HemeOnc Step Up Flashcards

(303 cards)

1
Q

what happens to O2 curve in alkalosis

A

decreased body temp, increased HbF shfit to left

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2
Q

what happens to O2 curve in acidosis

A

increased body temp
high altitude
exercise shift curve to right

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3
Q

what is a left shift fo O2 curve

A

increased Hb affinity

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4
Q

what is a right shift O2 curve

A

decreased affinity for O2. easier off loading

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5
Q

immediate Tx for CO poisoning

A

100% O2 mask

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6
Q

signs CO poisoning

A

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

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7
Q

causes of microcytic acnemia

A
iron deficiency
lead poisoning
chronic disease
sideroblastic
thalassemias
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8
Q

causes of normocytic anemia

A

hemolytic
chronic disease
hypovolemia

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9
Q

causes of macrocytic anemia

A

folate def
vit B12 def
liver disease
alcohol abuse

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10
Q

signs of hemolytic anemia

A

brown urine
HSM
jaundice

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11
Q

labs in hemolytic anemia

A

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

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12
Q

most common form anemia

A

iron deficiency

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13
Q

Coombs direct test

A

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

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14
Q

Coombs indirect test

A

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

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15
Q

schistocytes

A

seen in hemolytic anemia

RBC fragments

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16
Q

angular cheilitis

A

irritaiton of lips and corners of mouth

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17
Q

blood smear shows burr ceslls and schistocytes

A

Drug induced hemolytic anemia

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18
Q

wha drugs cause hemolytic anemia

A

penicillin, methyldopa

quinidine

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19
Q

warm reacting Ab are what

A

IgG

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20
Q

cold reacting Ab are what

A

IgM

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21
Q

Tx immune hemolytic anemia

A

corticosteroids

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22
Q

Coombs test is + when

A

drug induced hemolytic anemia and immune

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23
Q

finding in hereditary spherocytosis

A

HSM

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24
Q

Tx hereditary spherocytosis

A

splenectomy

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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