UA WI18-Chapter 10 Blood Disorders Part 1 Flashcards

(131 cards)

1
Q

A decrease in the number of RBC or less than normal quantity of Hb in blood.

A

Anemia

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2
Q

Increased amount of RBC in blood.

A

Polycythemia

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3
Q

What are the three main classes of anemia?

A

Excessive blood loss

Deficient RBC production

Excessive blood cell destruction

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4
Q

Sx. Common to all anemia.

A

Weakness, fatigue, pallor, malaise, dyspnea, tachycardia, murmur(systolic), low blood pressure, dizziness, headaches coldness, weak pulse etc.

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5
Q

Acute blood loss is caused _______ and results in _______.

A

Trauma, surgery, GI tract lesions, Gynecolgic disturbance

Results in hypovolemia

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6
Q

Term for decreased blood volume

A

Hypovolemia

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7
Q

In an acute blood loss case report, what would be found?

A

Decreased platelet

Increased neutrophils (shift left)

Changes in RBC morphology: normal initially—> hypochromic once volume restored

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8
Q

Chronic blood loss is caused by______.

When do symptoms appears?

A

Cancer, menstruation, fibroids in older women and angiodysplasia.

When iron store is depleted

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9
Q

In a chronic bleeding case report, what is reported?

A

Elevated platelets

Leukopenia

RBC morphology: Normocytic or microcytic/ hypochromic

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10
Q

What are the causes of deficient RBC production?

A

Iron-deficiency anemia

Vit. Deficiency anemia

Bone marrow or stem cell problem

Health disorders: i.e renal insufficiency

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11
Q

Iron deficient anemia is described as?

A

Low RBC amount or low Hb

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12
Q

Iron deficiency anemia causes?

A

Insufficient intake

Duodenal malabsorption

Iron loss from bleeding

Increased need- e.g growth spurt

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13
Q

Iron deficiency clinical presentation?

A
PICA
Koilonychia-spoon shaped brittle nails
Sleepiness
Angular cheilitis
Mouth ulcers
Hair loss
Missed menstrutation 
Depression
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14
Q

Iron deficiency hematology report?

A

HYPOCHOMIC/MICROCYTIC

Anisocytosis/poikilocytosis

Leukocytes unaffected

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15
Q

Megaloblastic anemia is described as?

A

A condition is which the bone marrow produces unusually large, structurally abnormal immature RBC

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16
Q

Causes of megaloblastic anemia

A

Vit. B12 , Vit. B9 or both

Both needed for maturation of blood cell and normal nerve cell function

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17
Q

Abnormal DNA synthesis and Delayed cell division, with normal Hb & RNA synthesis in megaloblastic anemia is a result of ?

A

B9 and B12 deficiency

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18
Q

Etiological factors of B12 deficiency

A

Deficient intake

Lack of IF

Malabsorption syndrome-ileum

Worm infection

Increased used (pregnancy)

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19
Q

Etiological factors of B9 deficiency

A

Deficient intake

Alcoholism

Malabsorption- duodenum, jejunum

Increased use (pregnancy)

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20
Q

Clinical manifestation of megaloblastic anemia a/w B12 deficiency?

A

Fatigue, dyspnea, muscle weakness, glossitis, GI problems

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21
Q

Megaloblastic anemia a/w with B12 deficiency will result in _______ which is characterized by_______.

A

Result in: irreversible nervous system pathology collectively know as Subacute combined degeneration of spinal.

Characterized by: Demyelination of posterior and lateral columns and psychological problems.

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22
Q

What is the AKA term for subacute combined degeneration of spinal cord?

A

Lichtheim disease

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23
Q

Lichtheim disease is clinically manifested by:

A

Numbness/tingling in fingers extremities

Loss of balance, vibration and ataxia

Paraplegia

Depression, dementia

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24
Q

Tx for megaloblastic anemia a/w B12 deficiency

A

B12 by supplement

Injection of b12

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25
Autoimmune disorder in which the body can’t make enough healthy RBC because it can’t absorb sufficient amounts og B12.
Pernicious anemia
26
Pernicious anemia produces Ab against_____?
Parietal cells Intrinsic factor Intrinsic factor receptors in ileum
27
Congenital pernicious is described as
Pernicious anemia passed down. -Babies w/ this can’t make enough IF or absorb B12 in small intestines so tx requires intramuscular B12 shots-
28
Megaloblastic anemia a/w B9 clinical manifestation
Angular cheilitis/glossitis, fatigue, dyspnea, muscle weakness, GI problems and Depression. No Neurological symptoms
29
Megaloblastic anemia a/w B9 affects pregnancy in what way?
Neural tube defects Low birth rates
30
Megaloblastic anemia hematology
Pancytopenia Hypersegmented neutrophils (senile) Morphology: Macrocytic, anisocytosis/poikilocytosis- Howell-jolly bodies MCV/ MCH increase or normal LDH increased
31
Disease in which the bone marrow and the blood stem cells are damaged.
Aplastic anemia
32
Causes of aplastic anemia
50% idiopathic Infection: Ds- hepatitis B/C, Epstein-Barr, Ss-parvovirus Ionizing radiation AI (e.g SLE) Drugs
33
Aplastic anemia clinical manifestations
Fatigue, pallor, tachycardia/palpitations/dyspnea, weakness
34
Thrombocytopenia results from aplastic anemia, what are associated risk?
Hemorrhage Bruising Petechiae
35
Leukopenia in aplastic anemia is associated with what risk?
Risk of Infection
36
Definition: low counts of immature RBC/reticulocytes
Reticulocytopenia | Result of Aplastic anemia
37
Aplastic anemia laboratory findings
Pancytopenia Normochromic/normocytic Decreased granulocytes (WBC) Increased serum ion (no RBC to take them)
38
Anemia in which the bone marrow and liver produce ringed sideroblast rather than healthy red blood cells.
Sideroblastic anemia
39
Define a sideroblast
Atypical abnormal uncleared erythoblast w/ iron granules
40
Why do sideroblast form ?
Due to reduce porphyria synthesis which helps incorporate iron into heme
41
Sideroblastic anemia etiology
50% idiopathic 30% acquired (MM, SLE, Liver cirrhosis etc) 20% hereditary (X-linked)
42
Sideroblastic anemia Sx/signs
Enlarged liver and spleen (MC) Skin paleness Fatigue Dizziness
43
Sideroblastic anemia lab finding
Ring sideroblast in bone marrow Anisocytosis/poikilocytosis/ basophillic stippling, target cells Increased serum iron & transferrin saturation
44
Anemia due to hemolysis, the abnormal breakdown of RBC
Hemolytic anemia | Intravascular or extravascular
45
Intrinsic etiology of hemolytic anemia include
Defect of RBC membrane production Defect in hemoglobin production Defective RBC metabolism
46
Extrinsic etiology of hemolytic anemia include
Immune-mediated disorders Drug side effects Trauma
47
Sx of hemolytic anemia
Fatigue Shortness of breath Jaundice Gallstone (due to increased bilirubin) Pulmonary hypertension (due to abnormal bilirubin)
48
Hemolytic anemia lab findings
Increased serum bilirubin (feces and urinary as well) Anisocytosis/poikilocytosis/ reticulocytosis Elevated LDH
49
Intrinsic hemolytic anemia characterized by the production of RBCs that are sphere-shaped and prematurely breakdown.
Hereditary spherocytic anemia
50
The sphere shaped RBC a/w hereditary spherocytic anemia is a result of ?
Membrane lipoprotein defects
51
Sx of hereditary spherocytosis
Fatigue, dyspnea Jaundice Gallstones Pulmonary hypertension Splenomegaly Aplastic crisis (temporary)
52
Hereditary spherocytosis lab
RBC- microcytic, hyperchromic MCV low/ MCH normal Increased unconjugated bilirubin in blood Increased urobilinogen in ruins and stool
53
Intrinsic anemia disorder a/w an inherited autosomal recessive hemoglobinopathy, characterized by structurally abnormal Hb. Qualitative defects
Sickle cell (group of disorders)
54
Sickle cell occurs when the _____, ______ on the ______ in the Hb molecule is replaced by other amino acids
Sixth amino acid Glutamic acid Beta-globin chain
55
The three detected mutation of sickle cells are
HbS- glutamic replaced with valine (E6V) HbC- glutamic replaced with lysine (E6K) HbE- Glutamic replaced with lysine on the 26 position on beta globin (E26K)
56
Sickle cell disease found in which geographic locations?
African, Caribbean, Mediterranean, middle eastern, East Indian ancestries
57
Sickle cell type that is the most common and occurs when copies of Hb S gene are inherited from both parents.
Hb SS sickle cell anemia AKA E6V
58
Sickle cell type that is the second most common and occurs when the Hb C gene is inherited from one parent and the Hb S from another
E6K AKA Hb SC sickle cell | Less severe
59
Sickle cell type that occurs when the Hb S gene is inherited from one parent and the Hb E from another.
Hb SE AKA E26K | Very severe
60
Sickle cell type that occurs when hemoglobin C is inherited from both parents.
Hb CC sickle cell AKA E6K
61
Sickle cell trait (SCT) is characterized by
Inheritance of Hb S and a normal gene Hb A- don’t have symptoms but can pass it on
62
Clinical manifestation of Sickle cell characterized by severe anemia. (Not present younger than 4-6months)
Due to hemolysis-Fatigue, tachycardia, jaundice, dyspnea, paleness
63
Clinical manifestation of sickle cell characterized by vasooclusion: (Not present younger than 4-6months)
Painful crisis & Tissue ischemia low back pain, joint pain Hand-foot syndrome/ SC dactylitis Chest/abdominal pain Bone infarcts
64
Sickle Lab findings
Sickle cell Target cell Increased sickle cell Hb, decreased normal Howell jolly bodies High reticulocyte count
65
Blood disorder that is autosomal recessive characterized by abnormal forms of hemoglobin resulting in weakening and destruction of RBC. Quantitative defect
Thalassemia
66
Two types of thalassemia based on part of O2 carrying protein RBC is lacking
Alpha- severe Beta- less severe
67
Both alpha and beta forms of thalassemia include the following form.
Major (from both parents) Minor ( one parent)
68
In which form of thalassemia are children normal at birth, then exhibit severe anemia. Also it is more prevalent in Mediterranean people known as Cooley’s anemia
Beta-thalassemia major
69
In which form of thalassemia can unborn babies die
Alpha-thalassemia major
70
The alpha and beta thalassemia minor are characterized by:
No Sx Microcytic RBC
71
Beta-thalassemia major is manifested by:
Anemia Iron overload Bone deformities Jaundice Splenomegaly Increased risk of infection
72
Bone deformities a/w thalassemia
Hair on end skull- accentuated trabeculae Honeycomb pattern- destruction due to multiple bone infarcts
73
Thalassemia lab findings:
Decreased Hb, RBC, MCV, MCH, MCHC Hypochromic microcytic, poikilocytosis, target cells, Cabot rings, ovalocytosis, Howell jolly bodies Increased bilirubin, iron, ferritin
74
Tx of thalassemia
Major forms-regular blood transfusion, folate supplements, bone marrow transplant, chelation therapy (reduce iron)
75
X-linked recessive genetic disorder that predisposes to spontaneous destruction of RBC and resultant jaundice in response to triggers like: Illness, food, medication
G6PDH deficiency AKA Favism
76
G6PDH function
Maintains pathway that produces gluthione, an antioxidant
77
G6PDH deficiency clinical manifestion
Paleness Jaundice Dark urine Fatigue Shortness of breath Tachycardia
78
G6PDH deficiency lab
Normocytic, normochromic Bite RBC (Degmacytes) Heinz bodies w/ inclusions WBC increase/platelet unaffected Increased indirect bilirubin, LDH Hematuria
79
Extrinsic hemolytic anemias etiological factors
``` Chemical poisoning Physical AI Paroxysmal nocturnal hemoglobinuria Infection Erythroblastosis fetalis ```
80
Erythroblastosis fetalis is described as
An occurrence when an Rh(-) mother gives birth to Rh(+) . Her body synthesis Ab against Rh(+), next Rh(+) is not conceived
81
State in which the proportion of blood volume that is occupied by red blood cells increases
Polycythemia
82
Two forms of polycythemia
Absolute- absolute #RBC increased Relative- decrease in volume of plasma w/ normal absolute RBC
83
What is the hallmark of polycythemia?
Elevated hematocrit w/ normal MCV
84
Polycythemia present when excessive amount of red blood cells is produced as a result of an abnormality of the bone marrow.
Absolute primary polycythemia AKA polycythemia Vera
85
Sx of polycythemia Vera
Itching/redness of skin Sudden severe burning pain in hands and feet Headache and vertigo Hepatoslpenomegaly ESR decreased
86
Polycythemia caused by either natural or artificial increases in the production of erythropoietin. Characterized only by increased production of RBC
Absolute secondary polycythemia/ Physiological polycythemia
87
Two conditions associated with absolute secondary polycythemia
1. Conditions that result in physiological appropriate polycythemia (adaptation) 2. Conditions that don’t result as a manifestation of physiological adaptation
88
Relative polycythemia results from
Loss of body fluid - burns, dehydration, stress.
89
Type of cancer of blood, bone marrow and lymphoid system characterized by an abnormal increase in immature or mature abnormal white blood cells
Leukemia
90
Leukemia is a broad base of diseases also know as?
Hematological neoplasms
91
Classification of leukemia characterized by a rapid increase in the number of immature blood cells due to bone marrow disorder
Acute (MC in young/middle aged people)
92
Classification of leukemia characterized by the excessive building up of RELATIVELY mature and still abnormal WBC
Chronic leukemia (MC in older people)
93
Leukemia classified based on Lymphocytes being affected
Lymphocytic / lymphoblastic leukemia’s
94
Leukemia classified based on RBC, some WBC and platelets being affected.
Myeloid/myelogenous leukemia
95
What are the general clinical manifestations of acute forms of leukemia
``` Generally non-specific Flu-like symptoms:fatigue, fever, malaise Anorexia Secondary infection Anemia/excessive bleeding Hepatosplenomegaly Lymphadenopathy ```
96
Acute lymphocytic leukemia described as?
Malignant immature lymphocytes growing w/in bone marrow. Affects young children Blood shows undifferential immature lymphocytes- (blueberries)
97
Acute myeloid leukemia is described as ?
Rapid growth of abnormal non-differentiated granulocytes, abnormal myeloblasts (WBC) accumulating in bone marrow causing a drop in RBC, Platelets and normal WBC amount. More often adults, avg age 67 year/ MC in males
98
Clinical manifestation of chronic leukemia’s
``` Insidious onset Non specific Sx Anemia Anorexia Bacterial, viral, fungal infection due to immunosuppression ```
99
Chronic lymphocytic leukemia is described as?
Abnormal growth of B cells in the bone marrow that most often affects middle-age adults or elder over the age of 55 and never children
100
Most people Dx with Chronic lymphocytic leukemia are diagnosed without sx as a result of what
As a result of routine blood test that returns high in WBC count
101
Form of leukemia classified by the increased and unregulated growth of predominantly myeloid cells (granulocytes) in bone marrow and the accumulation of these cells in the blood, lymphnodes and spleen
Chronic myeloid leukemia
102
Most people dx with CML have a genetic abnormality in their blood cells called Philadelphia (Ph) chromosome. Describe the Ph chromosome
The Ph chromosome cause the production of the enzyme tyrosine kinase protein that is always on causing the cell to divide uncontrollably
103
What is the affect of CML on cells?
Increased granulocytes of all types including mature myeloid cells. Basophils and eosinophils increased
104
Cancer of the lymph tissue that happens when B or T cells divide faster than normal and live longer than normal
Lymphoma
105
Two main types of lymphoma
Hodgkin lymphoma Non-Hodgkin lymphoma (MC)
106
Risk factors for lymphoma
- exposure to virus (e.g. hepatitis B, C, Epstein-Barr) - exposure to Helicobacter pylori - exposure to chemical (e.g. herbicides) and radiation - autoimmune diseases (e.g. Sjögren syndrome, SLE)
107
Clinical manifestation of lymphoma that is characterized by - painless enlargement of nodes - rubbery and swollen when examined - nodes of the cervical, supraclavicular, chest, groin, and across whole body
Lymphadenopathy
108
DDx between lymphomas: 1. One type 2. Begins w/ B cells 3. Age 15-35 &over 50 4. MC in Females 5. Lymphadenopathy begins in upper body and spreads to lower 6. Lymphnodes painful after alcohol consumption 7. In biopsy of lymphnodes: Reed-Steinberg cells found
Hodgkin lymphoma
109
Reed Sternberg cells are described as ? Found in hodgkins lymphoma
LArge often bi-uncleared cells with prominent nucleoli, originating from B-lymphocytes
110
Hereditary clotting factor disorders
Von willebrand disease Hemophilia
111
Acquired clotting factor disorders
Disseminated intravascular coagulation Circulating anticoagulants
112
Von Willebrand disease is described as?
The most common hereditary coagulation abnormality described in humans, developed through von willebrand factor deficiency
113
Von willebrand disease results in
Non formation of platelet plus and excessive bleeding
114
Von willebrand disease is a/w an autosomal dominant inheritance of which chromosome
12- always one parent with disease
115
Clinical manifestation of vWD
Bruising-nosebleed-gumbleeds Recurring GI bleeding Intensive menstruation hemorrhage (menorrhagia) Prolonged bleeding and unaffected platelet count
116
Hemophilia is described as?
Rare genetic disorder that prevents the blood from clotting properly due to lack of clotting factors 8, 9, & 11
117
Types of hemophilia and their associated factor
Hemophilia A- VIII (80% of cases) Hemophilia B/Christmas dz-lack of CF 9 Hemophilia C- deficiency of factor 11
118
T/F: Hemophila affects both genders equally
True
119
Hemophilia patients have parents that are affected/unaffected by bleeding disorder
Unaffected
120
Clinical manifestation of hemophilia
Usually discovered before 18 months old Bleeding larger in weight bearing joints—>hemarthrosis Bleeding tongue may close airway
121
Disseminated intravsacular coagulation is described as
Disorder in which CF become overreactive resulting is small clots in small vessels in the entire body
122
Explain whether or not DIC is a primary or secondary disorder.
DIC is a secondary disorder due to the fact it accompanies an underlying condition usually with critical illness
123
Clinical manifestation of DIC
Bleeding Drop in BP Dysfunction of all organs
124
Disorder where there is abnormally low platelets
Thrombocytopenia- regular platelet count should be between 150,000-450,000
125
Causes of thrombocytopenia
``` Nutrient deficiencies Hereditary syndromes Increased platelet destruction Drugs Various blood disorders ```
126
Clinical manifestations of thrombocytopenia
``` Low platelets Petechiae Purport Nosebleeds Bleeding gums ```
127
Rare disorder, causing extensive microscopic clots to form in the small blood vessels throughout the body
Thrombotic thrombocytopenic purpura
128
Five features of Thrombotic thrombocytopenic purpura
Thrombocytopenia Microangiopathic hemolytic anemia Neurologica Sx Kidney failure Fever
129
A disorder that usually occurs when an infection in the digestive system produces toxic substances that: destroy RBCs cause kidney injury form blood clots in small blood vessels
Hemolytic-uremic syndrome (one of the TTP)
130
What is the common causative factor of hemolytic uremic syndrome
Post GI infection with E.coli
131
Clinical manifestation of Hemolytic syndrome
MC cause of acquired acute renal failure in childhood GI problems Kidney failure Systemic problems