Hematologic Disorders Flashcards

(117 cards)

1
Q

examples of blood disorders

A
  • hemochromatosis
  • anemia
  • leukocytosis
  • leukopenia
  • leukemia
  • hodgkin’s
  • non-hodgkins lymphoma
  • polycythemia vera
  • thrombocytosis
  • thrombocytopenia
  • sickle cell disease
  • thalassemias
  • hemophilias
  • splenomegaly
  • multiple myeloma
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2
Q

Hemochromatosis

A
  • accum of excess iron in many organs
  • especially liver, pancreas, skin
  • leads to cell damage and functional insufficiency
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3
Q

Disorders of Iron Absorption

A

-Hemochromatosis

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4
Q

Early Symptoms of Hemocrhomatosis

A
  • fatigue
  • heart palpitations
  • imporence
  • loss menstrual periods
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5
Q

S/Sx Hemochromatosis

A
  • severe weakness/fatigue
  • joint pain/abdominal pain (2nd/3rd MCP)
  • signs liver disease, diabetes, heart probs
  • impotence, infertility, loss menstrual periods
  • porphyria, hepatitis
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6
Q

Hemochromatosis Blood Tests

A
  • elevated:
  • -transferrin saturation >50%
  • -serum ferritin >1000 ng/ml
  • -transaminase
  • -blood glucose
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7
Q

Later Symptoms of Hemochromatosis

A
  • grey/bronze skin
  • cirrhosis of liver
  • liver cancer
  • DM
  • hypopituitarism
  • decreased pit/gonadal function
  • joint disease
  • heart failure
  • abdominal pain
  • fatigue
  • certain infections
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8
Q

Disorderd of Erythrocytes

A

-Anemias

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9
Q

Anemia

A
  • reduction of total number of RBC in blood or decrease in quality/quantity of Hb
  • affects O2 transport
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10
Q

Anemias result from:

A
  • decreased RBC production
  • increased RBC destruction
  • combo
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11
Q

Anemia S/Sx

A
  • pallor
  • SOB
  • palpitations
  • lethargy
  • easily fatigued
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12
Q

decreased prod of RBC–>

A
  • altered Hb synthesis
  • altered DNA synthesis
  • bone marrow infiltration
  • red cell aplasia (fail to develop)
  • kidney disease (erythropoietin)
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13
Q

Anemia Pathophysiology:

A
  • decreased/defective production of RBC
  • increased RBC destruction
  • Blood loss
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14
Q

cause of RBC destruction

A

-infection causing hemolysis

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15
Q

Diagnostic Tests of Anemia

A
  • HCT/Hb
  • size of RBC
  • serum iron level-serum ferritin
  • iron-binding capacity in blood
  • blood factors needed to absorb vitamins
  • CBC
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16
Q

Types of Anemia

A
  • pernicious (lack B12)–macrocytic
  • folate–macrocytic
  • iron-deficiency
  • sideroblastic
  • hemolytic
  • post-hemorrhagic
  • anemia of chronic disease
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17
Q

Pernicious Anemia

A
  • autoimmune response
  • lack intrinsic factor for B12 absorption
  • possible genetic link
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18
Q

Pernicious Anemia more common in

A
  • northern europeans
  • female>males
  • genetic
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19
Q

Pernicious Anemia Sx

A
  • weakness
  • fatigue
  • paresthesias (feet/fingers)
  • difficulty walking
  • loss appetite
  • abdominal pain
  • atrophic glossitis
  • skin yellow
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20
Q

B12 needed for

A

-myelin in axons and SC

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21
Q

atrophic glossitis

A
  • beefy red tongue

- swollen with no center fold

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22
Q

Pernicious Anemia treatment

A

-replace B12 and intrinsic factor

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23
Q

Pernicious Anemia untreated

A

-permanent CNS damage

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24
Q

Folate Anemia causes

A

-impaired DNA synthesis and destruction of hematopoietic cells

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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