in AML

dec Hb Hct
dec platelets WBCs

250

bone marrow Bx in AML

blasts of myeloid origin and stain with myeloperoxidase

251

blood smear in AML

myeloblasts with notched nuclei and AUER rods

252

Tx AML

chemo and BM transplant

253

complications AML

relapse common
DIC
long term survivial poor

254

CLL

proliferation mature B cells
elderly patients >65

255

Signs Sx CLL

fatigue, infecitons, night sweats, fever, lymphadenopathy, HSM

256

labs in CLL

increased WBC >100,000

257

blood smear CLL

small lymphocytes with smudge cells

258

Tx CLL

supportive chemo tadiation for bulky masses
splenectomy for splenomegaly

259

complications CLL

malignant B cells may for auto Ab leading to severe hemolytic anemia.

260

CML

mature meyloid cell proliferation in middle aged adults
radiation exposure

261

what occurs with CML after stable for a couple eyars

blast crisis- usually fatal

262

signs Sx CML

fatigue, weight loss, night sweats, splenomegaly,

263

labs in CML

WBC >100,000 with high neutrophils
decreased leukocyte alkaline phosphatase

264

BM Bx in CML

granulocyte hyperplasia with cytogenic analysis showing philadelphia chromosome

265

Tx for CML

chemo with imatinib
BM transplant in younger patients

266

complications CML

blast crisis

267

philedelphia chromosome

almost always CML
5% ALL

268

Hair cell leukemia

proliferation B cells in middle aged men

269

Signs Sx hairy cell leukemia

fatigue, infections, abdominal fullness, massive splenomegaly and no lymphadenopathy
NO fever or night sweats

270

labs in hairy cell leukemia

dec Hb Hct platelets and WBCs
BM show lymphocytes

271

Tx hairy cell leukemia

chemo

272

blood smear hairy cell leukemia

lymphocytes with hairy projections of cytoplasm

273

purpose of radiation therapy

necrose tumor cells and decrease tumor size

274

what are some radiation induced malignancies

thyroid
CML
sarcomas

275

adverse of chemoTx in general

bone marrow suppression, alopecia, GI upset, infertility, neurotoxicity, hepatotoxicity, skin changes, pulm fibrosis, cardiomyopathy, renal toxicity

276

which drugs cause free radical damage and cytotoxic alkylization of DNA and RNA

clyclophosphamide, chlorambucil, ifosfamide, mechlorethamine
carmustine and streptozocin
busulfan
thitepa andhexamtheylmelamine
dacarbazine

277

which drugs inhibit spindle proteins and stop mitosis

vinca alkaloids like cinblastine and vincristine and etoposide

278

which drugs cause cytotoxic polymerization

taxanes like paclitaxel and docetaxel

279

which chemo drugs interfere with enzyme regulation or dNA regulation

cytarabine, 5-fluoreuracil, MTX, mercaptopurine
carboplatin and cisplatin

280

risk factors for hemolytic disease of newborn

abortion, amniocentesis, third trimester bleeding

281

Tx for hemolytic disease of newborn

RhoD Ig within 72 hours of Rh+ fetus or anytime blood may have mixed

282

fanconi anemia

autosomal recessive bone marrow failure

283

signs of fanconi anemia

fatigue, DOE, infections
short stature, abnormal skin pigments(cafe au lait and hypopigmented areas)
horseshoe kidney and thumb abnormalities

284

labs in fanconi anemia

dec Hb Hct platelets and WBC
increased AFP
BM show hypocellularity

285

Tx fanconi anemia

Antibiotics, tranfusions, stem cell transplants, growth factors, androgens, corticosteroids

286

complications fanconi anemia

death as child or leukemia

287

diamond blackfan anemia

congenital pure red anemia from defect in erythroid progenitor cells

288

Signs Sx diamond blackfan

faitue dyspnea, cyanosis, pallor
craniofacial abnormalties, thumb abnormalities, heart murmurs, mental retardation and hypogonadism

289

labs in diamond blackfan

dec Hb Hct reticulocyte count
inc MCV
decreased activity in BM but with increased EPO

290

Tx for diamond blackfan anemia

transfusions, corticosteroids, BM transplant

291

Neuroblastoma

neural crest cell tumor in adrenal glands or sympathetic ganglia

292

risk factors Neuroblastoma

NF
tuberous sclerosis, pheo, beckwith wiedemann syndrome, turners, low maternal folate

293

signs Sx neuroblastoma

abdominal distention with pain, weight loss, malaise, bone pain,, diarrhea, abdominal maa, HTN, horners, proptosis, movement disorders, hepatomegaly, fever, periorbital bruising!

294

labs in neuroblastoma

inc VMA and homovanillic acid in 24 hour urine collection

295

radiology findings in neuroblastoma

CT locate adrenal or ganaglion tumor

296

Tx neuroblastoma

surgical resection, chemo radiation

297

complications neuroblastoma

poor prognosis if present afeter 1 year of life

298

mets of neuroblastoma

bone and brain

299

rhabdomyosarcoma

tumor striated mm in children

300

signs Sx rhabdomyosarcoma

painful soft tissue mass with swelling

301

Dx rhabdomyosarcom

Bx and CT shows extent

302

Tx rhabdomyosarcoma

surgical debulking, radiatoin, chemo

303

most common soft tissue sarcoma in children

rhabdomyosarcoma