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Flashcards in Hematologic Disorders Deck (117):
1

examples of blood disorders

-hemochromatosis
-anemia
-leukocytosis
-leukopenia
-leukemia
-hodgkin's
-non-hodgkins lymphoma
-polycythemia vera
-thrombocytosis
-thrombocytopenia
-sickle cell disease
-thalassemias
-hemophilias
-splenomegaly
-multiple myeloma

2

Hemochromatosis

-accum of excess iron in many organs
-especially liver, pancreas, skin
-leads to cell damage and functional insufficiency

3

Disorders of Iron Absorption

-Hemochromatosis

4

Early Symptoms of Hemocrhomatosis

-fatigue
-heart palpitations
-imporence
-loss menstrual periods

5

S/Sx Hemochromatosis

-severe weakness/fatigue
-joint pain/abdominal pain (2nd/3rd MCP)
-signs liver disease, diabetes, heart probs
-impotence, infertility, loss menstrual periods
-porphyria, hepatitis

6

Hemochromatosis Blood Tests

-elevated:
--transferrin saturation >50%
--serum ferritin >1000 ng/ml
--transaminase
--blood glucose

7

Later Symptoms of Hemochromatosis

-grey/bronze skin
-cirrhosis of liver
-liver cancer
-DM
-hypopituitarism
-decreased pit/gonadal function
-joint disease
-heart failure
-abdominal pain
-fatigue
-certain infections

8

Disorderd of Erythrocytes

-Anemias

9

Anemia

-reduction of total number of RBC in blood or decrease in quality/quantity of Hb
-affects O2 transport

10

Anemias result from:

-decreased RBC production
-increased RBC destruction
-combo

11

Anemia S/Sx

-pallor
-SOB
-palpitations
-lethargy
-easily fatigued

12

decreased prod of RBC-->

-altered Hb synthesis
-altered DNA synthesis
-bone marrow infiltration
-red cell aplasia (fail to develop)
-kidney disease (erythropoietin)

13

Anemia Pathophysiology:

-decreased/defective production of RBC
-increased RBC destruction
-Blood loss

14

cause of RBC destruction

-infection causing hemolysis

15

Diagnostic Tests of Anemia

-HCT/Hb
-size of RBC
-serum iron level-serum ferritin
-iron-binding capacity in blood
-blood factors needed to absorb vitamins
-CBC

16

Types of Anemia

-pernicious (lack B12)--macrocytic
-folate--macrocytic
-iron-deficiency
-sideroblastic
-hemolytic
-post-hemorrhagic
-anemia of chronic disease

17

Pernicious Anemia

-autoimmune response
-lack intrinsic factor for B12 absorption
-possible genetic link

18

Pernicious Anemia more common in

-northern europeans
-female>males
-genetic

19

Pernicious Anemia Sx

-weakness
-fatigue
-paresthesias (feet/fingers)
-difficulty walking
-loss appetite
-abdominal pain
-atrophic glossitis
-skin yellow

20

B12 needed for

-myelin in axons and SC

21

atrophic glossitis

-beefy red tongue
-swollen with no center fold

22

Pernicious Anemia treatment

-replace B12 and intrinsic factor

23

Pernicious Anemia untreated

-permanent CNS damage

24

Folate Anemia causes

-impaired DNA synthesis and destruction of hematopoietic cells

25

S/Sx Folate Anemia

-cachexia
-sever cheilosis/angular stomatitis (cracks corners of mouth)

26

Treatment of Folate Anemia

-replace folate

27

Iron-Deficiency Anemia

-most common

28

Iron-Deficiency Anemia S/Sx

-pale skin
-fatigue
-irritability
-dizzy
-wakness
-SOB
-brittle nails
-decreased appetite
-frontal headache
-spoon nails
-restless leg syndrome
-pica

29

Iron Gets absorbed in:

-duodenum

-broken down in stomach acid

30

Iron-Deficiency Anemia Treatment

-replace iron: food
-red meat, liver, raisins, spinach, broccoli, egg yolks

31

Sideroblastic Anemia

-associated with alcoholism and medication use
-hereditary factor
-RBC still have nucleus

32

Sideroblastic Anemia Profile

-Male>female

33

Sideroblastic Anemia: Pathophys

-iron not synthesized into hemoglobin

34

S/Sx Sideroblastic Anemia

-spleen/liver enlargement
-bronze skin
-arrhythmias
-presence of sideroblasts

35

Sideroblastics Anemia Treatment

-pyridoxin therapy (B6)
-Iron depletion therapy
-phlebotomies

36

hemolytic anemia

-body destroying RBC
-caused by immune, drugs etc

37

Treatment of hemolytic anemia

-steroids for immunohemolytic anemia

38

Post-Hemorrhacig Anemia

-acute blood loss
-loss >1000ml of blood

39

Post-Hemorrhacig Anemia causes

-GI bleed from NSAID use

40

Post-Hemorrhacig Anemia Treatment

-restoration of blood volume

41

Anemia of Chronic Disease: disease examples

-AIDS
-chronic inflammatory disorders (RA, SLE, hepatitis, inflam bowel conditions)
-chronic renal failure

42

Treatment of Anemia of Chronic Disease

-treat underlying disorder to address anemia

43

Medical Management of Anemia

-alleviate/control cause

44

PT Guidelines for Anemic pt

-ex's under Dr. supervision
-anemia due to renal failure: max VO2 20% less than normal;
-Use RPE scale
-caution: chest pain

45

Sickle Cell Disease

-group of hereditary disorders characterized by an abnormal form of Hb within RBC
-RBC become sickle shaped after several cycles of oxygenation-deoxygenation

46

Sickle Cell Disease seen in:

-equitorial countries
-Africa

47

Sickle Cell Disease results in:

reduction of total # of RBC in blood or decrease in quality or quantity of Hb

48

S/Sx Sickle Cell Disease

-dactylitis (swelling fingers--clots in fingers/feet)
-spontaneous hemorrhages/infarctions
-pain (vascular occlusion)
-AVN of fem head
-osteomyelitis
-end stage renal failure
-blindness
-gallstones
-slenomegaly
-pneumonia

49

Extensive sickling can cause:

crisis

50

vasoocclusive crisis/thrombotic crisis

-cells obstruct blood flow

51

aplastic crisis

-profound anemia from deminished erythropoiesis

52

sequestration crisis

-large amts blood pooled in spleen and liver

53

hyperhemolytic crisis

-increased RBC lysis;
-can lead to death

54

Treatment of sickle cell disease

-address symptoms
-folate in diet
-bone marrow transplantation

55

Chest Syndrome

-sickled cells adhere to lung endothelium
-Sx: chest pain, SOB, fever, wheeing, cough, pulm HTN
-poor prognosis

56

Sickle cell disease under age 20

-stroke in 11%

57

Complications of Sickle cell disease

-hand and foot syndrome
-jaundice
-anemia
-autosplenectomy
-stroke
-chest syndrome

58

medical management of SCD

-transfusion
-bone marrow transplant
-stem cell transplant
-education
-emergency med attn
-pn control
-caution with ice: vasoconstriction

59

Prognosis of SCD

-life expectancy
-men: 42
-women: 48

60

SCD + Exercise

-low peak VO2
-low anaerobic threshold
-gas exchange abnormalities

61

Examples of Disorders of Abnormal Blood Component Levels

-Anemia
-Polycythemia
-Leukocytosis
-Leukopenia
-Thrombocytosis
-Thrombocytopenia

62

Polycythemia Vera

-Excess production of blood constituents (RBC, WBC, leukocytes) in the bone marrow
-causes increased blood volume and viscosity

63

Cause of Polycythemia Vera

-unknown
-likely acquired genetic alterations
-may have connection to intense radiation exposure
-common response to hypoxia (living at high altitudes)

64

Polycythemia causes increased risk for:

-thrombi
-angina
-extremely painful itching

65

Treatment of Polycythemia Vera

-reduce RBC and blood volume to prevent thrombosis
-phlebotomies
-radioactive phosphorus suppresses hematopoeisis/erythropoeisis

66

Side effects of radioactive phosphorus

-anemia
-leukopenia
-thrombocytopenia

67

Leukocytosis

-higher than normal leukocyte count
-normal protective response to physiologic stressors
-can occur with malignancies and hematological disorders

68

WBC Count in infectiouse diseases

->10,000 WBC/mm3

69

Leukopenia

-lower than normal leukocyte count
-increases risk for infection

70

Neutropenia

-lower than normal levels of neutrophils
-less than 2000/microliter

71

Neutropenia occurs with:

-prolonged infections
-autoimmune disorders
-chemotherapy
-defective neutrophil production or regulation

72

Thrombocytosis

-increased # of platelets
-over 750,000 is serious
-primary vs secondary

73

Thrombocytosis can increase risk of:

-thrombus
-but no symptoms

74

Primary Thrombocytosis

-"essential"
-due to myeloproliferative disease

75

Secondary Thrombocytosis

-"reactive"
-response to inflammatory process

76

Platelet Levels in Thrombocytosis

->750,000 platelets/mm3

77

Thrombocytopenia

-low platelet count
-<100,000 platelets/mm3

78

Thrombocytopenia can cause increased risk for:

-hemorrhage with trauma

79

______is the most common drug induced thrombocytopenia

-Heparin

80

Causes of Thrombocytopenia

-platelet destruction
-lack of platelet formation

-associated with congenital and acquired diseases

81

Leukemia

-abnormal proliferation of WBC in blood and other organs
-symptoms suggesting acute infection
-severe: enlarged lymph nodes or spleen

82

Leukemia Pathophys

-increased WBC production inhibits normal cell production
-causing: anemia, bleeding, infection

83

Types of Leukemia

-ALL
-AML
-Chronic Lymphocytic Leukemia
-Chronic Myelogenous Leukemia

84

Classification of Leukemia based on:

-acute vs chronic
-predominant cell type: lymphocytic, vs. myelogenous

85

Lymphocytic

-originates in bone marrow
-infiltrates other tissues
(lymphatic system)

86

Myelogeous

-myeloid stem cells (bone marrow)
-interfere with all blood cell maturation (hematopoeitic cells)

87

Acute Leukemia

-Blocking of cell-differentiation of cells (B cells)
-immature blast cells
-adults>children

88

ALL

-Acute Lymphoblastic Leukemia
-most prevalent leukemia in children

89

AML

-Acute Myelogenous Leukemia

90

Chronic Leukemia

-mature abnormal cells
-unknown cause

91

CML

-Chronic Myelogenous Leukemia
-common with radiation exposure
-genetic component

92

CLL

-Chronic Lymphocytic Leukemia
-most common leukemia in Western society

93

S/Sx Leukemia

-Infection
-fever
-bleeding
-anemia
-bone/joint pain
-neurologic manifestations
-enlarged spleen
-extreme fatigue
-weight loss
-lymphadenopathy
-night sweats

94

Leukemia treatment

-chemo
-remission induction therapy
-supportive care
-direct treatment of infection
-bone marrow transplant
-immunnotherapy-evolving
-Stem cell transplantation
-palliative care

95

Malignant Lymphomas

-Cancers in lymphatic system

-Hodgkin's Disease
-Non-Hodgkin's Lymphoma

96

HD

-Hodgkin's Disease

97

HD characterized by presence of:

-Reed-Sternberg Cells (owl eyes)
-cell most likely the malignant site

98

HD Profile

-young adults
-peak 20s-30s
-8% of all lymphomas

99

Risk Factors of HD

-HIV/AIDS
-Mononucleosis infection
-chronic illness
-immunosuppressants
-Cancer treatment
-SLE
-autoimmune diseases
-ulcerative colitis
-drug abuse
-obesity

100

HD S/Sx

-enlarged painless lymph nodes
-fatigue
-fever
-night sweats
-pruritis
-weight loss
-pulm/radicular symptoms (spread to CNS rare)
-progression-->immunodeficiency and infections

101

HD Treatment

-biopsy to stage
-radiation
-chemo

102

HD Prognosis

-good
-one of the most curable forms of cancer

103

NHL

-Non-hodgkin's Lymphoma
-solid tumors from lymph system cells

104

NHL Risk Factors

-age
-gender (male)
-environment
-viral infection
-immunocompromise

105

S/Sx NHL

-variable
-starts with lymphadenopathy

106

NHL Diagnosis

-biopsy is gold standard

107

NHL Treatment

-chemo
-radiation

108

NHL Prognosis

-depends how wide spread

109

Hemophilia

-Type A-C

-A/B: X-linked recessive
-C: autosomal recessive

110

Hemophilia Type A

-hereditary
-cause deficiency in factor VIII
-most common

111

Hemophilia Type B

-Hereditary
-cause deficiency in factor IX

112

von Willebrand disease

-hereditary
-cause deficiency in vWF
-most common bleeding disorder

113

vWF

-von Willebrand Factor
-transports/facilitates action of factor VIII

114

Hemophilia Treatment

-transfusion of plasma enriched with factor VIII

115

Hemophilia S/Sx

-bleeding
-bruising
-arthropathy (due to bleeding in joints)
-intramuscular hemorrhage

116

Benefits of Exercise in Bleeding Disorders

-improve work capacity
-joint protection through shock absorption
-decrease frequency of bleeds
-temporarily increase levels of clotting factors (VIII)
-improve immune function

117

Bleedign Disorder S/Sx

-fatigue
-joint pain