CLINICAL CARE OF THE HEMATOLOGIC SYSTEM Flashcards

1
Q

What is defined by a hematocrit in adults less than 41% in males or less than 37% in females?

A

Anemia

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2
Q

Anemias are classified according to either?

A
  1. Pathophysiologic basis
  2. according to cell size
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3
Q

What is commonly the cause of iron deficiency in adults?

A

bleeding

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4
Q

Labs/studies/EKG
Anemia

A
  1. CBC w/ Dif
  2. Iron with total iron binding capacity (TIBC)
    a. ferritin value <12mcg/L indicates Fe deficiency anemia
  3. Microscopic analysis
  4. Hemoglobin electrophoresis (evaluate for alpha or beta thalassemia)
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5
Q

What is the most important treatment of anemia?

A

Identification of the cause of blood loss, especially a source of occult blood loss

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6
Q

What is the most common cause of anemia world wide?

A

Iron deficiency

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7
Q

A serum ferritin level below 12mcg/L would be considered what?

A

Iron deficiency anemia

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8
Q

True or False

Anemia in males is possibly due to a disease process and requires further evaluation and a higher level of care

A

True

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9
Q

True or False

Prolonged aspirins or other anti-inflammatory drugs, may cause GI bleeding even without a lesion

A

True

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10
Q

Many patients with this develop pica, a craving for specific foods, often not rich in iron

A

Iron deficiency anemia

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11
Q

These are all physical findings for what?

  • fatigability
  • tachycardia
  • palpitations
  • tachypnea on exertion
  • smooth tongue
  • brittle nails
  • cheilosis
A

Iron deficiency anemia

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12
Q

Lab/Image findings for iron deficiency anemia

A
  1. CBC w/ dif
    a. decreased mean corpuscular volume (MCV)
  2. Iron with total iron binding capacity (TIBC)
  3. Ferritin value less than 12mcg/L
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13
Q

What is the most important treatment for iron deficiency anemia?

A

identification if the cause of blood loss, especially occult blood loss

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14
Q

What is the dose of oral iron supplement for patients with Iron deficiency anemia?

A

325mg 3 times a day for 3-6 months

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15
Q

What is the most common reason for oral iron supplements to not work?

A

patient non-compliance

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16
Q

When should you refer a patient with iron deficiency anemia to a hematologist?

A

if the patient is unresponsive to iron therapy

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17
Q

What is the daily absorption rate for vitamin B12?

A

5mcg

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18
Q

A serum vitamin B12 level of less than 100pg/mL would indicate what?

A

Vitamin B12 deficiency

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19
Q

How many mcg of stored vitamin B12 does the liver contain?

A

2000-5000mcg

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20
Q

Dietary vitamin B12 deficiency is extremely rare and is usually only seen in what?

A

Vegans

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21
Q

How long could it take for vitamin B12 deficiency to become apparent?

A

more than 3 years due to the body low daily losses

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22
Q

True or False

Abdominal surgery can lead to B12 deficiency

A

True

surgical resection of the ileum will eliminate the site of vitamin B12 absorption

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23
Q

What are these physical findings associated with?

  • Glossitis
  • Anorexia
  • Diarrhea
  • late stage patients will be pale
  • paresthesia
  • difficulty with balance
A

Vitamin B12 deficiency

****NEURO FINDINGS ARE UNIQUE TO THIS****

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24
Q

Lab/Image findings

Vitamin B12 deficiency

A
  1. CBC w/ dif - hallmark is megaloblastic anemia (large RBC’s)
  2. Strikingly high MCV
  3. Abnormally low B12 serum level. Deficiency <170pg/mL.
    Symptomatic <100pg/mL.
  4. Elevated LDH and bilirubin
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25
What is the treatment for B12 deficiency?
IM injection of B12. Daily for first week, weekly for first month, then monthly for life.
26
What are the key factors for the intrinsic pathway?
Key factors VIII, IX, XI, XII
27
What are the key factors for the extrinsic pathway?
Key factor VII
28
The intrinsic and extrinsic pathways converge to the common pathway comprised of what key component factors?
X, V, II
29
What lab measurement checks the function of the intrinsic pathway?
PTT
30
What lab measurement checks the function of the extrinsic pathway?
PT
31
What is the congenital deficiency of coagulation factor VIII?
Hemophilia A
32
What is the congenital deficiency of coagulation factor IX?
Hemophilia B
33
What is a systematic process with the potential for causing thrombosis and hemorrhage, it can present as an acute, life-threatening emergency?
Disseminated intravascular coagulation (DIC)
34
What is the major principle in the management of DIC?
Treating the underlying cause
35
What are these symptoms/physical exam findings of? - Bruising without trauma - Bleeding into joint spaces - Epistaxis - Bleeding from eyes - Very heavy vaginal bleeding for prolonged periods of time in females
DIC
36
What labs would you do for DIC ?
1. PT 2. PTT 3. INR 4. CBC
37
What is a X chromosome linked recessive disorder seen commonly in American black men?
G6PD deficiency
38
What percentage of American black men are affected by G6PD?
10-15%
39
Episodic hemolysis in response to oxidant drugs or infection is what?
G6PD deficiency
40
What is a hereditary enzyme defect that causes episodic hemolytic anemia because of the decreased ability of red blood cells to deal with oxidative stresses?
G6PD Deficiency
41
Oxidized hemoglobin denatures and forms precipitants called what? What do they do?
Heinz bodies, cause membrane damage
42
What is the treatment for G6PD deficiency ?
no treatment is necessary except to avoid oxidants \*\*remember primaquine\*\*
43
What is an autosomal recessive disorder in which an abnormal hemoglobin (hemoglobin s) leads to chronic hemolytic anemia with numerous clinical consequences ?
Sickle cell anemia
44
The hemoglobin S gene is carried in who?
8% of American blacks and 1 of 400 American black children
45
Physical exam findings for what? Jaundice, pigment (calcium bilirubinate) gallstones, hepatosplenomegaly, and poorly healing ulcers over the lower tibia.
Chronic hemolytic anemia associated with sickle cell anemia
46
Sickle cell anemia can cause what physical exam finding or symptom? \*\*
Acute painful episodes include the bones (especially the back and long bones) and the chest. These episodes last hours to days and may produce low-grade fever.
47
Labs/Studies/EKG Sickle cell anemia
CBC w/ Dif \*\*\*\*\* Hematocrit is usually low 20-30%
48
When allogeneic hematopoietic stem cell transplantation is performed before onset of significant end organ damage, it can cure more than \_\_\_\_% of children with sickle cell anemia who have a suitable HLA matched donor.
80%
49
What is the mainstay of treatment for sickle cell ?
Supportive care
50
What medication can you give a patient with sickle cell?
folic acid supplements 1mg oral daily
51
Sickle cell anemia becomes a chronic multisystem disease, leading to organ failure that may result in death. With improved supportive care, average life expectancy is now what?
between 40 and 50 years of age
52
Persons with sickle cell are usually clinically normal but can have acute painful episodes only under what conditions?
under extreme conditions such as vigorous exertion at high altitudes or in an unpressurized aircraft
53
What is a malignancy of the hematopoietic progenitor cell?
Leukemia
54
What are the classifications of leukemia?
1. Acute myeloid leukemia (AML) 2. Acute lymphoblastic leukemia (ALL)
55
Which form of leukemia comprises 80% of the acute leukemias of childhood and causing only 20% of adult leukemias?
Acute lymphoblastic leukemia (ALL)
56
What form of leukemia is primarily an adult disease with a median age of 60 years old and an increasing incidence with advanced age?
Acute myeloid Leukemia (AML)
57
What percentage of patients typically have blast cells present in their blood with leukemia?
90%
58
What do most patients with leukemia initially complain about?
fatigue
59
In leukemia bone tenderness may be present where?
1. Sternum 2. Tibia 3. Femur
60
What would a good differential diagnosis be for leukemia?
**Viral infection such as mononucleosis \*\*\***
61
Leukemia Labs/studies/EKG
1. hallmark of acute leukemia is the combination of pancytopenia with circulating blasts * *2. MORE THAN 20% BLASTS ARE REQUIRED TO MAKE A DIAGNOSIS OF ACUTE LEUKEMIA**
62
Approximately what percentage of adults with AML under the age of 60 years old achieve complete remission?
70-80%
63
Chemotherapy leads to cure in what percentage of adult patients?
35-40%
64
Bone marrow transplantation (younger adults with HLA-matched siblings) is curative in what percent of patients?
50-60%
65
What is a high white cell count relative to normal physiologic numbers ?
Leukocytosis
66
What is a low white cell count?
Leukopenia (\<4400 cell/microL)
67
The different etiologies of leukocytosis can be determined by what?
What cell line is involved
68
What form of leukocytosis could this be? - Bacterial infection - Inflammation - Metabolic disease
Neutrophilia
69
What form of leukocytosis could this be? - Viral Infection - Immune disease - Stress - Leukemia
Lymphocytosis
70
What form of leukocytosis could this be? - Skin diseases - Drug reaction - Parasite infection - Asthma
Eosinophilia
71
What form of leukocytosis could this be? -Chronic myeloid leukemia
Basophilia
72
What form of leukocytosis could this be? - Infection - Autoimmune disease
Monocytosis
73
A CBC w/ dif will determine what for leukocytosis and leukopenia?
the different cell line being affected
74
What is known as an abnormally low amount of circulating platelets ?
Thrombocytopenia
75
The risk of relevant spontaneous bleeding does not typically increase until the platelet count falls below what?
10,000-20,000/mcL unless the patient has dysfunctional platelets
76
Symptoms/Physical exam findings for thrombocytopenia
**PETECHIA** will help you determine if the deficiency is platelets rather than a coagulopathy (which would cause **BRUISING**)
77
What is known as an abnormally high amount of circulating thrombocytes? (\>450,000)
Thrombocytosis
78
What are the two different categories that thrombocytosis can be divided into?
1. Reactive Thrombocytosis 2. Autonomous Thrombocytosis
79
Thrombocytosis in response to things like anemia, blood loss, or infection is known as what?
Reactive Thrombocytosis
80
Thrombocytosis that is from etiologies of primarily cancer of bone marrow is known as what?
Autonomous thrombocytosis
81
What is the median age of presentation for thrombocytosis ?
50-60 years old
82
In what percentage of patients with thrombocytosis is splenomegaly present?
25%
83
When would you refer a patient with thrombocytosis to hematology?
if there is no sign of infectious cause or it does not resolve on its own in 2-4 weeks