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Heme Flashcards

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1
Q

How do you tell the difference btw CML and leukemoid?

A

Look similar b/c increase WBC with early cells

  • -alkaline phosphatase is decreased in CML
  • -Need t(9,22) cytogenics to diagnose CML
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2
Q

pre-surg prophylax against DVT

A

low molecular weight heparin. helps antithrombin III

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3
Q

What do you use if u can’t use heparin

A

Direct thrombin inhibitors

  • -Argatroban
  • -Lepirudin
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4
Q

epstein barr causes which malignancy

A

Burkett’s lymphoma. Will showa high Ki-67 (proliferation index0

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5
Q

High AFP in a pregnant woman

A

Neural tube defect

–Can also be yolk sac tumor

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6
Q

Cladribine mechanism

A

purine analog that is incorporated into DNA causing strands to break.

Resistant to adenosine deaminase

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7
Q

Indication cladribine

A

hairy cell leukemia

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8
Q

Purine analogs

A

6-thiopurine
fludarabine
cladribine

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9
Q

Pyrimidine analogs

A

5-FU
capecitabine
cytarabine
gemcitabine

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10
Q

Factor V leiden factor pts at risk for

A

DVT–>pulmonary thromboembolism
cerebral vein thrombosis
pregnancy loss

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11
Q

Why remove spleen from SCD pts

A

At risk for splenic infarction

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12
Q

What causes heparin induced thrombocytopenia?

A

Antibodies to heparin. Results in paradoxical thrombosis or bleeding. More common with unfractionated (high molecular weight) heparin

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13
Q

What are the direct thrombin inhibitors?

A

hirudin, lepirudin and argatroban

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14
Q

AML translocation

A

t(8,21)

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15
Q

Acute promyelocytic leukemia (AML variant) translocation

A

t(15,17). Will look similar to AML but with more promyelocytes (Large, basophilic nucleus)

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16
Q

Burkitt lymphoma translocation

A

t(8,14)

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17
Q

CML translocation

A

t(9,22)

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18
Q

Mantle cell translocation

A

t(11,14)

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19
Q

CLL genetics

A

13q deletion

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20
Q

Describe the APL mechanism

A

Transfer of retinoic acid receptor alpha to the PML gene. Fusion of two gene products occurs

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21
Q

Side effect of dauno/doxorubicin

A

irreversible dose-dependent cardiotoxicity

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22
Q

Which drugs cause bone marrow suppression?

A

chloramphenicol, ziduvine, phenylbutazone

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23
Q

Which drugs cause liver necrosis?

A

acetaminophen and halothane

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24
Q

Which drugs cause pulmonary fibrosis?

A

Busulfan and bleomycin

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25
Pure red cell aplasia--what do you need to look our for
rare severe hypoplasia of marrow erythroid elements. Associated with thymoma, lymphocytic leukemia, and Parvo B19
26
c-myc
Burkitt lymphoma. Chromosome 8 | --It's a transcription activator of apoptosis and differentiation
27
what does t(11,14) do?
mantle cell on chromosome 11 - More Cyclin D1 - Increased G1-S
28
Bcl-2
follicular lymphoma t(14,18) chromosome 18 inhibits apoptosis
29
BCR-ABL
inhibits apoptosis
30
t(14,18)
follicular lymphoma
31
pathophys of follicular
Bcl2 overexpression
32
ristocetin aggregation test
measures vWF dependent platelet aggregation. Activates GP Ib-IX receptors so they can bind to vWF.
33
Does vWF cause changes in PT or PTT?
Yes--PTT (carries factor VIII)
34
Hageman factor XII
No clotting problems but prolonged PTT
35
glanzmann thrombasthenia testing
response to adenosine diphosphate (ADP), epinephrine, and collagen are decreased --ristocetin normal
36
STARY SKY APPEARANCE
BURKITT LYMPHOMA
37
CLOSELY PACKED LYMPH NODE FOLLICLES
FOLLICULAR LYMPHOMA
38
large sheets of lymphocytes with large nuclei
Large B cell lymphoma
39
BLASTS
AML OR ALL | --ALL in kids more likely and usually B Cell rather than T cell
40
Blasts in a kid presenting with a mediastinal mass causing SVC syndrome or stridor
T cell ALL
41
B12 deficiency signs
elevated methylmalonic acid AND homocysteine
42
Folate deficiency signs
elevated homocystein
43
How to prevent hemorrhagic cystitis with chemotherapy?
hydration and mesna, which binds acrolein, a toxic metabolite
44
What membrane protein allows RBCs to carry CO2?
Chloride HCO3 antiporter - -accounts for the chloride shift. HCO3 enters, CL- leaves in tissues - -chloride levels low in venous blood
45
1/3 of platelet pool is stored in
the spleen
46
platelets contain what granules
alpha granules | dense granules
47
alpha granules
vWF | Fibrinogen
48
delta granules
ADP, calcium
49
lifespan of a platelet
8-10 days
50
granulocytes
neutrophil, eosinmophil, basophil
51
mononuclear cells
monocytes, lymphocytes
52
Normal neutrophil count
50-60
53
Normal lymphocyte count
25-33
54
hypersegmented poly
vitamin B12/folate deficiency
55
neutrophil granules
``` alkaline phosphatase collagenase lysozyme lactoferrin azurophilic granules ```
56
azurophilic granules
acid phosphatase peroxidase B-glucuronidase
57
produces histaminase and arylsulfatase
eosinophils
58
causes of eosinophilia
``` neoplastic asthma alelrgic collagen vascular disease parasites ```
59
basophils release
heparin histamine leukotrienes
60
drug: prevents mast cell degran
cromolynsodium
61
mast cells release
histamine heparin eosinophil chemotaxis
62
bradykinin activates
vasodilation permeability pain
63
other action of XIIa
activates kallikrein from prekallikrein
64
kallidrein
HMWK-->bradykinin | plasminogen-->plasmin
65
action of plasmin
degrades fibrin | activates complement cascade
66
hemophilia A
deficiency factor VIII
67
hemophilia B
deficiency factor IX
68
warfarin inhibits
epoxide reductase (reduces vitamin K)
69
Protein C
works with protein S to cleave and inactivate Va and VIIIa
70
what activates protein C?
thrombomodulin
71
antithrombin inactivates
II,VII,IX, X, XI, XII
72
what activates antithrombin?
heparin
73
what starts coagulation cascade?
platelet release of ADP and Ca2+
74
What induces GpIIb/IIIa expression?
ADP binding
75
anti-aggregation factors
PGI2 (prostacyclin) | NO
76
Ticlopidine and clopidogrel mechanism
inhibits ADP induced expression of GpIIb/IIIa
77
what cell expresses thrombomodulin?
Protein C
78
what do endothelial cells produce?
vWF thromboplastin tPA PGI2
79
Acute phase reactants in plasma cause
RBC aggregation
80
Increase ESR
autoimmune dz neoplasms GI disease pregnancy/infection
81
decrease ESR
``` sickle cell anemia CHF microcytosis low fibrinogen polycythemia ```
82
Acanthocyte
liver disease | abetalipoproteinemia
83
basophilic stippling
thalassemias anemia of chronic disease lead poisoning Baste the tail
84
elliptocyte
hereditary elliptocytosis
85
bite cell
G6PD deficiency
86
macro-ovalocyte
megaloblastic anemia, marrow failure
87
ringed sideroblasts
excess iron storage | --sideroblastic anemia
88
schistocyte
DIC TTP/HUS traumatic hemolysis
89
TTP
thrombocytic thrombocytopenia purpura - -blood clots in all the small vessels - -thrombocytopenia (purpura) - -MAHA--schistocytes - -neuro sx - -kidney failure
90
spherocyte
hereditary spherocytosis | autoimmune hemolysis
91
teardrop cell
bone marrow infiltration
92
target cell
HbC Asplenia Liver dz Thallassemia HALT don't shoot the TARGET
93
heinz bodies
G6PD, | --Similar appearance in alpha-thalassemia
94
howell jolly bodies
asplenia | napthlalene (moth ball) ingestion
95
RBC smear in iron deficiency
microcytosis | hypochromia
96
plummer vinson syndrome
``` iron deficiency anemia esophageal webs atrophic glossitis (smooth tongue) ```
97
y4 Hb in pt with hydrops fetalis
4 gene deletion of alpha thalassemia
98
Very little alpha globin with Beta globin tetramers
HgbH disease - -caused by only one functional alpha gene - -anemia
99
1-2 gene deletion of alpha chain
no significant sx
100
cis deletion of alpha thalassemia more common in
azns
101
trans deletion of alpha thalassemia more common in
africans
102
beta thalassemia prevalent in
mediterranean population
103
increase in HbA2 (over 3.5%) in asymptomatic pt
beta thalassemia minor
104
chipmunk face, crew cut on skull Xray | --severe anemia requiring blood transfusions
beta thalassemia major (homozygote)
105
hemoglobin in beta thalassemia major
HbF (a2, y2)
106
blood smear in beta thalassemia major
``` anisocytosis poikilocytosis microcytosis hypochromia schistocytes ```
107
house with chipped paint
lead poisoning
108
first line tx for LEAD
Dimercaprol and EDTA
109
tx for lead poisoning in a kid
succimer
110
sx of lead poisoning
1. lead lines on gingivae 2. encephalopathy, erythrocyte basophilic stippling 3. abdominal pain, sideroblastic anemia 4. drops of wrist and foot
111
pt with anemia increased iron, ferritin, normal TIBC hereditary cause
hereditary sideroblastic anemia
112
reversible causes of sideroblastic anemia
alcohol, lead, isoniazid
113
tx hereditary sideroblastic anemia
pyridoxine B6
114
cause hereditary sideroblastic anemia
d-ALA synthase defect
115
inheritance of sideroblastic anemia
X linked
116
causes of microcytic anemia
``` iron deficiency ACD thalassemias lead poisoning sideroblastic anemia ```
117
causes of normocytic nonhemolytic anemia
ACD aplastic anemia chronic kidney disease
118
macrocytic megaloblastic anemia
folate deficiency b12 orotic aciduria
119
nonmegaloblastic macrocytic anemia
liver dz alcoholism reticulocytosis
120
anemia - -normal MMA - -increased homocysteine
folate deficiency
121
anemia - -increased homocysteine - -increased MMA
B12 deficiency
122
causes of B12 deficiency
diphyllobothrium latum --crohn's vegans PPIs
123
signs of subacute combined degeneration
peripheral neuropathy loss of vibration and proprioception spasticity (loss of lateral corticospinal) dementia
124
treatment for orotic aciduria
uridine monophosphate
125
which drugs cause macrocytic anemia?
5-FU, AZT, hydroxyurea. these all interfere with DNA synthesis, but NO megaloblastic RBCs
126
anemia - -decreased haptoglobin - -increased LDH - -hemoblogin in urine
intravascular hemolysis
127
anemia | --increased LDH and high unconjugated bili
extravascular hemolysis. Means you're losing RBCs through spleen clearance, not lysis in vessels
128
findings in anemia of chronic dz
1. increased hepcidin 2. ferritin 3. decreased TIBC 4. decreased iron
129
cause of aplastic anemia
1. radiation, benzene, chloramphenicol, alkylatin agents 2. viral: parvo, EBV, HIV, HCV 3. fanconi's 4. autoimmune
130
fanconi's
proximal renal tubules[1] of the kidney in which glucose, amino acids, uric acid, phosphate and bicarbonate are passed into the urine
131
dry bone marrow type showing hypocellular marrow with fatty infiltration
aplastic anemia
132
signs of aplastic anemia
pancytopenia
133
positive osmotic fragility test
hereditary spherocytosis
134
hereditary spherocytosis prone to asplastic crisis from infection by
parvo B19
135
back pain with hemoglobinuria days later. Heiz bodies and bite cells
G6PD
136
G6PD: intravascular or extravascular hemolysis?
mostly extravascular
137
hemolytic anemia in newborn
pyruvate kinase deficiency | --low ATP=rigid RBCs
138
HbC cause
glutamic acid to lysine mutation at residue 6.
139
hemolytic anemia, pancytopenia, and venous thrombosis | --CD55/59 negative RBCs on flow
Paroxysmal nocturnal hemoglobinuria | --increased complement-mediated RBC lysis
140
how do you get PNH?
acquired mutation in stem cell | --loss of GPI anchor or decay accelerating factor (protect RBC from complement
141
tx PNH
eculizumab
142
crew cut on skull x ray with severe anemia and sudden vasoocclusive crises
sickle cell
143
mutation in sickle cell
Substitution of glutamic acid with valine at position 6
144
SCD child suddenly showing howell jolly bodies
autosplenectomy
145
kidney problems in SCD
renal papillary necrosis
146
tx sickle ell
hydroxyurea or bone marrow transplant
147
warm agglutinin type of antibody
IgG
148
cold agglutinin type of antibody
IgM
149
Warm agglutinin seen in
chronic anemia with | SLE, CLL, drugs
150
cold agglutinin seen in
chronic anemia with - mycoplasma pneumonia - mono - CLL
151
pathophys MAHA
RBCs damaged b/c of narrow vessel lumen
152
cause of MAHA
DIC TTP-HUS SLE Malignant HTN
153
macroangiopathic anemia
mechanical destruction RBC (valves, aortic stenosis) | --schistocytes
154
iron lab values in hemochromatosis
increased serum iron increased ferritin decreased TIBC increased transferrin saturation
155
pregnancy/OCP iron tests
increased transferrin decreased transferrin saturation normal iron and ferritin
156
steps in heme synthesis
1. glycine+succinylCoa 2. d-ALA 3. Porphobilinogen 4. HMB 5. Uroporphyrinogen 6. coproporphytingoen 7. protoporphyrin 8. heme
157
dALA synthase requires what vitamin
b6
158
lead poisoning blocks
d-ALA dehydratase | ferrochelatase
159
sideroblastic anemia is a defect in
d-ALA synthase
160
Acute intermittent porphyria is a block in
porphobilinogen deaminase
161
Porphyria cutanea tarda is a block in
uroporphyringen decarboxylase
162
accumulated protoporph and dALA
lead poisoning
163
accumulated porphobilinogen dALA and uroporphyrin (tea colored urine)
acute intermittent porphyria
164
battery/ammunition/radiator factory worker
lead poisoning
165
``` painful abdomen port wine urine polyneuropathy psych disturbances precipitated by drugs ```
acute intermittent porphyria
166
tx AIP
glucose and heme--inhibit ALA synthase
167
blistering photosensitivity with uroporphyrin in the urine
porphyria cutanea tarda
168
defect in porphyria cutanea tarda
uroporphyrinogen decarboxylase
169
PT tests
extrinsic pathway (I, II, V, VII, X)
170
PTT tests
intrinsic pathway (everything but VII and XIII)
171
clotting results in hemophilia
Increased PTT (factor VIII and IX)
172
clotting results in vitamin K deficiency
Increased PT, PTT
173
bernard soulier labs
decreased PC | increased BT
174
glanzmann's labs
increased BT | no change in PC
175
blood smear shows no platelet clumping
glanzmanns
176
cause idiopathic thrombocytopenic purpura
anti-GpIIb/IIIa antibodies | --spleen consumes platelet/antibody complex
177
labs in ITP
decreased platelets increased BT increased megakaryocytes
178
TTP cause
deficiency of ADAMTS13 | --cannot separate vWF multipers causing platelet aggregation and thrombosis
179
labs in TTP
decrased PC Increased BT schistocytes increased LDH
180
tx vWF
DDAVP
181
labs vWF
increased BT | --if severe, increased PTT
182
inheritance vWF
autosomal dominant
183
DIC labs
1. low PC 2. high BT, PT, PTT 3. Schisto 4. D dimers 5. low fibrinogen 6. low factor V and VIII
184
mutation in prothrombin gene mutation
3' untranslated region=more prothrombin
185
pt has no increase in PTT after heparin administration
antithrombin deficiency
186
pt has thrombotic skin necrosis with hemorrhage after warfrin
protein C or S deficiency. | --cannot inactivate factors V and VIII
187
what is contained in cryoprecipitate?
``` fibrinogen factor VIII factor XIII vWF fibronectin ```
188
serum changes with blood transfusions
hypocalcemia | hyperkalemia
189
leukemoid rxn labs
increased WBC with left shift | INCREASED leukocyte alkaline phosphatase
190
CML labs
increased WBC with left shift | DECREASED leukocyte alk phos
191
typical spread of hodgkin's
localized - single group of nodes - spreads contiguously
192
typical spread non-hodgkin's
multiple peripheral nodes - extranodal involvement - does not spread through blood
193
strongest prognostic indicator in hodgkins
stage
194
hodgkin's associated with
EBV
195
non-hodgkin's associated with
HIV/immunosuppression
196
hodgkin age group
young adulhood and over 55 years. usually men
197
non-hodgkins age group
20-40 years
198
B symptoms more common in hodgkins or non-hodgkins
hodgkins
199
Reed sternberg cells are positive for
CD30 and CD15
200
Most common form of hodgkin's
nodular sclerosing
201
prognosis of nodular sclerosing
good. prognosis with lymphocyte mixed/depleted is poor
202
t(8,14)
burkitt's
203
cmyc
burkitt's
204
endemic form of burkitt's in africa
jaw lesion
205
sporadic burkitt's
pelvis/abdomen
206
most common adult NHL
diffuse large B cell
207
t(11,14)
mantle cell
208
cyclin D1
mantle cell
209
t(14,18)
follicular
210
bcl02
follicular
211
which NHLs can be seen in children
classically burkitt's | 20% of diffuse large B cell lymphomas
212
CD5+ NHL
mantle cell
213
prognosis follicular lymphoma
difficult to cure | --indolent course
214
prognosis mantle cell
poor
215
adult T cell lymphoma caused by
HTLV-1
216
sx: adult T cell lymphoma
cutaneous lesions
217
adult t cell lymphoma affects people from
Japan west africa caribbean
218
cutaneous patches/nodules with CD4+ cells
mycosis fungoides/sezary
219
rouleaux formation
multiple myeloma
220
histology multiple myeloma
plasma cells with clock face chromatin--lots of em | --intracytoplasmic inclusions, white
221
waldenstrom's macroglobulinemia
M spike without lytic bone lesions
222
Risk of MM in MGUS
1-2% per year
223
ALL affects what age group
less than 15 years
224
mediastinal mass
T cell ALL
225
tdT and CALLA positive
ALL
226
Which ALL has the best prognosis?
t(12, 21)
227
smudge cells
small lymphocytic lymphoma/chronic lymphocytic leukemia
228
SLL and CLL typically affect what age group
>60 years
229
presentation of SLL/CLL
asymptomatic. autoimmune hemolytic anemia
230
TRAP positive
hairy cell leukemia
231
tx: hairy cell leukemia
Cladribin
232
t(15,17)
M3 type AML
233
median age of AML
65
234
auer rods
AML
235
Common presentation of M3 AML
DIC
236
tx: M3 AML
all trans retinoic acid
237
CML age group
30-60
238
t(9,22)
CML
239
blood mear in CML
increased neutrophil metamyelocytes basophils splenomegaly
240
CML can transform to
AML/ALL (blast crisis)
241
AMl-M3 subtype also known as
acute promyelocytic leukemia
242
peroxidase positive cytoplasmic inclusions
auer rods
243
child with lytic bone lesions and skin rash. cells show lots of birbeck granules on EM
langerhans histiocytosis
244
pathophysiology of langerhans histiocytosis
functionally immature dendritic cells cannot stimulate T lymphocytes.
245
Cells in langerhans histiocytosis express
S-100 and CD1a
246
JAK2 positive
myeloproliferative disorder -polycythemia vera essential thrombocytosis myelofibrosis
247
RBC, WBC, platelets in polycythemia vera
All increased
248
RBC, WBC, platelets in essential thrombocytosis
platelets increased
249
RBC, WBC, platelets in myelofibrosis
Low RBC. variable wbc and platelets
250
RBC, WBC, platelets in CML
low RBC lots of WBC and platelets
251
Lymphoid lineage
B cells T cells NK cells
252
relative polycythemia
decreased plasma volume (dehydration)
253
appropriate polycythemia
increased RBC 2ndary to O2 saturation
254
inappropriate absolute
increased RBC mass caused by 1. RCC, wilms' tumor 2. HCC 3. Hydronephrosis 4. OR ectopic EPO

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