Hematology Flashcards
(207 cards)
1
Q
What is a sideroblast?
A ringed sideroblast?
A
RBC with excessive iron in its mitochondria.
Normal to see these in the bone marrow.
Ringed sideroblasts = never normal. Have a ring of iron around the nucleus.
2
Q
Clinical conditions in which you might see ringed sideroblasts (6):
A
Lead poisoning Heme metabolism d/o EtOH Drugs Genetic disorders Myelodysplastic syndrome
3
Q
Four scenarios in which you might see target cells:
A
THAL: Thalassemias HbC disease Asplenia Liver disease
4
Q
Two pathologic RBC forms you might see in someone with liver disease:
A
Target cells
Acanthocytes
5
Q
What is physiologic anemia of pregnancy?
A
Increase in maternal plasma volume -> dilution of iron in the serum.
6
Q
Iron deficiency anemia is a _________ __________ anemia.
A
Microcytic
Hypochromic
7
Q
This type of anemia may manifest as Plummer-Vinson syndrome:
The famous triad:
A
Iron deficiency
Fe-deficiency
Esophageal webs
Atrophic glossitis
8
Q
How many copies of the gene for a-globin do you have?
A
Four.
9
Q
Which thalassemia is more prevalant in people of Mediterranean descent?
In Asian people?
African people?
A
b-thal = Mediterranean a-thal = Asian, African
10
Q
What is Hb Barts?
A
People make no a-globin.
In the fetus, so…
Gamma-globin makes a tetramer = Hb Barts
11
Q
What is HbH disease?
A
HbH disease = 3-gene deletion of a-globin.
Make only a little a-globin
The extra b-globin makes tetramers = HbH
12
Q
Four gamma-globin chains glued together, what am I?
A
Big trouble, you’re not making alpha-globin chains in utero.
Hb Barts -> hydrops fetalis.
13
Q
Four beta-globin chains glued together, what am I? What disease is this associated with?
A
HbH -> HbH disease.
A type of alpha-thalassemia.
14
Q
How many genes for b-globin do you have?
A
Two.
15
Q
When might you see target cells?
A
THAL Thalassemia HbC disease Asplenia Liver disease
16
Q
How do you confirm the dx of b-thalassemia minor?
A
Will see an increase in HbA2.
HbA2 = a-globin + d-globin
17
Q
Which thalassemia commonly puts people at risk for hemochromatosis?
A
b-thalassemia major (homozygous)
Often require blood transfusions.
18
Q
Two skeletal abnormalities seen in b-thalassemia major:
A
Marrow expansion ->
“Crew cut” appearance on X-ray
“Chipmunk” facies
19
Q
What is the structure of HbF?
A
a2g2
20
Q
The macrocytic anemias can be divided into these two functional distinctions:
What is the difference between them?
A
Megaloblastic = trouble synthesizing DNA Non-megaloblastic = unimpaired DNA synth.
21
Q
Three causes of megaloblastic anemia:
A
Folate def.
B12 def.
Orotic aciduria
22
Q
Common finding on blood smear in megaloblastic anemia (other than big-ass RBC):
A
Hypersegmented neutrophils
23
Q
Anemia in which methylmalonic acid is normal:
Anemia in which methylmalonic acid is elevated:
A
Anemia 2/2 folate deficiency
Anemia 2/2 B12 deficiency
24
Q
Four common causes of B12 deficiency:
A
Malabsorption Poor intake Pernicious anemia PPIs (Worms)
25
Which of the macrocytic anemias would you expect to see neurologic sx with?
B12 deficiency anemia
26
This macrocytic anemia is incurable by folate / B12 administration:
Anemia 2/2 orotic aciduria
27
What is the treatment for orotic aciduria?
Uridine monophosphate
28
In B12 and folate deficiency anemias, what is the homocysteine level like?
Increased.
29
What is the protein responsible for binding ferroportin in anemia of chronic disease?
Hepcidin
30
What kind of anemia is anemia of chronic disease?
Normocytic
| May progress to microcytic, hypochromic
31
Four common causes of aplastic anemia:
```
Severe vitamin B12 and folic acid deficiency
Radiation / drugs
Viruses (parvo B19, EBV, HIV, HCV)
Fanconi's
(Idiopathic)
```
32
Hypocellular bone marrow with fatty infiltrate:
Aplastic anemia
33
Sickle cell patients are vulnerable to these viruses (4):
Parvovirus B19
EBV
HIV
HCV
34
Chronic kidney disease causes this kind of anemia:
Normocytic, normochromic
35
Triad for pancytopenia:
Severe anemia
Leukopenia
Thrombocytopenia
36
Protein that transports iron in blood:
Transferrin
37
The primary storage protein for iron in the body:
Ferritin
38
What effect do pregnancy and OCPs have on transferrin levels?
Increase
39
```
What would you expect to see in hemochromatosis:
Serum Fe
Transferrin
Ferritin
% Transferrin sat.
```
```
Hemochromatosis:
Serum Fe HIGH
Transferrin LOW
Ferritin HIGH
% Saturation HIIIIIGH
```
40
Iron studies in thalassemia:
Fe:
TIBC:
Ferritin:
Normal Fe, TIBC, ferritin...
| Microcytic anemia with target cells.
41
This test is used to dx b-thal minor:
Hb electrophoresis
| Look for HbA2 (>3.5%)
42
Normocytic anemia + red urine in the morning:
Paroxysmal nocturnal hemoglobinuria
43
Microcytic anemia reversible with B6:
Sideroblastic anemia
44
This megaloblastic anemia is not correctable with B12 or folate:
Orotic aciduria
45
Sideroblastic anemia can be congenital or acquired.
What is the defect in congenital sideroblastic anemia?
What mode of inheritance?
Defective d-ALA synthase = no protoporphyrin, Fe accumulates in mitochondria.
X-linked recessive.
46
Name 3 reversible etiologies of sideroblastic anemia:
EtOH
Lead poisoning
Isoniazid administration
47
What is the treatment for acquiredsideroblastic anemia?
| Why does it work?
B6 = pyridoxine
| This is the co-factor for ALA-synthetase.
48
What is haptoglobin?
A hemoglobin scavenger molecule, binds up Hb outside the cell.
49
In what case might you see a decrease in serum haptoglobin?
Generally, intravascular hemolysis.
50
What is the difference between intravascular and extravascular hemolysis?
```
Intravascular = destruction in the vessels
Extravascular = destruction by the spleen
```
51
Three common causes of intravascular hemolysis:
1. PNH = complement-mediated lysis, defect is in GPI anchor or decay accelerating factor in complement pathway
2. Autoimmune hemolytic anemia
3. Mechanical damage to RBC
52
Extravascular hemolysis, 5 causes:
```
Hereditary spherocytosis
G-6-PD deficiency
Pyruvate kinase deficiency
Sickle cell disease
HbC disease
```
53
Expect elevated LDH in:
Hemolysis (both intra and extravascular)
54
Bilirubin is more an indicator of this kind of hemolysis:
Intravascular
55
Two defective proteins in hereditary spherocytosis:
Ankyrin
| Spectrin
56
Small, round RBCs with no central pallor:
Hereditary spherocytosis
57
High RDW with high MCHC:
Hereditary spherocytosis
58
Heinz bodies and bite cells:
G6PD deficiency
59
Oxidant stress causes hemolytic anemia:
G6PD deficiency
60
Positive osmotic fragility test:
Hereditary spherocytosis
61
Aplastic crisis (2):
Sickle cell disease
| Hereditary spherocytosis
62
Rigid RBCs:
Pyruvate kinase deficiency, inability to maintain RBC membrane 2/2 decreased ATP
63
Mode of inheritence for G6PD deficiency:
X-linked recessive
64
Triad seen in paroxysmal nocturnal hemoglobinuria:
Remember, it's complement mediated:
Hemolytic anemia
Pancytopenia
Thrombosis (platelets lysed too)
65
CD55/59(-) RBCs on flow cytometry:
Paroxysmal nocturnal hemoglobinuria
66
Hemolytic anemia in a newborn:
G6PD deficiency
67
Why are newborns initially asymptomatic in SS disease?
They still have HbF to protect them
68
Organisms you are at risk for infection from if you do not have a spleen:
Encapsulated
| ESoHPNC
69
Glutamic acid to lysine in b-globin:
| Glutamic acid to valine in b-globin:
```
lysine = HbC
valine = HbSS
```
70
Treatment for paroxysmal nocturnal hemoglobinuria:
Eculizumab
71
Two ways of diagnosing paroxysmal nocturnal hemoblobinuria:
```
Ham's test
Flow cytometry (CD55/59- cells)
```
72
Drug used in sickle cell disease:
| What does it do?
Hydroxyurea
| Increases HbF
73
Wedge-shaped splenic infarcts on CT:
Splenic sequestration crisis in sickle cell disease
74
Rate-limiting step in hemoglobin synthesis:
| Enzyme:
Glycine + succinyl CoA -> d-ALA
| Catalyzed by d-ALA synthetase
75
Warm agglutinin antibodies tend to be ___.
| Cold agglutinin antibodies tend to be ___.
IgG
| IgM
76
Four common sources of cold-agglutinin Ab:
```
Viruses
SLE
Malignancies
Congenial
Immune
```
77
Cold-agglutinin Ab are associated with (3):
Mycoplasma
EBV
CLL
78
What is a direct Coomb's test?
| Indirect Coomb's test?
Direct = patient's RBC + prepped Ab.
Indirect = patient's serum + normal RBC.
Looking for Ig bound to RBC.
79
When might you use a direct Coomb's?
| An indirect Coomb's?
Direct used in hemolytic disease.
| Indirect used in blood screening before transfusion.
80
Microangiopathic anemia -- you would see this kind of cell on peripheral smear:
Schistocyte
81
Splenectomy cured this guy:
Hereditary spherocytosis
82
Ham's test was positive:
Paroxysmal nocturnal hemoglobinuria
83
DEB test was positive:
Fanconi anemia
84
You see Heinz bodies:
G6PD deficiency
85
Osmotic fragility test was positive:
Hereditary spherocytosis
86
Painful cyanosis of fingers and toes, hemolytic anemia:
Cold agglutinin anemia
87
Red urine in the morning, fragile RBC:
PNH
88
Basophilic nuclear remnants in RBC:
Howell-Jolly bodies
| You got no spleen.
89
Autosplenectomy
Sickle cell disease
90
Drug used to treat sickle cell disease:
Hydroxyurea
91
This molecule binds exposed collagen when vascular endothelium is damaged:
What binds to it in turn?
vWF
| GpIb on platelets
92
Platelets use this molecule on their surface to bind vWF, which is bound to damaged endothelium:
They then release many things. Two are integral to initiating the coagulation cascade:
GpIb
| Calcium, ADP
93
Activated platelets display this molecule on their surfaces, other platelets stick to it:
Substance that induced this activation:
GpIIb / IIIa
| ADP
94
This molecule links platelets:
Fibrinogen
95
What do GpIIb / IIIa do?
| What does GpIb do?
These are molecules expressed on the surface of platelets, they bind fibrin and link platelet to platelet.
GpIb initially binds vWF, which is stuck to exposed collagen; it starts the process.
96
Drug that blocks GpIIb / IIIa:
Abciximab
97
Drugs (2) that block the receptor for ADP on platelets:
| These drugs are anti-platelet drugs, explain how this works:
Clopidogrel
Ticlopidine
ADP is necessary to induce GpIIb / IIIa expression on platelets. GpIIb / IIIa is the fibrinogen-binding molecule that links platelet-fibrinogen-platelet.
98
This drug is a direct inhibitor of GpIIb / IIIa.
| These two drugs are indirect:
Abciximab = direct
| Clopidogrel, ticlodipine = indirect
99
Two main functions of vWF:
Stabilize factor VIII
| Platelet adhesion to collagen via GpIb
100
Defects in vWF cause two main abnormalities in bleeding studies:
Increased bleeding time
| Increase in PTT (factor VIII is unstable w/o vWF, coag cascade affected)
101
Deficiency in GpIIb / IIIa =
Glanzmann's thrombasthenia
102
Deficiency in GpIb =
Bernard-Soulier syndrome
103
Bite cells
G6PD deficeincy
| The Heinz body got bitten out
104
Acanthocytes (2 pathologic causes):
Liver disease
| Abetalipoproteinemia
105
Hemophilia A = deficiency in what factor?
Factor 8 = factor AIGHT
106
Vitamin K deficiency results in decreased synthesis of:
Factors II, VII, IX, X
| Proteins C & S
107
Hemophilia A and B both increase...
PTT
108
Platelet abnormalities are associated with this kind of hemorrhage:
Microhemorrhage
109
Bernard-Soulier syndrome is a defect in:
Defect in platelet plug formation
| GpIb, can't stick to vWF
110
Platelet disorder with a roughly normal platelet count and increased bleeding time:
Bernard-Soulier syndrome
111
Platelet adhesion defect in Glanzmann's thrombasthenia:
GpIIb / IIIa - defect in platelet - platelet adhesion
| Remember platelet 2 platelet 3 platelet...
112
Pt has increased bleeding time. On blood smear, you see no platelet to platelet clumping:
Glanzmann's thrombasthenia
113
Pathophys. behind ITP:
IMMUNE
Anti-GpIIb / IIIa Ab
Spleen eats the platelets
114
Treatment for ITP (2):
IVIG
| Splenectomy
115
Normal platelet count, increased bleeding time (1).
| Low platelet count, increased bleeding time (3).
Normal platelets = Glanzmann's thrombasthenia.
| Low platelets = Bernard-Soulier, ITP, TTP
116
Schistocytes, high LDH, setting of thrombocytopenia:
TTP.
Defect in vWF metalloprotease.
Platelet consumption 2/2 vWF multimers.
Causes microangiopathic hemolytic anemia -> shearing RBCs.
117
TTP: What is the defect that causes this disorder?
Defect in ADAMTS13 = vWF metalloprotease
118
Sx of TTP are a pentad:
```
Nasty Fever Torched His Kidneys:
Neurologic
Fever
Thrombocytopenia
Hemolysis
Kidneys (uremia)
```
119
Hemolytic uremic syndrome in a kid may be caused by this pathogen:
E. coli 0157H7
120
Difference in presentation between HUS and TTP:
```
TTP = pentad of neurologic, fever, thrombocytopenia, hemolysis, renal involvement.
HUS = no neuro and no fever.
```
121
Treatment for vW disease:
| Why does this work?
Desmopressin (releases stored vWF for whatever reason)
122
Mode of inheritence for vW disease:
Autosomal dominant
123
Causes of DIC:
```
STOP Making Thrombi:
Sepsis
Trauma
OB
Pancreatitis
Malignancy
Transfusion
```
124
Your friend has vWF disease. What do her bleeding time / coag studies look like?
```
vWF = increased bleeding time (platelet dysfunction)
Increased PTT (factor VIII instability)
```
125
T/F: Aspirin increases PTT.
F. Aspirin affects platelet function, not the coag cascade.
It increases BLEEDING TIME.
126
Tinnitus on OD:
Aspirin
127
These drugs are ADP receptor inhibitors (4):
| How do they work?
```
Clopidogrel
Ticlopidine
Prasugrel
Ticagrelor
Block ADP receptor, activation necessary for GpIIb / IIIa induction and platelet + platelet + platelet action
```
128
Direct GpIIb / IIIa inhibitors (3):
Abciximab
Eptifibatide
Tirofiban
129
Where does this drug act in the platelet activation pathway -- Clopidogrel:
ADP receptor inhibitor
| Blocks GpIIb / IIIa induction
130
Where does this drug act in the platelet activation pathway -- Abciximab:
GpIIb / IIIa inhibitor
131
Where does this drug act in the platelet activation pathway -- Ticlopidine:
ADP receptor blocker
| Blocks GpIIb / IIIa induction
132
This drug acts like clopidogrel:
CLOpidogrel
TiCLOdipine
anti-"CLOT"
133
What is the cause of ITP?
Ab generated against the GpIIb / IIIa receptor
134
Life span of a platelet in circulation:
8-10d
135
DIC, labs (4):
D-dimer increase
Low platelets
High PT/PTT
Increased bleeding time
136
What is the mechanism of action of clopidogrel?
ADP receptor blocker
137
What is the mechanism of action of abciximab?
Blocks GpIIb / IIIa
138
What is the mechanism of action of tirofiban?
Blocks GpIIb / IIIa
139
What is the mechanism of action of ticlodipine?
ADP receptor blocker
140
What is the mechanism of action of enoxaparin?
LMW heparin
141
What is the mechanism of action of eptifibatide?
Blocks GpIIb / IIIa
142
What is a leukemoid reaction?
| Leukemoid reaction is distinguishable from CML on the basis of one lab finding, what is it?
Leukemoid reaction is an increased WBC with high neutrophils (L shift).
CML appears the same way. Difference is that leukocyte alk. phos is high in leukemoid reaction, low in CML. CML = not mature, doesn't get around to making alk. phos.
143
These two lymphomas are associated with EBV:
Burkitt's
| Non-Hodgkin
144
Two antigens that are (+) on Reed Sternberg cells:
CD30, CD15
145
Features of Hodgkin's lymphoma nodal involvement (3):
Localized
Rare extra-nodal involvement
Contiguous spread
146
Age distribution in Hodgkin's lymphoma:
Bimodal: 20 / 50 years of age.
147
Four subtypes of Hodgkin's lymphoma, order of best to worst prognosis:
Lymphocyte-predominant
Nodular sclerosing
Mixed
Lymphocyte-depleted
148
Features of nodal involvement in non-Hodgkin's lymphoma:
Multiple, peripheral nodes
Extranodal involvement common
Non-contiguous spread
149
"Owl's eye" cell:
Reed Sternberg cell
| Hodgkin's lymphoma
150
Burkitt's lymphoma on histology:
```
Starry sky
(sky = sheets of lymphocytes
stars = macrophages that eat them)
```
151
Three types of Burkitt's lymphoma:
What type is EBV associated?
Which type affects the pelvis / abdomen?
Endemic (EBV, huge jaw lesions)
Sporadic (pelvis / abdomen)
Immune-deficient
152
Most common non-Hodgkin lymphoma in adults:
Diffuse large B cell lymphoma
153
Translocation in diffuse large B cell lymphoma:
14;18
154
Genetic defect in Burkitt's lymphoma
c-myc translocated with Ig heavy chain
155
Genetic defect in follicular lymphoma:
bcl-2 translocated with Ig heavy chain
156
This CD5+ lymphoma has a poor prognosis:
Mantle cell lymphoma
157
Which lymphoma am I?
c-myc translocated with Ig heavy chain:
bcl-2 translocated with Ig heavy chain:
cyclin D translocated with Ig heavy chain:
c-myc = burkitts
bcl-2 = follicular
cyclin D = mantle cell
158
Cutaneous lesions, HTLV-1 associated lymphoma:
Adult T-cell lymphoma
159
Three disorders associated with marginal cell MALToma:
Sjogrens
Hashimoto
H. pylori
160
Intestinal T cell lymphoma is associated with this disease:
Celiac
161
"Starry sky" appearance on histology:
Burkitt's lymphoma
162
Monoclonal plasma cell proliferation, punched out lytic bone lesions, tumor usually produces these Ab (2):
This is multiple myeloma
Mostly IgG
Sometimes IgA
163
Bence Jones protein: What is it, what is it associated with?
Ig light chains in urine
| Associated with multiple myeloma
164
T/F: You can measure Bence-Jones protein on a urine protein dip.
F. Need urine protein electrophoresis.
165
Rouleaux formation, what is it and what disease do you see it in?
Stacked up RBCs, see this in multiple myeloma
166
On electrophoresis, what do you observe with multiple myeloma?
A Monoclonal M protein spike
167
Four signs / sx of multiple myeloma:
```
CRAB:
hyper-Calcemia
Renal insufficiency
Anemia
Bone lytic lesions / Back pain
```
168
Where would you see a monoclonal M protein spike made of IgM?
Waldenstrom's macroglobulinemia
169
This disorder is a precursor to multiple myeloma:
MGUS = monoclonal gammopathy of undetermined significance
170
What is a plasmacytoma?
A solid tumor of plasma cells.
171
T/F: Lytic bone lesions in a plasmacytoma.
| T/F: Lytic bone lesions in Waldenstrom's.
F on both counts. Only see these in multiple myeloma.
172
This plasmacytoma is seen in the head / neck region:
Extramedullary plasmacytoma
173
These two leukemias are associated with Down syndrome:
ALL
| AML
174
This leukemia is sometimes Philadelphia chromosome (+):
ALL
175
This leukemia is always Philadelphia chrom. (+):
Philadelphia CreaML cheese
| CML
176
Pancytopenia: acute or chronic leukemia?
Acute.
177
Smudge cells in a much much older adult:
CLL.
| Usually in people >60yo.
178
TRAP-staining B cell tumor:
Hairy cell leukemia.
179
Associated with autoimmune hemolytic anemia, warm or cold agglutinins:
CLL
180
Auer rods:
AML
181
Treatment for AML:
all-trans-retinoic acid
182
Treatment for CML:
Imatinib
183
Treatment for hairy cell leukemia:
Cladribine
184
Philadelphia chromosome translocation:
t(9;22)
185
Disorder associated with this translocation, protein involved:
t(8;14)
Burkitt's, c-myc
186
Disorder associated with this translocation, protein involved:
t(11;14)
Mantle cell lymphoma, cyclin D1
187
Disorder associated with this translocation:
| t(15;17)
M3 type of AML
188
Disorder associated with this translocation, protein involved:
t(14;18)
Follicular lymphoma, bcl-2
189
Disorder associated with this translocation, protein involved:
t(18;21)
AML
190
Disorder associated with this translocation, protein involved:
t(9;22)
This is the Philadelphia chromosome.
CreaML = CML
(Sometimes ALL)
191
What is an Auer body?
What leukemia do you see them in?
What happens if you treat this leukemia?
Peroxidase-positive cytoplasmic inclusion in myeloblasts.
AML M3 subtype.
Treatment -> lysis and DIC.
192
Lytic bone lesions and skin rash in a child, S-100(+) cells:
Langerhans cell histiocytosis.
| S-100 is a neural crest marker.
193
These three chronic myeloproliferative disorders are JAK2 positive:
Polycythemia vera
Essential thrombocytosis
Myelofibrosis
194
Cells proliferate without epo stimulation and cause this myeloproliferative disease:
Polycythemia vera
195
Cells proliferate without thrombopoieitin stimulation and cause this myeloproliferative disease:
Essential thrombocytosis
196
Fibrotic obliteration of bone marrow with teardrop cells:
Myelofibrosis
197
Polycythemia: 4 causes:
Polycythemia vera
Too much epo (from renal cell ca, pheo, HCC, or hemangioma)
Chronic hypoxia
Trisomy 21
198
This myeloproliferative disorder may present as intense itching after a hot shower:
Polycythemia vera
199
Symptoms / signs of polycythemia vera (4):
Plethora
HA
Splenomegaly
Itching
200
Most common type of non-Hodgkin lymphoma in adults?
| In children?
Diffuse large B cell in adults
| Lymphoblastic leukemia in kids
201
Most common leukemia in children:
ALL
202
Most common leukemia in adults in the US:
CLL
203
Leukemia associated with Auer rods:
AML
204
PAS (+) acute leukemia:
| PAS (-) acute leukemia:
ALL
| AML
205
This leukemia commonly presents with bone pain:
ALL
206
This leukemia is positive for peroxidase:
AML
207
BCR-ABL gene associated leukemia:
CML
| This is none other than the Philadelphia chromosome
