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Flashcards in Hematology Deck (207)
1

What is a sideroblast?
A ringed sideroblast?

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

Clinical conditions in which you might see ringed sideroblasts (6):

Lead poisoning
Heme metabolism d/o
EtOH
Drugs
Genetic disorders
Myelodysplastic syndrome

3

Four scenarios in which you might see target cells:

THAL:
Thalassemias
HbC disease
Asplenia
Liver disease

4

Two pathologic RBC forms you might see in someone with liver disease:

Target cells
Acanthocytes

5

What is physiologic anemia of pregnancy?

Increase in maternal plasma volume -> dilution of iron in the serum.

6

Iron deficiency anemia is a _________ __________ anemia.

Microcytic
Hypochromic

7

This type of anemia may manifest as Plummer-Vinson syndrome:
The famous triad:

Iron deficiency
Fe-deficiency
Esophageal webs
Atrophic glossitis

8

How many copies of the gene for a-globin do you have?

Four.

9

Which thalassemia is more prevalant in people of Mediterranean descent?
In Asian people?
African people?

b-thal = Mediterranean
a-thal = Asian, African

10

What is Hb Barts?

People make no a-globin.
In the fetus, so...
Gamma-globin makes a tetramer = Hb Barts

11

What is HbH disease?

HbH disease = 3-gene deletion of a-globin.
Make only a little a-globin
The extra b-globin makes tetramers = HbH

12

Four gamma-globin chains glued together, what am I?

Big trouble, you're not making alpha-globin chains in utero.
Hb Barts -> hydrops fetalis.

13

Four beta-globin chains glued together, what am I? What disease is this associated with?

HbH -> HbH disease.
A type of alpha-thalassemia.

14

How many genes for b-globin do you have?

Two.

15

When might you see target cells?

THAL
Thalassemia
HbC disease
Asplenia
Liver disease

16

How do you confirm the dx of b-thalassemia minor?

Will see an increase in HbA2.
HbA2 = a-globin + d-globin

17

Which thalassemia commonly puts people at risk for hemochromatosis?

b-thalassemia major (homozygous)
Often require blood transfusions.

18

Two skeletal abnormalities seen in b-thalassemia major:

Marrow expansion ->
"Crew cut" appearance on X-ray
"Chipmunk" facies

19

What is the structure of HbF?

a2g2

20

The macrocytic anemias can be divided into these two functional distinctions:
What is the difference between them?

Megaloblastic = trouble synthesizing DNA
Non-megaloblastic = unimpaired DNA synth.

21

Three causes of megaloblastic anemia:

Folate def.
B12 def.
Orotic aciduria

22

Common finding on blood smear in megaloblastic anemia (other than big-ass RBC):

Hypersegmented neutrophils

23

Anemia in which methylmalonic acid is normal:
Anemia in which methylmalonic acid is elevated:

Anemia 2/2 folate deficiency
Anemia 2/2 B12 deficiency

24

Four common causes of B12 deficiency:

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