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
Q

What is the treatment for B12 deficiency?

A

IM injection of B12. Daily for first week, weekly for first month, then monthly for life.

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26
Q

What are the key factors for the intrinsic pathway?

A

Key factors VIII, IX, XI, XII

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27
Q

What are the key factors for the extrinsic pathway?

A

Key factor VII

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28
Q

The intrinsic and extrinsic pathways converge to the common pathway comprised of what key component factors?

A

X, V, II

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29
Q

What lab measurement checks the function of the intrinsic pathway?

A

PTT

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30
Q

What lab measurement checks the function of the extrinsic pathway?

A

PT

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31
Q

What is the congenital deficiency of coagulation factor VIII?

A

Hemophilia A

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32
Q

What is the congenital deficiency of coagulation factor IX?

A

Hemophilia B

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33
Q

What is a systematic process with the potential for causing thrombosis and hemorrhage, it can present as an acute, life-threatening emergency?

A

Disseminated intravascular coagulation (DIC)

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34
Q

What is the major principle in the management of DIC?

A

Treating the underlying cause

35
Q

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
A

DIC

36
Q

What labs would you do for DIC ?

A
  1. PT
  2. PTT
  3. INR
  4. CBC
37
Q

What is a X chromosome linked recessive disorder seen commonly in American black men?

A

G6PD deficiency

38
Q

What percentage of American black men are affected by G6PD?

A

10-15%

39
Q

Episodic hemolysis in response to oxidant drugs or infection is what?

A

G6PD deficiency

40
Q

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?

A

G6PD Deficiency

41
Q

Oxidized hemoglobin denatures and forms precipitants called what? What do they do?

A

Heinz bodies, cause membrane damage

42
Q

What is the treatment for G6PD deficiency ?

A

no treatment is necessary except to avoid oxidants

**remember primaquine**

43
Q

What is an autosomal recessive disorder in which an abnormal hemoglobin (hemoglobin s) leads to chronic hemolytic anemia with numerous clinical consequences ?

A

Sickle cell anemia

44
Q

The hemoglobin S gene is carried in who?

A

8% of American blacks and 1 of 400 American black children

45
Q

Physical exam findings for what?

Jaundice, pigment (calcium bilirubinate) gallstones, hepatosplenomegaly, and poorly healing ulcers over the lower tibia.

A

Chronic hemolytic anemia associated with sickle cell anemia

46
Q

Sickle cell anemia can cause what physical exam finding or symptom? **

A

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
Q

Labs/Studies/EKG

Sickle cell anemia

A

CBC w/ Dif *****
Hematocrit is usually low 20-30%

48
Q

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.

A

80%

49
Q

What is the mainstay of treatment for sickle cell ?

A

Supportive care

50
Q

What medication can you give a patient with sickle cell?

A

folic acid supplements 1mg oral daily

51
Q

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?

A

between 40 and 50 years of age

52
Q

Persons with sickle cell are usually clinically normal but can have acute painful episodes only under what conditions?

A

under extreme conditions such as vigorous exertion at high altitudes or in an unpressurized aircraft

53
Q

What is a malignancy of the hematopoietic progenitor cell?

A

Leukemia

54
Q

What are the classifications of leukemia?

A
  1. Acute myeloid leukemia (AML)
  2. Acute lymphoblastic leukemia (ALL)
55
Q

Which form of leukemia comprises 80% of the acute leukemias of childhood and causing only 20% of adult leukemias?

A

Acute lymphoblastic leukemia (ALL)

56
Q

What form of leukemia is primarily an adult disease with a median age of 60 years old and an increasing incidence with advanced age?

A

Acute myeloid Leukemia (AML)

57
Q

What percentage of patients typically have blast cells present in their blood with leukemia?

A

90%

58
Q

What do most patients with leukemia initially complain about?

A

fatigue

59
Q

In leukemia bone tenderness may be present where?

A
  1. Sternum
  2. Tibia
  3. Femur
60
Q

What would a good differential diagnosis be for leukemia?

A

Viral infection such as mononucleosis ***

61
Q

Leukemia Labs/studies/EKG

A
  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
Q

Approximately what percentage of adults with AML under the age of 60 years old achieve complete remission?

A

70-80%

63
Q

Chemotherapy leads to cure in what percentage of adult patients?

A

35-40%

64
Q

Bone marrow transplantation (younger adults with HLA-matched siblings) is curative in what percent of patients?

A

50-60%

65
Q

What is a high white cell count relative to normal physiologic numbers ?

A

Leukocytosis

66
Q

What is a low white cell count?

A

Leukopenia (<4400 cell/microL)

67
Q

The different etiologies of leukocytosis can be determined by what?

A

What cell line is involved

68
Q

What form of leukocytosis could this be?

  • Bacterial infection
  • Inflammation
  • Metabolic disease
A

Neutrophilia

69
Q

What form of leukocytosis could this be?

  • Viral Infection
  • Immune disease
  • Stress
  • Leukemia
A

Lymphocytosis

70
Q

What form of leukocytosis could this be?

  • Skin diseases
  • Drug reaction
  • Parasite infection
  • Asthma
A

Eosinophilia

71
Q

What form of leukocytosis could this be?

-Chronic myeloid leukemia

A

Basophilia

72
Q

What form of leukocytosis could this be?

  • Infection
  • Autoimmune disease
A

Monocytosis

73
Q

A CBC w/ dif will determine what for leukocytosis and leukopenia?

A

the different cell line being affected

74
Q

What is known as an abnormally low amount of circulating platelets ?

A

Thrombocytopenia

75
Q

The risk of relevant spontaneous bleeding does not typically increase until the platelet count falls below what?

A

10,000-20,000/mcL unless the patient has dysfunctional platelets

76
Q

Symptoms/Physical exam findings for thrombocytopenia

A

PETECHIA will help you determine if the deficiency is platelets rather than a coagulopathy (which would cause BRUISING)

77
Q

What is known as an abnormally high amount of circulating thrombocytes? (>450,000)

A

Thrombocytosis

78
Q

What are the two different categories that thrombocytosis can be divided into?

A
  1. Reactive Thrombocytosis
  2. Autonomous Thrombocytosis
79
Q

Thrombocytosis in response to things like anemia, blood loss, or infection is known as what?

A

Reactive Thrombocytosis

80
Q

Thrombocytosis that is from etiologies of primarily cancer of bone marrow is known as what?

A

Autonomous thrombocytosis

81
Q

What is the median age of presentation for thrombocytosis ?

A

50-60 years old

82
Q

In what percentage of patients with thrombocytosis is splenomegaly present?

A

25%

83
Q

When would you refer a patient with thrombocytosis to hematology?

A

if there is no sign of infectious cause or it does not resolve on its own in 2-4 weeks