Pathophysiology: Hematology Flashcards
1
Q
Hemoglobinopathy
A
Disorder affecting the structure, function, or production of hemoglobin; inherited; range fr asymptomatic to deadly.
2
Q
Hemolysis / Hemolytic
A
Destruction of RBCs –> hemoglobin is liberated
3
Q
Coagulopathy
A
Bleeding disorder affecting clotting of the blood
4
Q
Polycythemia
A
Elevated hemoglobin
5
Q
Apparent Polycythemia
A
Plasma level is down so hemoglobin level seems elevated
6
Q
Aplasia
A
Incomplete, defective, or a stop in the usual regenerative process of blood
7
Q
Cytosis
A
More than the usual # of cells are circulating in the blood
8
Q
Cytopenia
A
Reduction in number of cells in the blood
9
Q
Anisocytosis
A
Variation in size and shape of RBCs
10
Q
Poikilocytosis
A
Presence of poikilocytes in the blood
11
Q
Poikilocytes
A
Abnormally shaped red cells
12
Q
Anemia
A
Hematocrit is less than 41% in males and less than 36% in women… i.e. hemoglobin is less than 13.5 g/dL in men or less than 12 g/dL in women
13
Q
Pathway to a platelet
A
pluripotential cell –> myeloid multipotential cell –> megakaryocyte colony forming cell –> Megakaryoblast –> Megakaryocyte –> Platelet
14
Q
Thrombocytopenia
A
Low Platelets
15
Q
Pathway to B or T Cell
A
Pluripotential Cell –> Lymphoid Multipotential Cell –> Lymphoid Colony Stimulating Factor –> Lymphoblast –> B and T lymphocytes
16
Q
To check for low folic acid, what lab should you draw?
A
Homocysteine
17
Q
To check for low B-12, what lab should you draw?
A
Methylmalonic Acid
18
Q
Mean Cell Volume for Macrocytic Anemia
A
100 and Above (Hemaglobin & Hematocrit will be low)
19
Q
MCV for Normocytic Anemia
A
MCV = 80-100 (Hemaglobin & Hematrocrit
20
Q
MCV for Microcytic Anemia
A
Under 80
21
Q
Anemias by Morphology
A
Macrocytic, Microcytic, Normocytic
22
Q
Microcytic Anemias
A
Iron Deficiency, Thalassemia (major/minor)
23
Q
Iron deficiency pathophysiology
A
Problem synthesizing hemoglobin b/c iron component is lacking
24
Q
Iron deficiency is _____________________
A
blood loss until proven otherwise
25
Etiology of iron deficiency (5):
1) blood loss (GIB, menstrual, blood donation), 2) decreased absorption (post gastrectoy, celiac disease, h.pylori), 3) increased demand (pregnancy), 4) dietary deficiency, 5) iron sequestration
26
Iron deficiency signs:
smooth tongue, brittle nails, koilonychias, cheilosis
27
Iron deficiency symptoms:
tachycardia, tachypnea on exertion, pica, fatigue, palpitations
28
Iron deficiency treatment:
Take oral iron (with Vitamin C)
29
Thalassemia
Defect in the globin part of the hemoglobin
30
Thalassemia Etiology / Types
Genetic / Alpha and Beta
31
Thalassemia Major / Minor
Major = homozygous, Minor = heterozygous
32
Thalassemia Treatment
NO IRON; don't over-treat, if necessary, blood transfusions
33
Alpha Thalassemia is common in:
SE Asia, China, ME, Africa; Asia can get Major, Africa will not
34
Beta Thalassemia is common in:
Mediterranean origin (lesser extent Asia/Africa)
35
Macrocytic Anemias:
Folic Acid Deficiency, B-12 Deficiency, Liver Disease, Myelodysplasia
36
Megaloblasts / Megaloblastic Anemias
Large, Immature Red Cells / Low B-12, Folic Acid
37
Pathophys of Megaloblastic Anemias
DNA synthesis is inhibited; cell grows, but does not go through division.
38
Folic Acid Deficiency Etiology
Malnutrition, alcoholism, pregnancy (increased demand)
39
B-12 Deficiency Etiology
Disruption in intestinal mucosa absorption: surgery, alcoholism, celiac disease
40
Pernicious Anemia
Body can't absorb enough B-12
41
Signs and Symptoms of B12/Folic Acid deficiency
Pallor, glossitis, diarrhea, paresthesia, memory impairment
42
Side Effects of All Anemias
Tachycardia, tachypnea, fatigue w/ exercise, palpitations
43
Myeloid Dysplastic Syndrome
Stem cell disorder involving myeloid lineage of blood cells; nuclear and cytoplasmic maturation in blood cells do not correspond
44
Myeloid Dysplastic Syndrome Etiology
Idiopathic or Toxic
45
Myeloid Dysplastic Blood looks like:
Tapioca pudding under a microscope
46
Treatment for Myeloid Dysplastic Syndrome:
Give EPO or blood transfusion
47
Thalassemia Complication
Iron overload!
48
Signs of Thalassemia in children
Facial bone changes
49
Round Macrocytes
Liver disease or Myeloid Dysplastic
50
Oval Macrocytes
Megaloblastic
51
Normocytic Anemia:
Anemia of Chronic Disease
52
Etiology of Anemia of Chronic Disease:
Chronic inflammation/conditions lead to reduced # of RBCs, i.e.: Autoimmune disease, cancer, chronic kidney disease, HB, HC, HIV / lack of EPO
53
Who often gets Anemia of Chronic disease?
Elderly / sick
54
What are the components of a CBC? (10)
RBC count, WBC count, Platelet Count, Hemoglobin, Hematrocrit, Reticulocyte Count, RDW, MPV, MCV, MCHC
55
RDW
RBC Distribution Width
56
Hemoglobin
Iron containing oxygen transport metalloprotien in RBCs
57
Hematrocrit
Volume RBCs / Plasma = %
58
MPV
Mean Platelet Volume
59
MCHC
Mean Corpuscular Hemoglobin Concentration - measure of the concentration of hemoglobin in a given volume of packed RBCs
60
Polythemia
Increased hemoglobin and hematocrit
61
Leukocytosis
High WBCs
62
Leucopenia
Low WBCs
63
Thrombocytosis
High Platelet Count
64
Thrombocytopenia
Low Platelet Count
65
Hemolytic Anemia
Abnormal breakdown of RBCs
66
Intrinsic Hemolytic Anemia / Examples (2)
Defects in component of RBC (i.e. membrane, enzyme, hemoglobin) / Ex: Sickle Cell, G6PD deficiency
67
Extrinsic Hemolytic Anemia
Immune or microangiopathic hemolytic anemia / agent like Clostridium or Plasmodium attacking
68
List the Hemolytic Anemias (7):
Sickle Cell (Hb SS disease), Hemoglobin S-C disease, Sickle Cell Trait, Hereditary Spherocytosis, G6PD Deficiency, Autoimmune Hemolytic Anemia, Cold Agglutinin Disease
69
Sickle Cell Anemia SS's genetic inheritance
Autosomal recessive
70
Etiology of Sickle Cell SS Anemia
Base substitution of valine for glutamine leads to unstable production of hemoglobin that leads to mis-shapen, "sickled" RBCs
71
Signs and symptoms of Sickle Cell SS
Onset in 1st year of life; jaundice, gallstones, splenomegaly, poor healing
72
Sickle Cell SS labs
Chronic low hematocrit, reticulocytes, elevated WBCs, thrombocytosis, high bilirubin
73
Sickle Cell SS treatment
EPO, folic acid, transfusions
74
Hemoglobin S-C disease etiology
Base substitution at same place as Sickle Cell SS but instead it is valine for glutamine
75
Hemoglobin S-C disease is __________ than Sickle Cell SS
Milder
76
In hemoglobin S-C, RBCs will look _______ in a smear
sickled
77
Consistency of sickled cells
sticky
78
Sickle Cell Trait Genetics
Heterozygous
79
Between Sickle Cell Trait, Sickle Cell SS, Hemoglobin S-C, which is asymptomatic?
Sickle Cell Trait
80
How does Sickle Cell Trait appear on a blood smear?
Totally normal - no sickling; no RBCs on smear.
81
Complications of Sickle Cell Trait:
Possible sudden cardiac death and rhabdomylosis during exercise, increase in thromboemolism, chronic kidney disease
82
Etiology of Hereditary Spherocytosis
Autoimmune Disorder of RBC membrane leading to chronic hemolytic anemia; decrease in surface to volume ration result in spherical shaped cell.
83
Hereditary Spherocytosis Genetics
Autosomal Dominant
84
Hereditary Spherocytosis lab
Always high reticulocytes, spherocytes always present, Microcytosis
85
Hereditary Spherocytosis Treatment
Splenectomy
86
G6PD Deficiency Genetics
x-linked recessive
87
How G6PD deficiency causes hemolytic anemia
B/c of lack of G6PD enzyme, exposure to oxidative drugs/food causes episodes of hemolytic anemia
88
G6PD Deficiency Complications
Kidney/Liver failure
89
Autoantibody Hemolytic Anemia etiology
IgG binds to the outside of RBCs and coats them, making them look like a foreigner. Body attacks RBC, leaving spirochete which gets stuck in the spleen; likes warmth.
90
Autoantibody Hemolytic Anemia S&S
Rapid onset anemia - LIFE THREATENING
91
Autoantibody Hemolytic Anemia Lab Results:
Reticulocytes, spirochetes
92
Autoantibody Hemolytic Anemia Management:
Splenectomy
93
Cold Agglutinin etiology
IgM antibodies attach to RBCs in areas of lower temp; when RBCs move to higher temp, IgM dissociates & signals Kupffer cells to sequester RBCs, causing hemolytic anemia
94
Cold Agglutinin S&S
Exposure to cold
95
Hemochromatosis Genetics
Autosomal Recessive; HFE gene mutation
96
Hemochromatosis
Decrease in hepcidin which regulates iron, causing increased iron absorption / over load
97
Hemochromatosis prevention
Patients should avoid foods rich in iron
98
Conditions of Bone Marrow Failure (3):
Aplastic anemia, pure red cell aplasia, myelodysplasia
99
Aplastic Anemia
Bone marrow is not producing mature blood cells from hematopoietic stem cells
100
Hemochromatosis genetics
Autosomal Recessive; mutation of HFE gene that expresses hepcidin
101
Consequences of Hemochromatosis
Iron builds up in kidneys, liver, lung, pancreas, heart, adrenals, pituitary
102
Treatment for Hemochromatosis
"Donate blood" frequently except you just throw away the excess pints
103
Treatment for Aplastic Anemia
EPO or myeloid growth factor
104
Pure Red Cell Aplastic Anemia
Rare autoimmune disorder mediated by T lymphocytes causing RBCs to be formed incorrectly.
105
Treatment for Pure Red Cell Aplastic Anemia
Immunosuppressive therapy
106
Myeloid Dysplasia
Morphologic abnormalities in 2 or more hematopoietic cell lines
107
Myeloid Dysplasia on labs
Left shift in CBC; particularly presence of dwarf megakaryocytes w/ unilobed nucleus; MCV elevated
