HematologyJaynstein (Exam 1) Flashcards
1
Q
T/F: Factor V Leiden is the most common inherited form of coagulopathy
A
true!
2
Q
The liver produces all coagulation factors except factor ____
A
VIII
3
Q
Hematocrit is the % of a sample of whole blood occupied by ______
A
intact RBC’s
4
Q
If RDW is elevated, this suggests _______
A
large variability in sizes of RBCs
5
Q
Where are common sites of blood loss?
A
GI tract
Urinary tract
Menses/menorrhagia
Blood donations
6
Q
Complications of Thalassemia
A
- Hemochromatosis= iron overload
- Increased infection risk
- Bone deformities (secondary to bone marrow expansion)
7
Q
What is the triad of sx for TTP?
A
Thrombocytopenic purpura
Microangiopathic hemolytic anemia
Neurological symptoms (seizure, AMS, TIA)
8
Q
Von Willebrand Factor carries factor ______?
A
8
vWF carries factor VIII
9
Q
How can you distinguish petechiae and purpura from a skin rash?
A
Petechiae, purpura, and ecchymoses do NOT blanch with pressure.
10
Q
S/Sx of anemia
A
- Fatigue
- Tachycardia
- Hepatosplenomegaly
- Dyspnea on exertion
- Pallor
- Bone tenderness
11
Q
2 steps used for describing anemia’s:
A
1) Describe based on RBC size using the MCV
2) Describe based on Hgb concentration with MCH and MCHC
12
Q
Normal RDW range
A
11-15%
13
Q
What is the normal range for platelets?
A
150k-450k/mL
14
Q
Describe the process of antiphospholipid antibody syndrome.
A
An autoimmune disease in which people produce abnormal proteins (antiphospholipid antibodies)–>creates turbulent and stagnate blood flow–>thrombus forms
15
Q
T/F? This patient should be admitted for IV antibiotics to treat the GI infection, which will fix the HUS.
A
False - antibiotics will cause lysis of bacteria releasing more toxins.
16
Q
Normal Platelet count
A
100,000-450,000/mcL
17
Q
List 3 causes of anemia
A
- RBC loss
- Decreased RBC production
- Increased RBC destruction
18
Q
What is the pathophysiology of ITP?
A
IgG antibodies bind to platelets tagging them for destruction by the spleen
19
Q
Describe lab findings for Thalassemia
A
- Mild to mod microcytic, hypochromic anemia.
- Normal iron studies
- NL to elevated reticulocyte count – bone marrow trying to compensate
- Blood smear shows target cells
- Hgb electrophoresis is definitive
20
Q
Define hemarthroses
A
Bleeding into a joint capsule
21
Q
T/F? An acceptable replacement anticoagulant is warfarin
A
False - replace with any other anticoagulant other than warfarin
22
Q
Normal hemoglobin range for males?
A
13.5-16.5d/dl
23
Q
Ferritin Lab:
- measures?
- normal range?
A
Stored iron, first lab to become low
- < 30mcg/dL in a pt with decreased H/H is always iron deficiency anemia
24
Q
what is the hallmark lab value in polycythemia vera?
A
hematocrit >60%, but will see elevations in all cell lines.
25
Dx of APS?
-lab assessment for presence of antiphospholipid antibodies (aPL)
(3 possible lab tests exist and need positive results on at least 2 different ones to be able to dx)
Also: a positive test in the absence of a clot IS NOT a dx, just acknowledgement of risk
26
What are 3 main causes of thrombocytopenia?
| think broad
Decreased platelet production
Increased platelet destruction
Other (sepsis or blood loss)
27
Majority of iron absorption occurs in the _______. Once absorbed, it's transferred by _____
- duodenum
| - transferrin
28
Blood smear findings of sickle cell anemia include which of the following?
a) Target cells
b) Sickled cells
c) Basophilic stippling
d) Howell-Jolly bodies
a, b, and d
29
Tx for HIT?
Stop heparin and replace with another anticoagulant
| Avoid heparin indefinitely
30
Clinical findings for Thalassemia are _____ dependent
severity
| **more severe cases can have pallor, splenomegaly, growth failure, bone deformities, jaundice
31
Using hematocrit and Hgb, define anemia in an adult female.
- Hematocrit: <37%
| - Hgb <12 g/dL
32
What are the two main categories for normocytic anemia?
Hemorrhagic and non-hemorrhagic
33
Reticulocyte count= # of ______ RBC's
immature
34
s/sx of polycythemia vera?
pruritis, HA, fatigue, splenomegaly
35
What is HIT?
Heparin Inducted Thrombocytopenia
36
T/F: IDA is life-threatening
False! rarely life-threatening
37
What is the triad of HUS?
Thrombocytopenia, renal failure, hemolytic anemia
38
What are some causes of decreased platelet production?
Bone marrow suppression/failure (medications, chemo, anaplastic anemia)
Bone marrow destruction (lysis, cancer)
39
Thalassemia Tx: major cases
- May require chronic blood transfusions, splenectomy, and bone marrow transplant
- Avoid iron supplements and Sulfonamides
**Only cure for major cases is allogeneic stem cell transplant
40
What is APS the underlying cause to?
- DVTs
- ischemic strokes
- miscarriages
41
What labs are likely to be low in Von Willebrand's disease?
Plasma vWF and Factor VIII may be low
42
Describe Normocytic, Microcytic, and Macrocytic anemia using MCV
Normocytic= 80-100fl
Microcytic= <80 fl (decreased MCV)
Macrocytic= >100 fl (increased MCV)
43
MCH=
mean corpuscular hemoglobin (aka the average weight of Hgb per RBC)
44
What do you tell the parent of the child regarding the prognosis of idiopathic thrombocytopenic purpura?
The disease is self limiting and should resolve on its own
45
Hemochromatosis Tx
Chelation therapy= use of a chemical compound that combines with the metal poisoning for rapid and safe excretion
46
What is the treatment for G6PD?
avoiding known oxidative drugs
47
Thalassemia is defined as ______ _______ anemia
Microcytic hypochromic anemia.
48
Tx for folic acid deficiency?
Folic acid 1mg PO QD
49
The reticulocyte count comes back high, does this indicate hemolytic anemia or non-hemolytic anemia?
hemolytic anemia
50
What are your expected results on a blood smear of a patient with anaplastic anemia?
No abnormal cells
51
A child presents for evaluation of a rash, and easy bruising. He is thrombocytopenic, and further questioning reveals he recently got over a viral illness. What thrombocytopenia should you think of?
Idiopathic thrombocytopenic purpura
52
What is thrombocytopenia?
Low platelet count
53
What foods are rich in folic acid?
fruits and vegetables
54
Why is hemophilia more common in men?
Hemophilia is a sex linked hereditary gene that is located on the X chromosome.
Because men have only 1 X chromosome, they will have hemophilia if they acquire the gene on their only X chromosome.
55
Hemophilia A is due to a deficiency in factor _____,
| while hemophilia B is due to a deficiency in factor ________.
VIII (8),
| XI (9)
56
What is the treatment for an adult with ITP?
```
If platelets <20k or bleeding:
Prednisone 1-2mg/kg/day
IV immunoglobulin 1g/kg x 2 days
Platelet transfusions as necessary
Splenectomy is curative if chronic
```
57
Normal hematocrit range for females
36-44%
58
Examples of Microcytic anemias
- Iron Deficiency Anemia (IDA)
- Thalassemia
- Sideroblastic Anemia
59
What will happen to the Pt's Hct level after starting Ferrous sulfate?
-hct level will be halfway to normal in 3 weeks, and within normal by 2 months!
**If hct is NOT normal in 2 months--> wrong dx, continued blood loss, or pt is non-compliant
-“May” d/c Ferrous sulfate 3-6 months after hct is WNL
60
_____ is the leading cause of anemia worldwide.
Iron deficiency anemia (IDA)
61
What is the treatment for all clotting disorders?
- Acute: heparin/LWMH, possible thrombectomy or thrombolysis
- Preventative: anticoagulants or ASA
- Avoid OPCs if possible
- Minimize risks: smoking cessation, manage co-morbidities (HTN, DM, HLD)
62
What is the most common congenital coagulopathy?
Von Willebrand's Disease
63
Thalassemia is a hereditary disease of impaired ____ synthesis
Hgb
| **Normal Hgbis comprised of 2α chains and 2β chains, those with Thalassemia have a defect in one of those chains
64
T/F? You can diagnose ITP if the patient is thrombocytopenic, has purpura, and recently had a viral infection.
False - you must exclude other causes of thrombocytopenia.
65
Treatment for DIC?
Treat underlying disorder.
DIC is not a disease, but an event that accompanies other diseases, so treat the cause.
DIC can result from sepsis, pregnancy, trauma, cancer
66
Is hemophilia possible in women?
Yes, but less common than in men
67
IDA is caused by either insufficient dietary intake of iron, poor absorption of iron, or ____
chronic blood loss (note: More common in women (menstruating)
68
T/F? Humans cannot synthesize folic acid
True - it must come from diet
69
Is hemophilia more common in men or women?
Men
70
Equation for calculating Red blood cell distribution width (RDW)
= (Standard deviation of red cell volume ÷ mean cell volume) × 100
71
Treatment for HUS in adults?
Admission - corticosteroids, packed RBC's, plasmapheresis, aspirin, splenectomy
(same as tx for TTP)
72
Transferrin Saturation %
| expresses how much serum ____ is bound
iron
73
MCHC normal range=
31-37%
74
Sideroblastic anemia is usually _____
acquired-- ie alcoholism, lead/copper/zinc poisoning, dietary B6 or copper deficiency, OCP
75
What is an aPTT and what is it used for?
Activated Partial Thromboplastin Time
| It evaluates the intrinsic pathway
76
Hgb is measured in grams of Hgb per ___ mL of whole blood (g/dL)
100
77
Thalassemia Tx: mild cases
- No specific treatment, monitoring
- Folate supplements
- Avoid iron supplements and Sulfonamides
78
You are evaluating an adult male patient for fatigue. Labs are as follows: H&H 8.4/36%. MCV 92. Reticulocyte count: normal.
What types of anemia do you suspect?
Anemia of chronic disease/renal failure
| anaplastic anemia
79
List 4 examples of conditions that can cause a decreased production of RBCs
- Impaired Hgb synthesis
- Inhibition of DNA synthesis
- Disturbance of proliferation and differentiation of stem cells
- Bone marrow infiltration
80
Spontaneous bleeding does not typically occur until what platelet count?
<20k/mL
81
What does protein C do?
it has an anticoagulant property as an inhibitor of coag factors V and VIII
82
How to dx Factor V Leiden?
activated protein C (APC) resistance blood tests and PCR for Factor V Leiden
83
Lab Findings for Sideroblastic anemia & Tx
Labs show a microcytic anemia with normal iron studies
*KNOW: Diagnostic--> bone marrow has ringed sideroblasts
Tx: rarely needed, address acquired issues, blood transfusion if necessary
84
Evidence of capillary rupture can be seen in petechiae, purpura, and ecchymoses. What is the size range for each of them?
Petechiae - less than 3mm
Purpura - 3-10mm
Ecchymoses - >10mm
85
MCV=
mean corpuscular volume (aka the average size of RBC's)
86
Lead Poisoning Sx
SxS vary based on level and acuity:
-NV, C, abd pain
-AMS
-Pallor
Kids: irritability, behavioral issues, delays
-Metallic taste in mouth
-“lead line”
87
APS tx?
For pts with thrombotic event or recurrent miscarriages:
- Acute: heparin/LWMH, possible thrombectomy or thrombolysis
- Preventative: anticoagulants or ASA
- Avoid OPCs if possible
- Minimize risks: smoking cessation, manage co-morbidities (HTN, DM, HLD)
For pts without thrombotic event or recurrent miscarriages:
- ASA
- heparin DOC in pregnancy
- avoid OPCs and minimize risks
88
You are seeing a patient with signs of capillary rupture, who is bleeding from his nose and eyes. Hematuria is noted. What condition do you suspect?
Disseminated intravascular coagulation (DIC)
| Bleeding from multiple sights should make you think of DIC
89
What is secondary hemostasis?
Activation of clotting factors forming a fibrin complex that stabilizes the platelet plug.
90
What foods are vitamin B12 found in?
All foods of animal origin
91
Dx and tx of protein C deficiency?
Dx: during w/u after thrombotic event with protein C assay test
Tx: acute, preventative with anticoags
92
Describe a ferrous sulfate PO medication plan with a patient.
- Start with lower dosing (can be poorly tolerated)
- This is the ideal Tx, so do whatever you can to make the Pt compliant (ie Take with food, fewer x/day, before bed, etc. )
93
Symptoms of sickle cell anemia?
General symptoms of anemia as well as vaso-occlusive crises causing ischemic pain.
94
What is polycythemia vera?
acquired myeloproliferative disorder causing an increase in RBC production, and often WBCs and platelets as well. This causes a thickening of the blood, which predisposes to thrombotic events.
95
T/F? ITP acquired in adulthood is less worrisome than in children
False - ITP acquired in adulthood is more likely to become chronic
96
What is the pathophysiology of thrombotic thrombocytopenic purpura?
There is extensive microscopic intravascular clotting, which wastes a bunch of platelets. This decreases the functional amount of platelets in circulation.
97
What is the most common cause for Vitamin B12 deficiency?
Pernicious anemia
98
Give some examples of things that would increase a PT
Warfarin use, liver dysfunction, Vitamin K deficiency, DIC
99
Iron is stored as ____ in the liver, spleen, bone marrow, & muscle
ferritin
100
For a Pt with IDA, what are the indications for parental (IV) tx?
If Pt is intolerant of PO, GI disease inhibiting absorption, or has continued blood loss that cannot be corrected.
**Dose is calculated per pt
101
Tx for hemophilia?
Prevention!
avoiding drugs that can interfere with clotting (aspirin and NSAIDS)
Factor replacement prior to surgery
Early recognition of sx and tx with replacement factor VII or IX
102
What is the treatment for vitamin b12 deficiency?
Vitamin B12 injections - tapered down to only once a month after initial doses
PO cobalamin 1000mcg qd
103
Anemia is defined as a decrease in the number of ______
fully functioning and circulating RBC’s
104
Hemophilia is characterized by __________.
Prolonged coagulation time.
105
T/F? Bleeding into joints, soft tissue, urine/stool, and brain are common in patients with hemophilia.
True - hemophilia can cause bleeding anywhere.
| Joints, soft tissue, urine/stool, and brain are common sites of bleeding
106
Which is the more severe cause of RBC destruction, causes intrinsic to the RBC or extrinsic to the RBC?
Intrinsic causes are more severe and harder to treat. Examples are sickle cell anemia and G6PD.
107
IDA is classified as what type of anemia?
Microcytic hypochromic anemia-- BUT may be normocytic anemia early on
108
Serum Iron Lab value:
| useful or nah?
Can have considerable daily variation, so NO it's not exceptionally useful
--May be low or normal
109
What medication should be avoided in patients with Von Willebrand's Disease?
Aspirin
110
if MCV is >100 fl =
Macrocytic anemia
111
What are some symptoms of thrombocytopenia?
Evidence of capillary rupture, long bleeding time after trauma or easy bruising, mild gingival and nasal bleeding.
112
T/F? HELLP syndrome is much more likely to occur in conjunction with pre/eclampsia.
True - 15% occur with pre/eclampsia vs. 0.7% occurring without.
113
List relevant iron studies in order of sensitivity:
- Ferritin
- Total iron binding capacity (TIBC)
- Serum iron
- Transferrin saturation %
114
MCH normal range=
26-34 pg/cell
115
Your patient has a low H & H with a MCV of 110. What deficiencies may this patient have and what two labs do you order next?
Macrocytic anemias are commonly due to vitamin b12 or folic acid deficiencies. Therefore, you should order a Vitamin B12 (cobalamin) level and a folate level.
116
T/F? Spontaneous hemarthroses occurs during Von Willebrand's Disease.
False - Hemarthroses does not occur during Von Willebrand's Disease
117
MCHC =
mean corpuscular hemoglobin concentration (aka average Hgb per RBC)
118
How can a transferrin saturation % be indicative of IDA?
if it's low
119
You are seeing a patient with a low H & H with an MCV of 88. What lab do you order next?
Reticulocyte count
120
Using hematocrit and Hgb, define anemia in an adult male.
Hematocrit: <41%
| Hgb: < 13.5 g/dL
121
Which of the following is NOT a symptom of HELLP syndrome?
a) RUQ abd pain
b) epistaxis
c) blurry vision
d) fatigue
e) this are all symptoms of HELLP syndrome
e)
| HELLP syndrome has fairly non-specific symptoms
122
What is a PT and what is it used for?
Prothrombin time
IT evaluates the extrinsic pathway.
IT is also an excellent marker of current liver function
123
What does TIBC measure?
Measures the capacity to bind iron
| -normal range= 240-450 mcg/dL
124
if MCV 80-100 fl =
normocytic anemia
125
is protein S deficiency essentially the same as protein C deficiency just with a different letter?
yes, yes it is
126
IDA Dx (list lab profile values)
- Hgb: decreased
- MCV: decreased
- RDW: increased
- Ferritin: decreased
- TIBC/transferrin: increased
- transferrin saturation %: decreased
127
Thalassemias are broken down into which 3 things
Carriers (trait),
Minor (few to no Sx) and
Major (symptomatic)
128
What finding on a blood smear is indicative of G6PD?
Heinz bodies
129
Lead poisoning Tx
```
A BLL ≥45 mcg/dL requires the
initiation of outpatient chelation
therapy, and a BLL ≥70 mcg/dL
requires hospitalization and
chelation therapy
```
**Lead deposits lead to organ dysfunction
-Check CMP, UA, &
KUB XR
130
Describe Normochromic, Hypochromic, and hyperchromic anemia using MCH and MCHC
Normochromic= MCH 26-34 pg/cell or MCHC 31-37%
Hypochromic= MCH <26 pg/cell or MCHC <31%
Hyperchromic= MCH >34pg/cell or MCHC >37%
131
T/F? ADAMTS13 is the only cause of TTP.
False- is can occur secondary to another process.
132
What is the pentad of sx for TTP?
```
Thrombocytopenic purpura
Microangiopathic hemolytic anemia
Neurological symptoms (seizure, AMS, TIA)
Fever
Renal failure
```
133
Treatment for TTP?
ADMIT
| corticosteroids, packed RBC's, plasmapheresis (removal of vWF) aspirin, splenectomy
134
What symptoms could present in a patient with Vitamin B12 deficiency that should not be present in a patient with a folic acid deficiency?
Patients with B12 deficiencies can have CNS complaints, like weakness, paresthesia, loss of vibratory sense and loss of balance.
135
Vitamin K is necessary for synthesis of factor ____, ____, ____, and ____.
2, 7, 9, 10
136
what will you see in a bone marrow aspiration with polycythemia vera?
will be hypercellular
137
When can HELLP syndrome occur?
During the last trimester of pregnancy to several weeks post partum
138
Tx for HELLP syndrome?
Induce delivery (highly favored after 34 weeks)
consider steroids
Platelet transfusions
Treat pre/eclampsia
139
You are evaluating a pediatric patient who is thrombocytopenic, has hematuria and proteinuria, and is recovering from a GI infection. What syndrome should you think of?
Hemolytic uremic syndrome
140
IDA Tx: PO Tx options
- Diet: red meat, spinach, beans, lentils
| - Ferrous Sulfate – 325mg TID (10mg absorbed)
141
Sickle cell anemia is confirmed by what test?
Hemoglobin electrophoresis - finding 85-90% Hemoglobin S
142
What is the cause of aplastic anemia
Bone marrow failure due to injury or abnormal expression of stem cells.
Can be caused by radiation, chemo, toxins, SLE, or drugs (sulfas, phenytoin, carbamazepine)
143
if MCV <80 fl =
microcytic anemia
144
What underlying enzyme defect is related to TTP?
ADAMTS13
145
List 4 conditions that can cause increased destruction of RBCs
- RBC membrane defect
- Hgb defect
- Autoimmune
- Microangiopathic (hemolytic)
146
Normal hemoglobin range for females?
12- 15 g/dl
147
tx for polycythemia vera?
phlebotomy: one unit (500cc's) weekly until <45%, then PRN
148
What medication causes release of vWF, and is used before surgery in patients with Von Willebrand's Disease?
Desmopressin
149
For a Pt with suspected lead poisoning, what is the protocol
-Obtain lead levels in suspected cases
-Screening is targeted towards at risk children (not universal)
-Children on Medicaid obtain lead levels at 12 and 24 months
-Obtain lead level stick or venous sample)
Capillary level > 5 μg per dL -->venous sample to confirm
150
How can a TIBC value be indicative of IDA?
If it's high (>450)
151
Iron is an essential mineral and is necessary for functioning ____
Hgb
152
Tx for sickle cell anemia?
Hydroxyurea 500-750mg QD to stimulate erythropoiesis
Blood transfusions as necessary
folic acid supplement
153
How long after starting heparin might a patient develop HIT?
Usually about 5 days into treatment
154
It is wise to give a prescription for folate when you give a prescription for what medication?
Methotrexate
155
_______ anemia occurs when Hgb synthesis is reduced because of failure to incorporate iron--> iron is available, but the body can’t incorporate it
Sideroblastic anemia
156
Christmas disease is a deficiency of factor _______.
IX
Christmas has 9 letters.
Christmas disease is a deficiency of factor 9
157
IDA: S/Sx?
- Tachycardia
- DOE
- Fatigue
- Pale skin & mucosa
- Brittle nails
- Angular cheilitis
- Pruritus
- Pica
- Anxiety, tingling, numbness
158
Treatment for HUS in children?
Admission for IV fluids
159
What is primary hemostasis?
Platelet activation, adhesion, and aggregation leading to creation of a hemostatic plug
160
Normal hematocrit range for males
41-50%
161
Give some examples of things that would increase an aPTT
Heparin use, hemophilia, DIC, APS
162
What does the HELLP syndrome stand for?
Hemolysis, Elevated liver enzymes, and Low Platelet count
