HematologyJaynstein (Exam 1) Flashcards

1
Q

T/F: Factor V Leiden is the most common inherited form of coagulopathy

A

true!

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2
Q

The liver produces all coagulation factors except factor ____

A

VIII

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3
Q

Hematocrit is the % of a sample of whole blood occupied by ______

A

intact RBC’s

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4
Q

If RDW is elevated, this suggests _______

A

large variability in sizes of RBCs

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5
Q

Where are common sites of blood loss?

A

GI tract
Urinary tract
Menses/menorrhagia
Blood donations

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6
Q

Complications of Thalassemia

A
  • Hemochromatosis= iron overload
  • Increased infection risk
  • Bone deformities (secondary to bone marrow expansion)
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7
Q

What is the triad of sx for TTP?

A

Thrombocytopenic purpura
Microangiopathic hemolytic anemia
Neurological symptoms (seizure, AMS, TIA)

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8
Q

Von Willebrand Factor carries factor ______?

A

8

vWF carries factor VIII

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9
Q

How can you distinguish petechiae and purpura from a skin rash?

A

Petechiae, purpura, and ecchymoses do NOT blanch with pressure.

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10
Q

S/Sx of anemia

A
  • Fatigue
  • Tachycardia
  • Hepatosplenomegaly
  • Dyspnea on exertion
  • Pallor
  • Bone tenderness
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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

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12
Q

Normal RDW range

A

11-15%

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13
Q

What is the normal range for platelets?

A

150k-450k/mL

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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

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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.

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16
Q

Normal Platelet count

A

100,000-450,000/mcL

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17
Q

List 3 causes of anemia

A
  • RBC loss
  • Decreased RBC production
  • Increased RBC destruction
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18
Q

What is the pathophysiology of ITP?

A

IgG antibodies bind to platelets tagging them for destruction by the spleen

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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
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20
Q

Define hemarthroses

A

Bleeding into a joint capsule

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21
Q

T/F? An acceptable replacement anticoagulant is warfarin

A

False - replace with any other anticoagulant other than warfarin

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22
Q

Normal hemoglobin range for males?

A

13.5-16.5d/dl

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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

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24
Q

what is the hallmark lab value in polycythemia vera?

A

hematocrit >60%, but will see elevations in all cell lines.

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25
Q

Dx of APS?

A

-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

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26
Q

What are 3 main causes of thrombocytopenia?

think broad

A

Decreased platelet production
Increased platelet destruction
Other (sepsis or blood loss)

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27
Q

Majority of iron absorption occurs in the _______. Once absorbed, it’s transferred by _____

A
  • duodenum

- transferrin

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28
Q

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

a, b, and d

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29
Q

Tx for HIT?

A

Stop heparin and replace with another anticoagulant

Avoid heparin indefinitely

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30
Q

Clinical findings for Thalassemia are _____ dependent

A

severity

**more severe cases can have pallor, splenomegaly, growth failure, bone deformities, jaundice

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31
Q

Using hematocrit and Hgb, define anemia in an adult female.

A
  • Hematocrit: <37%

- Hgb <12 g/dL

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32
Q

What are the two main categories for normocytic anemia?

A

Hemorrhagic and non-hemorrhagic

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33
Q

Reticulocyte count= # of ______ RBC’s

A

immature

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34
Q

s/sx of polycythemia vera?

A

pruritis, HA, fatigue, splenomegaly

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35
Q

What is HIT?

A

Heparin Inducted Thrombocytopenia

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36
Q

T/F: IDA is life-threatening

A

False! rarely life-threatening

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37
Q

What is the triad of HUS?

A

Thrombocytopenia, renal failure, hemolytic anemia

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38
Q

What are some causes of decreased platelet production?

A

Bone marrow suppression/failure (medications, chemo, anaplastic anemia)
Bone marrow destruction (lysis, cancer)

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39
Q

Thalassemia Tx: major cases

A
  • 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

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40
Q

What is APS the underlying cause to?

A
  • DVTs
  • ischemic strokes
  • miscarriages
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41
Q

What labs are likely to be low in Von Willebrand’s disease?

A

Plasma vWF and Factor VIII may be low

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42
Q

Describe Normocytic, Microcytic, and Macrocytic anemia using MCV

A

Normocytic= 80-100fl

Microcytic= <80 fl (decreased MCV)

Macrocytic= >100 fl (increased MCV)

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43
Q

MCH=

A

mean corpuscular hemoglobin (aka the average weight of Hgb per RBC)

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44
Q

What do you tell the parent of the child regarding the prognosis of idiopathic thrombocytopenic purpura?

A

The disease is self limiting and should resolve on its own

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45
Q

Hemochromatosis Tx

A

Chelation therapy= use of a chemical compound that combines with the metal poisoning for rapid and safe excretion

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46
Q

What is the treatment for G6PD?

A

avoiding known oxidative drugs

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47
Q

Thalassemia is defined as ______ _______ anemia

A

Microcytic hypochromic anemia.

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48
Q

Tx for folic acid deficiency?

A

Folic acid 1mg PO QD

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49
Q

The reticulocyte count comes back high, does this indicate hemolytic anemia or non-hemolytic anemia?

A

hemolytic anemia

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50
Q

What are your expected results on a blood smear of a patient with anaplastic anemia?

A

No abnormal cells

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51
Q

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?

A

Idiopathic thrombocytopenic purpura

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52
Q

What is thrombocytopenia?

A

Low platelet count

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53
Q

What foods are rich in folic acid?

A

fruits and vegetables

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54
Q

Why is hemophilia more common in men?

A

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.

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55
Q

Hemophilia A is due to a deficiency in factor _____,

while hemophilia B is due to a deficiency in factor ________.

A

VIII (8),

XI (9)

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56
Q

What is the treatment for an adult with ITP?

A
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
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57
Q

Normal hematocrit range for females

A

36-44%

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58
Q

Examples of Microcytic anemias

A
  • Iron Deficiency Anemia (IDA)
  • Thalassemia
  • Sideroblastic Anemia
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59
Q

What will happen to the Pt’s Hct level after starting Ferrous sulfate?

A

-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

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60
Q

_____ is the leading cause of anemia worldwide.

A

Iron deficiency anemia (IDA)

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61
Q

What is the treatment for all clotting disorders?

A
  • 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)
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62
Q

What is the most common congenital coagulopathy?

A

Von Willebrand’s Disease

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63
Q

Thalassemia is a hereditary disease of impaired ____ synthesis

A

Hgb

**Normal Hgbis comprised of 2α chains and 2β chains, those with Thalassemia have a defect in one of those chains

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64
Q

T/F? You can diagnose ITP if the patient is thrombocytopenic, has purpura, and recently had a viral infection.

A

False - you must exclude other causes of thrombocytopenia.

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65
Q

Treatment for DIC?

A

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
Q

Is hemophilia possible in women?

A

Yes, but less common than in men

67
Q

IDA is caused by either insufficient dietary intake of iron, poor absorption of iron, or ____

A

chronic blood loss (note: More common in women (menstruating)

68
Q

T/F? Humans cannot synthesize folic acid

A

True - it must come from diet

69
Q

Is hemophilia more common in men or women?

A

Men

70
Q

Equation for calculating Red blood cell distribution width (RDW)

A

= (Standard deviation of red cell volume ÷ mean cell volume) × 100

71
Q

Treatment for HUS in adults?

A

Admission - corticosteroids, packed RBC’s, plasmapheresis, aspirin, splenectomy
(same as tx for TTP)

72
Q

Transferrin Saturation %

expresses how much serum ____ is bound

A

iron

73
Q

MCHC normal range=

A

31-37%

74
Q

Sideroblastic anemia is usually _____

A

acquired– ie alcoholism, lead/copper/zinc poisoning, dietary B6 or copper deficiency, OCP

75
Q

What is an aPTT and what is it used for?

A

Activated Partial Thromboplastin Time

It evaluates the intrinsic pathway

76
Q

Hgb is measured in grams of Hgb per ___ mL of whole blood (g/dL)

A

100

77
Q

Thalassemia Tx: mild cases

A
  • No specific treatment, monitoring
  • Folate supplements
  • Avoid iron supplements and Sulfonamides
78
Q

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?

A

Anemia of chronic disease/renal failure

anaplastic anemia

79
Q

List 4 examples of conditions that can cause a decreased production of RBCs

A
  • Impaired Hgb synthesis
  • Inhibition of DNA synthesis
  • Disturbance of proliferation and differentiation of stem cells
  • Bone marrow infiltration
80
Q

Spontaneous bleeding does not typically occur until what platelet count?

A

<20k/mL

81
Q

What does protein C do?

A

it has an anticoagulant property as an inhibitor of coag factors V and VIII

82
Q

How to dx Factor V Leiden?

A

activated protein C (APC) resistance blood tests and PCR for Factor V Leiden

83
Q

Lab Findings for Sideroblastic anemia & Tx

A

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
Q

Evidence of capillary rupture can be seen in petechiae, purpura, and ecchymoses. What is the size range for each of them?

A

Petechiae - less than 3mm
Purpura - 3-10mm
Ecchymoses - >10mm

85
Q

MCV=

A

mean corpuscular volume (aka the average size of RBC’s)

86
Q

Lead Poisoning Sx

A

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
Q

APS tx?

A

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
Q

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?

A

Disseminated intravascular coagulation (DIC)

Bleeding from multiple sights should make you think of DIC

89
Q

What is secondary hemostasis?

A

Activation of clotting factors forming a fibrin complex that stabilizes the platelet plug.

90
Q

What foods are vitamin B12 found in?

A

All foods of animal origin

91
Q

Dx and tx of protein C deficiency?

A

Dx: during w/u after thrombotic event with protein C assay test

Tx: acute, preventative with anticoags

92
Q

Describe a ferrous sulfate PO medication plan with a patient.

A
  • 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
Q

Symptoms of sickle cell anemia?

A

General symptoms of anemia as well as vaso-occlusive crises causing ischemic pain.

94
Q

What is polycythemia vera?

A

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
Q

T/F? ITP acquired in adulthood is less worrisome than in children

A

False - ITP acquired in adulthood is more likely to become chronic

96
Q

What is the pathophysiology of thrombotic thrombocytopenic purpura?

A

There is extensive microscopic intravascular clotting, which wastes a bunch of platelets. This decreases the functional amount of platelets in circulation.

97
Q

What is the most common cause for Vitamin B12 deficiency?

A

Pernicious anemia

98
Q

Give some examples of things that would increase a PT

A

Warfarin use, liver dysfunction, Vitamin K deficiency, DIC

99
Q

Iron is stored as ____ in the liver, spleen, bone marrow, & muscle

A

ferritin

100
Q

For a Pt with IDA, what are the indications for parental (IV) tx?

A

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
Q

Tx for hemophilia?

A

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
Q

What is the treatment for vitamin b12 deficiency?

A

Vitamin B12 injections - tapered down to only once a month after initial doses
PO cobalamin 1000mcg qd

103
Q

Anemia is defined as a decrease in the number of ______

A

fully functioning and circulating RBC’s

104
Q

Hemophilia is characterized by __________.

A

Prolonged coagulation time.

105
Q

T/F? Bleeding into joints, soft tissue, urine/stool, and brain are common in patients with hemophilia.

A

True - hemophilia can cause bleeding anywhere.

Joints, soft tissue, urine/stool, and brain are common sites of bleeding

106
Q

Which is the more severe cause of RBC destruction, causes intrinsic to the RBC or extrinsic to the RBC?

A

Intrinsic causes are more severe and harder to treat. Examples are sickle cell anemia and G6PD.

107
Q

IDA is classified as what type of anemia?

A

Microcytic hypochromic anemia– BUT may be normocytic anemia early on

108
Q

Serum Iron Lab value:

useful or nah?

A

Can have considerable daily variation, so NO it’s not exceptionally useful
–May be low or normal

109
Q

What medication should be avoided in patients with Von Willebrand’s Disease?

A

Aspirin

110
Q

if MCV is >100 fl =

A

Macrocytic anemia

111
Q

What are some symptoms of thrombocytopenia?

A

Evidence of capillary rupture, long bleeding time after trauma or easy bruising, mild gingival and nasal bleeding.

112
Q

T/F? HELLP syndrome is much more likely to occur in conjunction with pre/eclampsia.

A

True - 15% occur with pre/eclampsia vs. 0.7% occurring without.

113
Q

List relevant iron studies in order of sensitivity:

A
  • Ferritin
  • Total iron binding capacity (TIBC)
  • Serum iron
  • Transferrin saturation %
114
Q

MCH normal range=

A

26-34 pg/cell

115
Q

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?

A

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
Q

T/F? Spontaneous hemarthroses occurs during Von Willebrand’s Disease.

A

False - Hemarthroses does not occur during Von Willebrand’s Disease

117
Q

MCHC =

A

mean corpuscular hemoglobin concentration (aka average Hgb per RBC)

118
Q

How can a transferrin saturation % be indicative of IDA?

A

if it’s low

119
Q

You are seeing a patient with a low H & H with an MCV of 88. What lab do you order next?

A

Reticulocyte count

120
Q

Using hematocrit and Hgb, define anemia in an adult male.

A

Hematocrit: <41%

Hgb: < 13.5 g/dL

121
Q

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

A

e)

HELLP syndrome has fairly non-specific symptoms

122
Q

What is a PT and what is it used for?

A

Prothrombin time
IT evaluates the extrinsic pathway.
IT is also an excellent marker of current liver function

123
Q

What does TIBC measure?

A

Measures the capacity to bind iron

-normal range= 240-450 mcg/dL

124
Q

if MCV 80-100 fl =

A

normocytic anemia

125
Q

is protein S deficiency essentially the same as protein C deficiency just with a different letter?

A

yes, yes it is

126
Q

IDA Dx (list lab profile values)

A
  • Hgb: decreased
  • MCV: decreased
  • RDW: increased
  • Ferritin: decreased
  • TIBC/transferrin: increased
  • transferrin saturation %: decreased
127
Q

Thalassemias are broken down into which 3 things

A

Carriers (trait),
Minor (few to no Sx) and
Major (symptomatic)

128
Q

What finding on a blood smear is indicative of G6PD?

A

Heinz bodies

129
Q

Lead poisoning Tx

A
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
Q

Describe Normochromic, Hypochromic, and hyperchromic anemia using MCH and MCHC

A

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
Q

T/F? ADAMTS13 is the only cause of TTP.

A

False- is can occur secondary to another process.

132
Q

What is the pentad of sx for TTP?

A
Thrombocytopenic purpura
Microangiopathic hemolytic anemia
Neurological symptoms (seizure, AMS, TIA)
Fever
Renal failure
133
Q

Treatment for TTP?

A

ADMIT

corticosteroids, packed RBC’s, plasmapheresis (removal of vWF) aspirin, splenectomy

134
Q

What symptoms could present in a patient with Vitamin B12 deficiency that should not be present in a patient with a folic acid deficiency?

A

Patients with B12 deficiencies can have CNS complaints, like weakness, paresthesia, loss of vibratory sense and loss of balance.

135
Q

Vitamin K is necessary for synthesis of factor ____, ____, ____, and ____.

A

2, 7, 9, 10

136
Q

what will you see in a bone marrow aspiration with polycythemia vera?

A

will be hypercellular

137
Q

When can HELLP syndrome occur?

A

During the last trimester of pregnancy to several weeks post partum

138
Q

Tx for HELLP syndrome?

A

Induce delivery (highly favored after 34 weeks)
consider steroids
Platelet transfusions
Treat pre/eclampsia

139
Q

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?

A

Hemolytic uremic syndrome

140
Q

IDA Tx: PO Tx options

A
  • Diet: red meat, spinach, beans, lentils

- Ferrous Sulfate – 325mg TID (10mg absorbed)

141
Q

Sickle cell anemia is confirmed by what test?

A

Hemoglobin electrophoresis - finding 85-90% Hemoglobin S

142
Q

What is the cause of aplastic anemia

A

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
Q

if MCV <80 fl =

A

microcytic anemia

144
Q

What underlying enzyme defect is related to TTP?

A

ADAMTS13

145
Q

List 4 conditions that can cause increased destruction of RBCs

A
  • RBC membrane defect
  • Hgb defect
  • Autoimmune
  • Microangiopathic (hemolytic)
146
Q

Normal hemoglobin range for females?

A

12- 15 g/dl

147
Q

tx for polycythemia vera?

A

phlebotomy: one unit (500cc’s) weekly until <45%, then PRN

148
Q

What medication causes release of vWF, and is used before surgery in patients with Von Willebrand’s Disease?

A

Desmopressin

149
Q

For a Pt with suspected lead poisoning, what is the protocol

A

-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
Q

How can a TIBC value be indicative of IDA?

A

If it’s high (>450)

151
Q

Iron is an essential mineral and is necessary for functioning ____

A

Hgb

152
Q

Tx for sickle cell anemia?

A

Hydroxyurea 500-750mg QD to stimulate erythropoiesis
Blood transfusions as necessary
folic acid supplement

153
Q

How long after starting heparin might a patient develop HIT?

A

Usually about 5 days into treatment

154
Q

It is wise to give a prescription for folate when you give a prescription for what medication?

A

Methotrexate

155
Q

_______ anemia occurs when Hgb synthesis is reduced because of failure to incorporate iron–> iron is available, but the body can’t incorporate it

A

Sideroblastic anemia

156
Q

Christmas disease is a deficiency of factor _______.

A

IX
Christmas has 9 letters.
Christmas disease is a deficiency of factor 9

157
Q

IDA: S/Sx?

A
  • Tachycardia
  • DOE
  • Fatigue
  • Pale skin & mucosa
  • Brittle nails
  • Angular cheilitis
  • Pruritus
  • Pica
  • Anxiety, tingling, numbness
158
Q

Treatment for HUS in children?

A

Admission for IV fluids

159
Q

What is primary hemostasis?

A

Platelet activation, adhesion, and aggregation leading to creation of a hemostatic plug

160
Q

Normal hematocrit range for males

A

41-50%

161
Q

Give some examples of things that would increase an aPTT

A

Heparin use, hemophilia, DIC, APS

162
Q

What does the HELLP syndrome stand for?

A

Hemolysis, Elevated liver enzymes, and Low Platelet count