Heme - Ewwwww Flashcards
1
Q
precursor of granulocytes
A
myeloblast
2
Q
immediate precursor to neutrophil
A
band cell
3
Q
precursor to platelets
A
megakaryocyte
4
Q
kidney shaped nucleus
A
monocyte (macrophage in blood)
5
Q
releases major basic protein
A
eosinophils
6
Q
WBC where granules cover nucleus
A
basophil
7
Q
Wright - Giemsa stain stains what?
A
AT rich segments of DNA
8
Q
mutation in ankyrin, spectrin, or band 3 - disease and type of hemolysis
A
hereditary spherocytosis - extravascular
9
Q
increased osmotic fragility of RBCs (disease and morphological finding)
A
Hereditary spherocytosis - Howell Jolly body
10
Q
Heinz bodies (2 diseases stain used)
A
G6PD Deficiency and alpha thalassemia - supravital (methylene blue or bromocresol green)
11
Q
Hb values for men and women in anemia
A
male: < 13.5 female: < 12.5
12
Q
MCV: microcytic, normal, macrocytic
A
micro: < 80 normal: 80-100 macro: >100
13
Q
Name components of Hb and diseases from the deficiencies
A
Heme (iron + protoporphyrin): iron def. anemia, anemia of chronic disease (Fe stuck in macrophages)
Protoporphyrin: sideroblastic anemia
Globin: thalassemia
14
Q
most common nutrional deficiency in the world
A
iron
15
Q
where is iron absorbed
A
duodenum
16
Q
molecule that transports iron in blood
A
transferrin
17
Q
molecule that stores iron and where?
A
ferritin in liver and bone marrow macrophages
18
Q
what is TIBC
A
total iron-binding capacity, measures transferrin molecules in blood (%saturation tells % of transferrin bound by iron)
always opposite of ferritin
19
Q
common iron deficiencies by age group: infant/children, adults, elderly
A
infants/children: diet
adults: peptic ulcer disease (males) and menorrhagia (females)
elderly: colon polyps/carcinoma (developed) hookworms in (developing)
20
Q
consequences on gastrectomy on iron absorption
A
decreased acid production, more iron in Fe+3 state, less absorption
21
Q
signs of iron deficiency
A
paleness, koilonychia, pica
22
Q
RDW and what is it called when its high?
A
red cell distribution width (spectrum of size of RBCs)
wide distribution is called anisocytosis
23
Q
esophageal web, atrophic glossitis, beefy-red tongue
A
Plummer-Vinson syndrome - iron deficiency anemia
24
Q
anemia assoc. with chronic inflammation (disease, type of anemia, and most common population)
A
anemia of chronic disease - microcytic - hospitalized pts
25
acute phase reactant from liver that sequesters iron (name and why)
hepcidin - to prevent bacteria from accessing iron
26
ringed sideroblasts: disease - enzyme - causes
sideroblastic anemia - ALA synthase - alcoholism, lead poisoning, vit B6 def
27
ferritin: low
TIBC: high
serum iron: low
% sat: low
iron def. anemia
28
ferritin: high
TIBC: low
serum iron: low
% sat: low
anemia of chronic disease
29
ferritin: high
TIBC: low
serum iron: high
% sat: high
sideroblastic anemia
30
protection from plasmodium falciparum (3 diseases)
thalassemia
sickle cell trait
G6PD Deficiency
31
HbA
alpha2,beta2
32
HbA2
alpha2, delta2
33
HbF
alpha2, gamma2
34
HbH - structure and disease and chromosome (# and mutation)
Beta tetramer - alpha thalassemia (3 genes deleted) - chromosome 16 - deletion
35
Hb Barts - structure and disease and chromosome (# and mutation)
Gamma tetramers - alpha thalassemia (4 genes deleted) hydrops fetalis - chromosome 16 - deletion
36
B0 and B+ (disease, type of mutation, chromosome # - meaning)
Beta thalassemia - point mutation in promotor or splicing site - chromosome 11 - B0: absent production, B+: diminished production
37
target cell: disease(s) and type of anemia
```
B-thalassemia minor (B/B+) - microcytic, hypochromic
Sickle Cell (intravascular hemolysis)
```
38
aplastic crisis with parvo B19 (3)
B-thalassemia major
Hereditary spherocytosis
Sickle cell
39
crewcut appearance on x-ray
B-thalassemia major, sickle cell
40
chipmunk facies
B-thalassemia major, sickle cell
41
no HbA, inc HbA2, inc HbF
B-thalassemia major
42
hypersegmented neutrophil
macrocytic anemia >5 lobes
43
magaloblastic anemia (deficiencies)
B12 and folate
44
where is folate absorbed?
jejunum
45
macrocytic anemia, glossitis, inc. homocysteine, normal methylmalonic acid (disease and neural state)
folate deficiency - no neuro symptoms
46
where is B12 absorbed?
ileum
47
pernicious anemia: most common cause of what? diease and explanation
vit B12 deficiency - autoimmune destruction of parietal cells, less intrinsic factor, less B12 absorption
48
Schilling test
tests for pernicious anemia - can't absorb labeled B12 and it gets excreted
49
diphyllobothrium latum leads to?
vit B12 deficiency
50
inc. methylmalonic acid - disease and consequences
vit B12 deficiency - cant be converted to succinyl CoA, builds up and integrates into spinal cord myelin - loss of proprioception and vibratory sense - also spastic paresis
51
2 causes of normocytic anemia
inc peripheral destruction or underproduction
52
reticulocyte - what is it, what does it look like?
young RBC released from bone marrow - larger cells with bluish cytoplasm (due to residual RNA)
53
high reticulocyte count (>3%) in anemia, explain why and how to fix it
RC falsely elevated due to decrease in total mature RBCs (so % of RC goes up) - correct it by multiplying RC by (hematocrit/45)
54
unconjugated bilirubin is the breakdown product of what? (specifically)
protoporphyrin
55
Bone marrow erythroid hyperplasia, increased unconj. bilirubin, decreased serum haptoglobin, hemoglobinuria
hemolytic anemia
56
Howell-Jolly Bodies
hereditary spherocytosis
sickle cell
hemolytic anemias
57
mutation in sickle cell: gene, type, products
B chain of Hb - point mutation - glutamic acid replaced by valine
58
poikilocytosis: shape and disease
pencil cells - severe iron deficiency anemia and beta major thalassemia
59
HbS
two abnormal Bchain Hb genes - sickle cell
60
how does hydroxurea work? (mechanism and disease)
raises HbF levels, helps with sickle cell and beta thalassemia major
61
dactylitis, autosplenectomy, howell jolly bodies, hematuria and proteinuria
sickling crisis
62
most common cause of death in children with sickle cell
infection from encapsulated organisms (strep penumonia and H. influenzae) due to autosplenectomy
63
most common cause of death in adults with sickle cell?
acute chest syndrome: chest pain, pneumonia, lung infiltrates, SOB from thrombosis
64
affects of sickle cell TRAIT on kidney?
only sickles in renal medulla (microinfarcts) leading to microscopic hematuria and decreased ability to concentrate urine
65
what is the metabisulfite screen used for?
causes HbS to sickle in any amount, positive in disease and trait
66
HbC crystals on blood smear: mutation in what gene and what products?
Beta chain of Hb - glutamic acid replaced by lysine (Ly-C-ine)
67
RBCs more susceptible to destruction by complement, episodic intravascular hemolysis, hemoglobinuria and -emia (disease, underlying problem, explanation of episodic)
Paroxysmal nocturnal hemoglobinuria (PNH)
mutation in PIGA gene (makes GPI: anchoring protein for DAF) DAF protects against complement-mediated damage by inhibiting C3 convertase - no GPI, no DAF
Happens at night because of shallow breathing in sleep, drop in pH from CO2 which activates complement
68
sucrose test screens for what disease?
Paroxysmal nocturnal hemoglobinuria (PNH)
69
acidified serum test confirms what disease?
Paroxysmal nocturnal hemoglobinuria (PNH)
70
CD55 is also known as? absent in what disease?
DAF (decay accelerating factor) and PNH
71
complications of PNH?
iron deficiency anemia
| AML (10%)
72
Forms of G6PD: name, severity, type of hemolysis
| Also normal form
African: G6PDA- mild intravascular hemolysis
Mediterranean: severe intravascular hemolysis
Normal: G6PD B
73
exacerbated with fava beans, sulfa drugs
| Disease and common complaint
G6PD deficiency - back pain (Hb is nephrotoxic)
74
enzyme studies for G6PD deficiency should be performed when? why?
weeks after hemolytic episode. During crisis all cells without G6PD are destroyed so all the cells screened during the crisis have the enzyme
75
IgG mediated destruction of RBCs is what kind of hemolysis?
Extravascular
76
IgM mediated destruction of RBCs is what kind of hemolysis?
Intravascular
77
IgG mediated destruction of RBCs is what kind of agglutinin?
warm
78
IgM mediated destruction of RBCs is what kind of hemolysis?
cold
79
what disease do you treat with IVIG and why?
IgG mediated hemolytic anemia so spleen eats foreign IgG instead of IgG deposited on native RBCs
80
IgG mediated hemolytic anemia is associated with what other diseases/drugs? (3)
SLE (most common)
CLL
penicillin
81
IgM mediated hemolytic anemia is associated with what disease?
Mycoplasma pneumonia
| infections mononucleosis
82
Direct Coombs test: method, what it confirms, disease
Anti-IgG added to patient's RBC (agglutination if RBCs are coated with Ab)
presence of antibody-coated RBCs
Immune hemolytic anemia
83
Indirect Coombs test: method and disease
Anti-IgG and test RBCs are mixed with pt serum (agglutination occurs if serum Abs are present)
presence of Ab's in pt's serum
Immune hemolytic anemia
84
bite cell: how is it formed?
macrophages in spleen remove Heinz bodies from RBCs
85
schistocytes: how are they formed? Name a few diseases
shearing forces in circulation
| DIC, prosthetic heart valves, aortic calcification (stenosis)
86
parvovirus B19 infects what cells? leading to what?
Progenitor red cells - halts erythropoiesis - aplastic crisis in people with preexisting marrow stress
87
Diseases leading to dry tap
aplastic anemia
Hairy cell
myelophthisic process
88
viruses leading to aplastic anemia
Hepatitis (not A B C G)
CMV
EBV
herpes zoster
89
Outcome of primary hemostasis
weak platelet plug
90
Outcome of secondary hemostasis
stabilization of platelet plug by coagulation cascade
91
step of primary hemostasis mediated by reflex neural stimulation and endothelin release
step 1: transient vasoconstriction
92
Weibel-Palade bodies contain what substances?
vWF
| P-selectin
93
what does von willebrand factor (vWF) bind to and with what receptor?
exposed subendothelial collagen
| GP1b receptor
94
where does vWF come from? (2 locations)
Weibel-Palade bodies in endothelial cells
| alpha-granules of platelets
95
dense granules in platelets release what to expose what?
ADP to expose GPIIb/IIIa
96
what is the role of TXA2 in primary hemostasis and how is it made?
platelet aggregation
| made by COX
97
what molecule binds platelets in a platelet plug?
fibrinogen
98
petechiae are a sign of?
thrombocytopenia (they are not seen in qualitative disorders)
99
mucosal and skin bleeding, hemoptysis, hematuria are features of what?
disorder in primary hemostasis
100
autoimmune production of IgG against platelet antigens, what disease?
Immune thrombocytopenic purpura (ITP)
| most common cause of thrombocytopenia in adults and children
101
acute Immune thrombocytopenic purpura (ITP)
| who? cause? outcome?
children
viral infection or immunization
self-limited
102
chronic Immune thrombocytopenic purpura (ITP) who? cause? outcome?
adults (women)
assoc with SLE
shortlived thrombocytopenia in offspring (IgG can cross placenta)
103
decreased platelets
normal PT/PTT
increased megakaryocytes
Immune thrombocytopenic purpura (ITP)
104
treatment of Immune thrombocytopenic purpura (ITP)?
IVIG to raise platelet count
| splenectomy (removes source of Ab and site of platelet destruction)
105
Decrease in ADAMTS13: disease, major system affected, and normal function of enzyme
thrombotic thrombocytopenia purpura (TTP)
CNS
normally cleaves vWF
106
pathologic formation of platelet microthrombi in small vessels? Disease and blood smear findings
microangiopathic hemolytic anemia
| schistocytes (from shearing across microthrombi)
107
Disease associated with E. coli O157:H7 from undercooked beef? Common to see fever and renal insufficiency. Findings?
hemolytic uremic syndrome (HUS)
| platelet microthrombi and schistocytes
108
GPIb deficiency: disease, what type of platelet disorder, and blood smear findings
Bernard-Soulier syndrome (BS "Big Suckers")
qualitative
thrombocytopenia (platelets destroyed because they dont have GPIb)
enlarged platelets
109
GPIIb/IIIa deficiency: disease, what type of platelet disorder, and what is impaired
Glanzmann thrombasthenia
qualitative
platelet aggregation
110
what does thrombin do and where does it come from?
converts fibrinogen to fibrin
| end product of coagulation cascade
111
what four compounds can activate the coagulation cascade?
Tissue Thromboplastin (VII - extrinsic)
Subendothelial collagen (XII - intrinsic)
phospholipid surface of platelets
calcium (from platelet dense granules)
112
excess bleeding post surgery or wisdom tooth removal indicates what kind of disorder?
problem with secondary hemostasis (coagulation cascade)
113
males presents with history of joint bleeding and post-surgical bleeding. Lab findings show elevated PTT and decreased levels of FVIII. What disease, what pathway is affected?
```
Hemophilia A (8)
intrinsic
```
114
factor IX deficiency, 2 names
Hemophilia B
| Christmas disease
115
you expect a defect with factor VIII. Upon mixing of normal plasma with patient's plasma you see that the PTT does not return to normal. What disease? and what disease would it return to normal?
Anti-FVIII Ab's - ab's destroy new factor VIII from donor plasma too
It corrects in hemophilia A because the deficient factor is replaced
116
Your pt recently had a large-volume transfusion. What effect do you except it to have on his hemostasis abilities?
transfusion dilutes coagulation factors, results in a relative deficiency
117
which coagulation factors require vit K gamma carboxylation?
II VII IX X protein C and S
118
what is the major source of vit K? when would this be a problem (3 instances)?
normal flora in the gut
newborns with sterile colon, get vit K injection at birth
long term antibiotics: disrupt vit K producing bacteria
malabsorption: vit K is a fat sol vit
119
What can heparin do to platelets? Complication? What don't you treat with and why?
Cause formation of IgG Ab's that leads to platelet destruction.
fragments of destroyed platelets can activate remaining platelets leading to thrombosis
Don't treat with warfarin -> skin necrosis
120
Is DIC a primary or secondary disease process? Name 5 causes.
almost always secondary
1. pregnancy complications: thromboplastin in amniotic fluid can activate coagulation
2. sepsis: endotoxin from E coli or N meningitidis
3. adenocarcinoma: mucin activates coagulation
4. AML: primary granules in Auer rods can activate coagulation
5. rattlesnake bite: venom -> coagulation
121
Elevated D-dimer indicates what disease? How is it formed?
best screening test for DIC
| degradation product of cross-linked fibrin
122
How is plasmin formed and what does it do?
| What inactivates it?
converted from plasminogen by tissue plasminogen activator (tPA)
It cleaves fibrin and serum fibrinogen, destroys coagulation factors, and blocks platelet aggregation (prevents clotting!)
alpha2-antiplasmin inactivates
123
pt. has had radical prostatectomy and presents with increased bleeding. D-dimers are not present. What disease is this and what is another cause of it?
Disorder of fibrinolysis (removal of thrombus after damaged vessel heals)
urokinase from prostatectomy activates plasmin
resembles DIC
can also be caused by liver cirrhosis due to reduced production of alpha2-antiplasmin
124
lines of Zahn are indicative of what? what are they made out of?
thrombosis
alternating layers of platelets/fibrin and RBCs
125
Virchow's triad (3)
stasis
endothelial cell damage
hypercoaguability
126
prostacyclin (PGI2) does what?
vasodilation and inhibition of platelet aggregation
127
how does thrombomodulin work and where does it come from?
secreted by endothelial cells to prevent thrombosis normally
it makes thrombin activate protein C which inactivates factors V and VIII
128
elevated homocysteine causes what problems and how do you get it at elevated levels (2 ways, one mild, one high levels (give assoc. signs))
it damages endothelial cells
mild elevation seen in folate or vit. B12 deficiencies
high levels seen in cystathionine beta synthase (CBS) deficiency (thrombosis, mental retardation, lens dislocation, long slender fingers)
129
3 common sites of DVTs
deep leg veins
hepatic vein
cerebral vein
130
explain the pathogenesis of warfarin skin necrosis starting with the common deficiency that leads to it. Explain how to handle it.
Protein C or S deficiency (autosomal dominant)
C and S usually inactivate V and VIII. giving warfarin results in a temporary deficiency of proteins C and S relative to other factors (due to C and S having a shorter half life). Protein C is an anticoag so when it is out of balance with the coagulation factors, you clot.
If C and S deficiency, go-give heparin (to prevent clot) until old factors II, VII, IX and X degrade so all the factors are at the same level and C and S/
131
Most common inherited cause of hypercoagulable state? how does it lead to hypercoaguability?
Factor V Leiden
mutated factor V lacks cleavage site for deactivation by proteins C and S
132
what is the mutation in prothrombin 20210A and why is it a problem?
point mutation in prothrombin -> increased gene expression
increased prothrombin leads to increased thrombin -> thrombus formation
133
explain the relationship between ATIII deficiency and heparin including mechanism of action and treatment options
heparin normally activates ATIII which in turn inactivates thrombin and coagulation factors
in the deficiency, PTT does not rise with standard heparin dosing (heparin normally works by activating ATIII)
treat with high heparin to activate limited ATIII then give warfarin to maintain anticoag state
134
how do oral contraceptives lead to a hypercoagulable state?
estrogen increases production of coagulation factors
135
cholesterol clefts are indicative of what?
atherosclerotic embolus
136
pt with fracture presents with dyspnea and petechiae, why?
fat embolus
137
what is caisson disease and how is it treated?
chronic form of bends with gas emobli leading to multifocal ischemic necrosis in bone
decompression chamber
138
name a complication of laparoscopic sugery
gas embolus - air pumped into abdomen
139
3 common sites for thromboembolism and where can they embolize to?
femoral, iliac, popliteal veins
lungs
140
pt presents with SOB, hemoptysis, V/Q mismatch and elevated D-dimer. what is causing the symptoms? what is the cause of death in this pt
pulmonary embolism
cardiac failure due to electromechanical dissociation in heart
141
pt presents with bleeding defects and has thin superficial vessels, what disease?
hereditary telangiectasia
142
CD34 is a marker for what cells?
hematopoietic stem cells
143
how do neutrophil numbers change in a severe infection like gram-negative sepsis?
neutropenia - increased movement of neutrophils into tissues
144
which type of circulation cell is most sensitive to radiation?
WBC
145
Name four instances leading to lymphopenia and give an example of each
immunodeficiency: DiGeorge
high cortisol: cushing syndrome
autoimmune destruction: SLE
radiation: cancer therapy
146
which CD decreases in left shift and what is it?
CD16 - Fc receptor
147
In what cells do you see Dohle bodies and under what conditions? What color are they?
neutrophils during sepsis
| sky blue
148
name two conditions leading to monocytosis
malignancy and SLE
149
name 3 causes of eosinophilia
```
allergic type I hypersensitivity reactions
parasitic infections
Hodgkin lymphoma (produces IL-5)
```
150
name the most common condition leading to basophilia
Chronic Myeloid Leukemia (CML)
151
What is the only bacteria that leads to lymphocytic leukocytosis?
Bordetella pertussis
152
What cells (specifically) are elevated with EBV infection?
CD8+ T cells
153
Name the 3 most common sites of EBV infection
oropharynx
Liver
B cells
154
Describe the monospot test:
what antibodies?
how long to get a positive result?
what does a negative/positive test indicate?
detects IgM Abs
positive within 1 week
Negative test: CMV
Positive test: EBV
155
Exposure to ampicillin creates a rash in what infection?
EBV
156
You advise your patient to avoid contact sports for a year. What infection does he have and why would you do this?
EBV - increased risk for splenic rupture
157
In what cells does the EBV virus lay dormant and name two risks from this.
Recurrence
| B-cell lymphoma (usually after aquiring immunodeficiency)
158
What cells are atypical in EBV infection and what do they look like?
Lymphocytes
| Look like monocytes, but are just CD8+ cells with extra cytoplasm and enlarged nuclei
159
what cells have punched out nucleoli?
blast cells
160
Patient presents with fatigue, bleeding, and infection. What do they have and explain the mechanism
Acute leukemia
Increased blast cells in the marrow "crowd out" normal hematopoiesis
fatigue from anemia, bleeding from thrombocytopenia, infection from neutropenia
161
What area of the lymph node contains T cells? B cells?
T: paracortex
B: cortex
162
what cells are in the medulla of a lymph node?
dendrocytes, histiocytes, macrophages
163
What does your WBC count look like in acute leukemia?
high - lots of blasts in blood
164
Positive TdT staining indicates what disease? What cells are TdT negative?
Acute Lymphoblastic leukemia (ALL)
| Negative in myeloid blasts and mature lymphocytes
165
what disease is associated with Down's syndrome and has an onset after 5 years?
Acute lymphoblastic leukemia (ALL)
166
name the 3 CD markers in the most common form of ALL
B-ALL
| CD10, CD19, CD20
167
t(12,21) - disease, prognosis, age group
ALL - good - children
168
t(9,22) - diseases, prognosis, age group
ALL - bad - adults
| CML
169
Name the CD markers for T-ALL
CD2-CD8
| NOT CD10
170
Patient presents with bone pain, enlargement of LN, spleen, and liver and a mediastinal mass. What is the disease and what 2 areas must be treated directly? What age group does this affect most commonly?
T-ALL
scrotum and CSF
teenagers
171
Myeloperoxidase positive staining indicates what disease?
Acute myeloid leukemia
172
What are Auer rods made of and in what disease are they seen?
crystal aggregates of MPO
| AML
173
What disease is associated with Down's syndrome and has an onset before age 5?
AML (acute megakaryoblastic leukemia subtype)
174
t(15,17) what disease? what is the protein involved? age group? treatment?
AML (acute promyelocytic leukemia - APL)
retinoic acid receptor (RAR) on chromosome 17
older adults 50-60
all-trans retinoic acid
175
Primary granules in abnormal promyelocytes increase the risk for what?
DIC
176
proliferation of monoblasts, lack MPO - what disease and common site of involvement?
AML (acute monocytic leukemia)
| infiltrate gums
177
Name the 3 subsets of acute myeloid leukemia and their MPO status
Acute promyelocytic leukemia - MPO+
Acute monocytic leukemia - MPO-
Acute megakaryoblastic leukemia - MPO-
178
smudge cell
Chronic lymphocytic leukemia (CLL)
179
CLL what cells and what CDs?
B cells
CD5 (normally on T cells!)
CD20
180
most common adult leukemia, two names
CLL or SLL
181
most common cause of death in CLL? why? complication?
infections from hypogammaglobulinemia
neoplastic cells dont produce Igs
Can make Abs against own RBCs -> autoimmune hemolytic anemia
182
CLL can transform into what? signs?
Diffuse large B-cell lymphoma
| enlarged lymph node or spleen
183
CD markers for hairy cell?
CD11c, CD25, CD103+, CD20
184
tartrate-resistant acid phosphatase
Hairy cell leukemia
185
kidney shaped nuclei with "fried egg" appearance
hairy cell
186
accumulation of cells in red pulp of spleen... cells and disease
hairy cells
| hairy cell leukemia
187
proliferation of naive B cells?
CLL
188
proliferation of mature B cells?
hairy cell leukemia
189
HTLV-1 - assoc disease and type of cell involved
Adult T-cell leukemia/lymphoma (ATLL)
| CD4+
190
Japanese patient, skin rash, lymphadenopathy, hepatosplenomegaly, hypercalcemia. Disease and 1 other common finding.
Adult T-cell leukemia/lymphoma (ATLL)
| lytic (punched-out) bone lesions
191
lytic punched out bone lesions
```
Adult T-cell leukemia/lymphoma (ATLL) (with rash)
Multiple myeloma (vertebral column)
```
192
Name the 4 myeloproliferative disorders and what cells are predominantly involved
Chronic Myeloid Leukemia (CML) - Mature granulocytes
Polycythemia Vera (PV) - RBCs
Essential Thrombocythemia (ET) - platelets
Myelofibrosis - megakaryocytes
*All mature myeloid cells are elevated, distinction based on predominant cell type
193
Three complications of myeloproliferative disorders and why they happen
hyperuricemia and gout (high turnover of cells)
marrow fibrosis (spent phase)
transformation to acute leukemia
194
BCR-ABL fusion
| Translocation, disease, what cell type is predominant
t(9,22) - CML - basophils
195
Imatinib (Gleevec) - disease it treats and how
CML - blocks tyrosine kinase activity (which is increased with t(9,22) BCR-ABL fusion
196
3 drugs to treat CML
Imatinib
IFN-gamma
Hydroxyurea
197
Is CML negative or positive for Leukocyte Alkaline Phosphatase (LAP) stain? Why is LAP generated and it is used to distinguish which two processes?
negative
LAP is generated to fight infection
raised in leukemoid reaction (absent in CML)
198
CML can progress to what?
AML (2/3 of the time)
| ALL (1/3)
199
JAK2 kinase mutation (3)
```
Polycythemia vera (PV)
Essential thrombocythemia (ET)
Myelofibrosis
```
200
blurry vision, venous thrombosis, flushed face, itching after bathing
polycythemia vera (PV)
201
How do you distinguish PV from reactive polycythemia?
Check EPO levels (decreased in PV, elevated in reactive)
202
Two treatments for polycythemia vera (PV)?
phlebotomy: to decrease RBC count
| hydroxyurea
203
Budd Chiari - what is it and what is it associated with
```
Hepatic vein thrombosis
Polycythemia Vera (PV)
```
204
What disease shows excess PDGF?
Myelofibrosis
205
Tear drop RBCs
Myelofibrosis - RBCs get stretched trying to leave fibrotic bone marrow
Beta-thalassemia major
iron deficiency anemia
206
gout, splenomegaly, tear-drop RBCs, frequent infections, bleeding and thrombosis disease and why each happens
Myelofibrosis
splenomegaly from extramedullary hematopoiesis
tear drop RBCs: RBCs get stretched trying to leave fibrotic bone marrow
last 3: bone marrow can't produce enough other cells, megakaryocytes crowding -> fibrosis
207
painful lymphadenopathy seen in?
acute infection
208
painless lymphadenopathy
chronic inflammation, metastatic carcinoma, lymphoma
209
follicular hyperplasia of lymph node, what cells, what disease?
B-cell
| rheumatoid
210
paracortex hyperplasia of lymph node, what cells, what disease?
T-cell
| viral infections
211
B-cell region of cortex of lymph node: give regions
follicle: inner
mantle: around follicle
margin: around mantle
212
hyperplasia of sinus histiocytes (in lymph node) what does it indicate
lymph node is draining a tissue with cancer
213
What kind of cancer produces rash? (generally)
T cell
214
which B cells are more differentiated?
Small
215
intermediated sized B cell lymphoma
Burkitt
216
t(14,18) disease and genes involved
follicular lymphoma
| BCL2 on 18 and Ig heavy chain on 14
217
rituximab treats what disease and what is it's target?
follicular lymphoma
| anti-CD20
218
BCL2 inhibits what?
apoptosis
219
tingible body macrophages are used to distinguish what from what?
they are present in follicular hyperplasia, absent in lymphoma
220
t(11,14) what disease what genes
Mantle cell lymphoma
| Cyclin D1 on 11, Ig heavy chain on 14
221
how do you treat MALToma?
treat H. pylori infection
222
which lymphoma is assoc with chronic inflammatory states?
marginal zone lymphoma like Hashimoto thyroiditis, sjogren, H pylori
223
which cancers can progress to diffuse large B-cell lymphoma (2)
follicular lymphoma
| CLL
224
extranodal mass, African child. disease, translocation, genes, appearance on microscopy, associated virus
```
Burkitt's lymphoma
t(8,14)
c-myc on 8, Ig heavy chain on 14
starry sky
EBV
```
225
fever, weight loss, night sweats, most common form of non-Hodgkin's lymphoma. Disease, how it grows, age group
diffuse large B-cell lymphoma
grows in sheets
late adulthood
226
a chinese man presents with a translocation of t(8,14), where is his mass?
abdomen - sporadic burkitt's lymphoma
227
Reed-sternberg cells
Hodgkin lymphoma
228
mirror imaged nuclei - cell type and disease
Reed-sternberg - Hodgkin
229
CD15 and CD30
Classical Hodgkin - no 20, still B cell
230
CD45+, CD20+, CD15-, CD30-
| disease and common morphological finding
Variant Hodgkin
| popcorn cell
231
popcorn cell - disease and subtype
Variant Hodgkin - lymphocyte rich
232
lacunar cells - disease, subtype, typical patient
nodular sclerosis Hodgkin lymphoma, young female
233
Name the 4 subtypes of hodgkin lymphoma and give the best and worst prognosis
```
Nodular Sclerosis
lymphocyte rich (variant) (best prognosis) (popcorn is the best snack evar!)
```
mixed cellularity
lymphocyte-depleted (worst prognosis)
234
rouleaux formation (2)
Multiple myeloma
| Polycythemia Vera
235
RANK what does it do?
receptor on osteoclast, bone destruction "lytic lesions"
236
M spike - disease (3) and what it's made of
Multiple myeloma, IgG or IgA
Monoclonal Gammopathy of undetermined significance (MGUS)
Waldenstrom macroglobulinemia (IgM)
237
bone pain, proteinuria, primary AL amyloidosis, rouleaux formation
multiple myeloma
238
Name 3 complications with multiple myeloma
bone pain with hypercalcemia
AL amyloidosis
proteinuria - Bence Jones proteins - renal failure
239
lymphadenopathy, retinal hemorrhage, M spike - disease and treatment
Waldenstrom macroglobulinemia
| plasmapheresis removes IgM
240
Birbeck granules
Langerhans cell histiocytosis
241
CD1a, HLA-DR and S100+ disease and population
Langerhans cell histiocytosis
| young adult smokers
242
benign proliferation of Langerhans cells - disease and cells
eosinophilic granuloma
| eosinophils
243
basophilic stipling
sideroblastic anemia
lead poisoning
megaloblastic anemia
Beta Thalassemia major
244
What are Howell-Jolly bodies made of, when do you see them, and what stain do you use to see them?
excess chromatin
see when spleen isn't fully functional (usually removes the bodies)
Wright-Geimsa stain (DNA stain)
245
prussian blue stains?
iron
246
alcoholics have what common vitamin deficiency?
folate
247
normal ratio of fat to cells in bone marrow?
1:1
248
downy cells
EBV - infectious mononucleosis
249
ballerina skirt (on what cells with what infection?
T cells - EBV
250
EBV infects what cells and can progress to what cancer?
infects B cells
| B cell lymphoma
251
first lymph nodes enlarged in Hodgkin lymphoma?
axial
252
positive PAS staining
Acute Lymphoblastic Leukemia (ALL)
253
Hairy cell help
Hairy old men TRAP bloody animals (lodged in red pulp), eat fried eggs, and shoot blanks (dry tap). Hairy old men are slow -> progresses slowly
254
Tamm Horsfall protein
Multiple myeloma
255
Russel bodies (disease, what they're made of, appearance)
Multiple myeloma, extended RER and Golgi due to Ig accumulation, "fried egg" and eosinophilic
256
CD13 CD33
Acute myeloblastic leukemia (AML)
257
lots of band cells
chronic myeloid leukemia
258
dragging sensation of abdomen
chronic myeloid leukemia
259
reticulin stain: what does it stain and its used to detect what disease?
stains collagen
| myelofibrosis
260
MPL point mutation
myelofibrosis
261
leukoerythroblastic key word for?
myelofibrosis
