Anemia Flashcards
(142 cards)
1
Q
Decreased ability of blood to carry oxygen
A
Anemia
2
Q
Normal range of MCV
A
80-100 fL
3
Q
Normal value of MCH
A
28-34 pg
4
Q
Normal range of MCHC
A
32-36 g/dL
5
Q
Adequate/excess iron that is not able to be effectively incorporated into heme
A
Sideroblastic anemia
6
Q
Adequate iron stores that have impaired release for incorporation into heme/RBCs
A
Anemia of chronic disease/inflammation
7
Q
Iron disorder that in NOT anemia, with excess iron absorption
A
Hemochromatosis
8
Q
Microcytic hypochromic
Decreased serum iron
Decreased ferritin
Increased TIBC
A
IDA
9
Q
Acquired sideroblastic anemia as seen in myelodysplastic syndromes
A
Refractory anemia
10
Q
Microcytic hypochromic
Increased serum iron
Increased ferritin
Decreased TIBC
A
Sideroblastic anemia
11
Q
Acquired anemia characterized by abundant iron stores, yet iron cannot be readily incorporated into serum or RBCs for use
A
Anemia of chronic inflammation
12
Q
_______ decreases iron release from macrophages and hepatocytes
A
Hepcidin
13
Q
_______ competes with transferrin for plasma iron
A
Lactoferrin
14
Q
Normal MCV and MCH
Increased serum iron
Increased ferritin
Decreased TIBC
A
Anemia of chronic inflammation
15
Q
Iron problem that does not involve anemia
A
Hemochromatosis
16
Q
Iron is stored as _____ and ______
A
Ferritin and hemosiderin
17
Q
Gene associated with hepcidin mutations
A
HAMP gene
18
Q
Gene associated with Hemojuvelin mutations
A
HJV gene
19
Q
- Anemia that results from defective DNA synthesis
- vitamin B12 and folic acid deficiencies
A
Megaloblastic anemia
20
Q
Lack of iron to make adequate heme
A
IDA
20
Q
Classic 2 part test used to determine if the cause of vitamin B12 deficiency is malabsorption, dietary deficiency, or a lack of intrinsic factor
A
Schilling test
21
Q
Vit B12 deficiency parasite
A
D. Latum
22
Q
Macrocytosis without megaloblastic changes
A
Non malignant macrocytic anemia
23
Q
Disorders occuring as a result of decreased or absent production of hematopoietic cells in the bone marrow
A
Hyperproliferative disorders
24
Rare disorders characterized by pancytopenia in the peripheral circulation. Results from decreased bone marrow production
Aplastic anemia
25
- chromosomes are susceptible to breakage and cell may not be able to repair DNA damage
- cells also show accelerated telomere shortening and apoptosis
- FANCA mutations
Fanconi's anemia
26
Very rare disorder in which chromosomes have short telomeres
Dyskeratosis congenita
27
Autosomal recessive disorder leading to neutropenia and/or anemia and thrombocytopenia
Shwachmann-Diamond syndrome
28
Bone marrow exhibits decreased production of RBCs and RBC precursors, whereas other cell lines are present and produced normally
Pure red cell aplasia
29
Fanconi's anemia
Dyskeratosis congenita
Shwachmann-Diamond syndrome
Inherited aplastic anemia
30
Diamond-blackfan anemia
Congenital dyserythropoietic anemia
Anemia of chronic kidney disease
Congenital pure red cell aplasia
31
Group of inherited rare disorders leading to ineffective erythropoiesis
CDA
32
Individuals are unable to produce erythropoietin, leading to decreased production of RBCs
Anemia of chronic kidney disease
33
Disorders resulting from genetic mutations that lead to structural changes in the Hgb molecule
Hemoglobinopathies
34
Most hemoglobinopathies occur because of amino acid substitutions in the _______ chains
Beta globin
40
Abnormal hgb which usually affects african patients
Hgb S
41
Sickle cells
Drepanocytes
42
Homozygous for Hgb S
Sickle cell disease
| Major hgb present: S
43
Sickle screen test
Dithionite solubility
44
Heterozygous for Hgb S
Sickle cell trait
| Major hgb present: A
45
Hemoglobinopathy which usually affects patients of west africa
Hgb C
46
Hgb S mutation
Beta 6 (glu-val)
47
Hgb C mutation
Beta 6 (glu-lys)
48
Combination disorders
Hgb SC disease
Hbg S-beta thalassemia
Hgb C-harlem
49
Double mutation beta 6 (glu-val) and beta 73 (asn-asp)
Hgb C-harlem
50
Hemoglobinopathy which usually affects SE asians particularly thailand
Hgb E
51
Hgb E mutation
Beta 26 (glu-val)
52
Combination disorder with inheritance of mutations for both Hgb S and Hgb C
Most common of the compound disorders
Hgb SC
53
Disorders caused by genetic mutations that lead to quantitative changes in the amount of globin chains produced, resulting in an imbalance of globin chain synthesis
Thalassemia
53
Thalassemia caused by mutations or deletions in the alpha globin chains
Alpha thalassemia
54
Four gene deletion
Hgb present: bart, portland, H
Hydrops fetalis/hgb bart
55
Crystals look like bars of gold or the washington monument
Hgb C
56
4 main groups of alpha thalassemia
Hydrops fetalis/hgb bart
Hgb H disease
Alpha thalassemia minor
Silent carrier
57
Alpha globin is coded on _____
| Beta globin is coded on _____
Chromosome 16
| Chromosome 11
57
Three gene deletion
| Hgb present: A2, bart
Hgb H disease
58
Two gene deletion
Alpha thalassemia minor
58
One gene deletion
Silent carrier
58
Beta thalasseima (4 main groups)
Alpha thalassemia major
Beta thalassemia intermedia
Beta thalassemia minor
Silent carrier
58
Alpha thalassemia major is also known as _______
Cooley's anemia
59
Severe hemolytic anemia, diagnosed when hgb F levels normally decrease (patient cannot make hgb A)
Alpha thalassemia major
60
Deletion in the beta globin chain leading to increased production of hgb F
Hereditary persistence of fetal hemoglobin (HPFH)
61
Beta-**** fusion gene
Hgb lepore
62
Disorders of premature RBC destruction, leading to anemia
Hemolytic anemia
63
- incidence is 1/3000 of northern european ancestry
| - mutations affect genes coding for membrane proteins
Hereditary spherocytosis
64
Mutations in genes coding for spectrin or band 4.1, leading to disruption of the cell shape in circulation
Incidence is 1/2000-4000 (africa and mediterranean)
Hereditary elliptocytosis
65
Spur cell anemia
Acanthocytosis
66
Rare inherited disorders with neurologic symptoms and acanthocytosis
Neuroacanthocytosis
67
Abetalipoproteinemia, McLeod's syndrome
Neuroacanthocytosis
68
Rare acquired disorder resulting from stem cell mutation in the PIGA gene
PNH
69
In PNH, cells lack glycosylphosphatidylinositol-anchored proteins, including ____ and _____
CD55; CD59
70
- Most common RBC enzyme deficiency
- RBCs are unable to reduce glutathione, which is needed to battle oxidant damage in the cell, leading to oxidation of hgb into heinz bodies, which are then removed from circulation
G6PD deficiency
71
Leads to ATP depletion and increase in 2,3 BPG
Pyruvate kinase deficiency
72
Group of disorders characterized by intravascular fragmentation of RBCs as they move through blood vessels obstructed by microclots or endothelial damage
Microangiopathic hemolytic anemia
73
Activation of all parts of the hemostatic systems leading to the production of fibrin clots, the consumption of platelets and coagulation proteins, and degradation of fibrin. Clotting and bleeding both occur
Disseminated intravascular coagulation
74
Patients have long VWF multimers that bind vascular endothelium and platelets, triggering platelet aggregation. Platelets are used up in this process and microclots block small blood vessels, which leads to sharing of the RBCs in circulation
Thrombotic thrombocytopenic purpura
75
Gene mutation in TTP
ADAMTS 13
76
Microangiopathic hemolytic anemia with thrombocytopenia and renal involvement as a result of clots forming in the microvasculature of the kidney
Hemolytic Uremic Syndrome (HUS)
77
Acquired disorder, usually found among children with a history of hemorrhagic E. Coli and S. Dysenteriae infections
HUS
78
Relatively uncommon complication of pregnancy; more likely to affect patients with pre-eclampsia toward the end of their pregnancy
HELLP syndrome
79
Vascular insufficiency in the placenta can lead to dysfunction in the maternal endothelium, causing platelet activation and fibrin disposition in the small vessels
HELLP syndrome
80
Severe increase in blood pressure, leading to acute organ damage
Endothelial cells are damaged, leading to activation of the hemostatic system
Hypersensitive crisis and malignant hypertension
81
Prosthetic heart valves
| Mild hemolysis resulting from RBCs flowing around the implanted valves
Mechanical damage
82
Condition occasionally seen in long distance runners or others who engage in intense exercise
Exercise induced
March hemoglobinuria
83
Infectious agents which can cause mechanical destruction of circulating RBCs
Malaria (plasmodium)
Babesia (B. microti)
Bacteria (C. perfringens)
84
Causes of mechanical hemolysis
```
MHA
DIC
TTP
HUS
HELLP Syndrome
Hypertensive crisis and malignant hypertension
Mechanical damage
March hemoglobinuria
Infectious agents
Drugs, chemicals, venoms, thermal injury
```
85
RBC lifespan is shortened because of presence of antibodies usually IgG or IgM
Immune-mediated hemolytic anemia
86
Caused by autoantibodies that attach to the RBC surface
Autoimmune hemolytic anemia
87
Most common autoimmune anemia
| Secondary particularly in B-cell lymphoid malignancies such as CLL
Warm AIHA
88
Usually caused by IgM antibodies that react best at 4C
Cold agglutinin disease
89
Acute CAD can be secondary to ______
Mycoplasma pneumoniae
90
Acute cold AIHA associated with the Donath Landsteiner antibody (anti-P autoantibody)
PCH
91
Characterized by a sudden onset of anemia with hallmarks of hemolytic anemia after a patient is exposed to a medication
Drug induced hemolytic anemia
92
Hemolytic anemia resulting from immune incompatibility of donor and recipient (or immune incompatibility of mom and baby)
Alloimmune hemolytic anemia
93
Transfusion reactions
AHR
DHTR
HDFN
94
Occurs within hours of transfusion of incompatible blood products
AHR
95
AHRs are most commonly caused by _____
ABO incompatibility
96
Reaction may occur days to weeks after initial transfusion
DHTR
97
Occurs when maternal IgG antibodies cross the placenta and enter fetal, circulation
HDFN
98
Increase in neutrophils
| Bacterial infection, inflammation
Neutrophilia
99
Decrease in neutrophils
Congenital (rare)
Acquired (common)
Neutropenia
100
Increased eosinophils
| Parasitic infections and allergies
Eosinophilia
101
Increased basophils
| Hypersensitivity reactions
Basophilia
102
Increased monocytes
| Pxs recovering from infection
Monocytosis
103
Increased lymphocytes
Lymphocytosis
104
Decreased lymphocytes
Lymphocytopenia
105
Pale bluish inclusions in the cytoplasm of neutrophils
| Bacterial infections and inflammation
Dohle bodies
106
Large bluish black granules in the cytoplasm of neutrophils
| Inflammation
Toxic granulation
107
Vacuoles within the cytoplasm that are often indicative of phagocytosis
Bacterial or fungal infection
Vacuolization
108
Occurs when neutrophils have more than 5 segments
Hypersegmentation
109
Autosomal dominant mutation of the lamin B receptor
Pelger huet anomaly
110
Autosomal dominant mutation of the MYH9 gene
| With dohle-like inclusions
May hegglin anomaly
111
Autosomal recessive disorder leading to the inability to fully degrade mucopolysaccharides
Alder reilly anomaly
112
Autosomal recessive mutation of the LYST gene
Chediak higashi syndrome
113
Mutations in the genes needed to form cell adhesion molecules, particularly beta integrins
Leukocyte adhesion disorder
114
Clonal hematopoietic stem cell disorders that result in the overproduction and accumulation of cells in the granulocytic, RBC, and platelet cell lines, leading to chronic neoplasms
Myeloproliferative neoplasms
115
Characterized by production and accumulation of neutrophils in all stages of maturation
Chronic myelogenous leukemia
116
Terminal phase of CML
Blast crisis
117
Characterized by increased RBCs, granulocytes, and platelets in the peripheral blood, with notable increases in RBC and hgb, while erythropoietin levels remain normal to decreased
Patients with JAK2 V617F mutations
Polycythemia vera
118
Characterized by increased platelets and megakaryopoiesis; however, platelets may not function normally
Essential thrombocytopenia
119
Characterized by bone marrow fibrosis, extramedullary hematopoiesis, and increases in megakaryocytes
Chronic idiopathic myelofibrosis
Myelofibrosis with myeloid metaplasia
Primary myelofibrosis
120
Elevated WBC count with a proportional increase in neutrophils and their precursors in the bone marrow
Chronic neutrophilic leukemia
121
Elevated absolute eosinophil count with no evidence of reactive eosinophilia
Chronic eosinophilic leukemia
122
Group of chronic disorders with accumulations of mast cells within the organ systems
Mastocytosis
123
Group of neoplastic disorders characterized by peripherak blood cytopenias and dyspoiesis that occur in one or more cell lines
Myelodysplastic syndromes
124
Peripheral blood and bone marrow findings in myelodysplastic syndromes
```
RCUD
RCMD
RARS
RAEB-1
RAEB-2
5q-syndrome
```
125
Elevated WBC count with monocytosis and less than 20% blasts and promonocytes in the peripheral blood and bone marrow
CMML
126
Similar to CMML but occurs in children up to age 14
JMML
127
Blood picture is similar to that of CML but cells exhibit dysplasia
ACML
128
Acute leukemia characterized by the presence of mote than 20% lymphoid blasts in the peripheral blood and/or bone marrow
Acute lymphoblastic leukemia
129
Characterized by the presence of greater than 20% blasts in the peripheral blood and/or bone marrow
Acute myeloid leukemia
130
Leukemia patients who have a history of MDS or other similar disorders
Acute myeloid leukemia with myelodysplasia-related changes
131
Myeloid neoplasms associated with treatment with chemo therapy and/or radiation
Therapy-related myeloid neoplasms
132
Mature B cell disorder with an indolent course
Chronic lymphocytic leukemia
| Small lymphocytic lymphoma
133
Rare B cell disorder that affects both T cell and B cell lines
Similar to CLL but with prolymphocytes
Prolymphocytic leukemia
134
Chronic B cell neoplasm with lymphocytes showing threadlike or "hairy" projections
Hairy cell leukemia
135
B cell disorder leading to increase in plasma cells
Plasma cell neoplasms
136
Distinct punched out nucleoli
B cell prolymphocytic leukemia
137
Starry sky pattern
Burkitt lymphoma
138
Localized tumor of the lymph nodes
Hodgkin lymphoma
139
Manifests with the presence of Reed sternberg cells
Hodgkin lymphoma
140
Owl's eye or popcorn
Reed sternberg cells
141
Example of a T cell lymphoma
Sezary syndrome
