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Master the Boards: Hematology > Anemia > Flashcards

Anemia Flashcards

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1
Q

What is Anemia?

A

decreased RBC (hgb) in blood

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2
Q

What symptoms would be expected with a HCT>30%?

A

no symptoms

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3
Q

What symptoms would be expected with a HCT of 25-30%?

A

Dyspnea that worsens on exertion and fatigue

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4
Q

What symptoms would be expected with a HCT of 20-25%?

A

lightheadedness

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5
Q

What symptoms would be expected with a HCT of <20%?

A

Syncope and chest pain

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6
Q

What is the best initial test for Anemia?

A

CBC

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7
Q

What is the first clue to the etiology of Anemia?

A

Mean Corpuscular Volume (MCV)

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8
Q

What is Microcytic Anemia characterized by?

A

Low MCV (normal 80-100)

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9
Q

What are most causes of Microcytic Anemia related to?

A

Production issues

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10
Q

What are common causes of Microcytic anemia?

A

Iron Deficiency
Thalassesmia
Sideroblastic anemia
Anemia of Chronic disease

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11
Q

What is the main cause of Iron deficiency anemia?

A

Blood loss

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12
Q

What is the cause of Sideroblastic anemia?

A

Inability to incorporate Fe into Heme

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13
Q

What are common etiologies of Sideroblastic anemia?

A

Suppressive effect of alcohol on bone marrow
lead poisoning, isoniazid and B6 deficiency

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14
Q

What can cause anemic of chronic disease?

A

Any cancer or chronic infection

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15
Q

What is the underlying issue in anemia of chronic disease?

A

Iron is trapped in macrophages or in ferritin so Hgb synthesis cannot occur: Hepcidin is increased

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16
Q

What is the most common etiologies of Normocytic Anemia?

A

Acute blood loss of hemolysis: causes a large drop in HCT rapidly so MCV doesn’t have time to change

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17
Q

What is a very common cause of Normocytic Anemia?

A

Chronic Renal Failure

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18
Q

What are Packed Red Blood Cells (PRBC)?

A

Concentrated form of blood: unit of whole blood with about 150mL of plasma removed

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19
Q

How much should 1 unit of PRBC raise Hematocrit?

A

3 points or 1g/dL of Hb

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20
Q

What is Fresh Frozen Plasma (FFP)?

A

replaces clotting factors in patients with elevated PT, aPTT, or INR + Bleeding

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21
Q

What is Cryoprecipitate?

A

Used to replace fibrinogen: provides high levels of clotting factors (VIII, VWF)

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22
Q

When do you give Platelets?

A

if Platelet count <50,000

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23
Q

What condition is a platelet infusion contraindicated in?

A

TTP

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24
Q

What is in Prothrombin Complex Concentrate (PCC)?

A

All the Vitamin K dependent factors used to reverse warfarin toxicity

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25
What is the most likely diagnosis for anemia with blood loss (GI bleed)?
Iron deficiency
26
What is the most likely diagnosis for anemia with Menstruation?
Iron deficiency
27
What is the most likely diagnosis for anemia with cancer or chronic infection?
Anemia of Chronic Disease
28
What is the most likely diagnosis for anemia with Rheumatoid arthritis?
Anemia of Chronic Disease
29
What is the most likely diagnosis for anemia with Alcoholism?
Sideroblastic anemia
30
What is the most likely diagnosis for anemia with Asymptomatic presentation?
Thalassemia
31
After a CBC, what is the best initial test for anemia?
Iron studies
32
What is a low Ferritin level specific for?
Iron deficiency anemia
33
What happens to the total iron-binding capacity (TIBC) in iron deficiency anemia?
Increases due to unbound sites
34
What is high iron suggestive of in a microcytic anemia?
Siderblastic anemia: only microcytic anemia with elevated circulating iron levels
35
What does normal iron in anemia suggest?
Thalassemia
36
What does low iron in anemia suggest?
Chronic Disease: iron trapped in storage "ferritin"
37
What happens to Ferritin levels in anemia of chronic disease?
they increase
38
What happens to TIBC in anemia of chronic disease?
it decreases
39
What is a unique lab finding for Iron deficiency anemia?
RBC distribution of width (RDW) is increased
40
Why is RDW increased in iron deficiency anemia?
Newer cells are more iron deficient and smaller
41
What is the single most accurate test for iron deficiency anemia?
Bone marrow biopsy for stainable iron which is decreased
42
What is the most accurate test for Thalassemia?
Hemoglobin Electrophoresis: except for Alpha Thalassemia
43
What is the only form of Thalassemia with a different most accurate test?
Alpha thalassemia
44
What is the most accurate test for alpha thalassemia?
Genetic studies
45
What is the most accurate test for Sideroblastic anemia?
Prussian blue staining for ringed sideroblasts -can see basophilic stippling in any form of siderblastic anemia
46
Thalassemia electrophoresis findings with 1 alpha gene deletion?
Normal
47
Thalassemia electrophoresis findings with 2 alpha genes deleted?
Mild anemia with normal electrophoresis
48
Thalassemia electrophoresis findings with 3 alpha genes deleted?
Moderate anemia with Hemoglobin H (beta-4 tetrads) and increased reticulocytes
49
Thalassemia electrophoresis findings with 4 alpha genes deleted?
Hemoglobn Bart: gamm-4 tetrads CHF causes death in utero
50
What is Beta Thalassemia Minor?
1 beta gene deletion
51
What electrophoresis findings are seen in Beta Thalassemia minor?
HbA slightly decreased HBA2 slightly increased HBF slightly increased
52
What is Beta Thalassemia major?
2 beta genes defective
53
What electrophoresis findings are present in Beta Thalassemia major?
Little to no HbA Increased HbA2 and HbF
54
How do you treat Iron Deficiency?
Replace with Oral Ferrous Sulfate -occasionally requires IV Iron
55
How is Thalassemia trait treated?
No treatment needed for Thalassemia trait
56
How is beta thalassemia major (cooley anemia) Treated?
Lifelong transfusion therapy
57
How do you manage iron overload from chronic transfusion therapy?
Deferasirox or Deferiprone: PO iron chelators Deferoxamine: parentaeral Iron Chelator
58
What is Luspatercept?
Tissue growth factor that decreases transfusion dependence in beta thalassemia and myelodysplasia
59
What is the only type of anemia that responds to Erythropoietin replacement?
Anemia of chronic disease from end-stage renal failure
60
How do you treat Sideroblastic Anemia?
Correct the underlying cause B6, Pyridoxine replacement
61
What TB drug commonly causes Sideroblastic Anemia?
Isoniazid
62
What is Macrocytic Anemia?
High MCV: RBC are larger than normal
63
What are the 2 most common causes of Macrocytic anemia?
B12 and folate deficiency
64
What are less common causes of Macrocytic anemia?
Sideroblastic anemia Alcoholism Antimetabolit meds: Azathioprine, 6-MP, Hydroxyurea Liver disease or hypothyroidism Zidovudine or Phenytoin Myelodysplastic Syndrome Cold Agglutinins: falsely elevate MCV by clumping cells
65
What are common causes of B12 Deficiency?
Pancreatic Insufficiency, Pernicious anemia, Vega, Crohn Disease, Celiac Disease, Tropical Sprue, Radiation/damage to terminal ileum blind loop syndrome (gastric bypass) Diphyllobothrium Latum PPI, Metformin
66
What are common causes of Folate Deficiency?
Dietary Deficiency, Psoriasis and skin loss/turnover, Phenytoin, Sulfa drugs, Methotrexate
67
What nutrient deficiencies are seen in Celiac Disease?
B12, Folate and Iron Deficiency
68
What happens to the reticulocyte count in macrocytic anemia?
Decreases
69
What is the first step after CBC shows a macrocytic anemia?
Peripheral Blood smear to look for Hypersegmented Neutrophils -then get B12 and Folate levels
70
What is the most common neurological abnormality caused by B12 deficiency?
Peripheral Neuropathy
71
What cancer does pernicious anemia increase the risk of?
Gastric Cancer
72
What is the classical neurological presentation of B12 Deficiency?
Posterior Column Damage to position and vibratory sensation "subacute combined degeneration"
73
What deficiency is associated with an increased Methylmalonic Acid (MMA) level?
B12 deficiency
74
What lab abnormalities are seen with both B12 and Folate Deficiency?
Megaloblastic Anemia Elevated LDH Elevated Indirect Bilirubin Decreased reticulocyte count Hypercellular Bone Marrow Macroovalocytes Increased Homocysteine levels
75
How do you confirm a diagnosis of Pernicious Anemia?
Anti-intrinsic factor and anti-parietal cell antibodies
76
Why is the reticulocyte count low in macrocytic anemia?
RBCs are destroyed as they leave the marrow
77
What enzymes are needed to absorb B12?
Pancreatic enzymes: they free B12 from carrier proteins
78
What other blood cell abnormalities can B12 and Folate deficiency result in?
Pancytopenia
79
How do you treat Macrocytic anemia?
replace what is deficient -Folate will fix anemia but not Neurological symptoms if B12 is deficient
80
What is a potential complication of B12 or Folate replacement?
Hypokalemia from rapid cell production in bone marrow -Cells are generated so rapidly they use up all the potassium
81
What Can all forms of Hemolytic anemia produce?
Sudden decrease in Hct Increased LDL, Indirect Bilirubin and Reticulocytes Decreased haptoglobin Slight increase in MCV: reticulocytes are larger than normal cells Hyperkalemia: Cell breakdown Folate deficiency: increased production uses it up
82
What is Sickle Cell Disease?
Chronic, well-compensated hemolytic anemia with a high reticulocyte count
83
What patient demographic is Sickle Cell Disease associated with?
African American Patients
84
What causes Sickle Cell Disease?
Point mutation at Position 6 of the beta globin chin: Valine replaces glutamic acid
85
What are common symptoms of Sickle cell?
Bilirubin gallstones; elevated bilirubin Increased infection: autosplenectomy Osteomyelitis: from salmonella Retinopathy Stroke Lower extremity skin ulcers Avascular necrosis of the femoral head
86
What is the best initial test for avascular necrosis of the femoral head?
X-ray
87
What is the most accurate test for avascular necrosis of the femoral head?
MRI
88
How does acute, painful vaso-occlusive crisis present in Sickle cell?
Sudden, severe pain in the chest, back, and thighs with possible fever
89
What are common causes of Acute crisis in Sickle Cell?
Hypoxia Dehydration Infection/fever Cold Temperatures
90
How do children with Sickle Cell commonly present?
Dactylitis: inflammation of fingers
91
What arises from chronic kidney damage in Sickle Cell?
Papillary Necrosis
92
What is the best initial test for Sickle Cell?
Peripheral Blood Smear
93
What does Sickle Cell Trait (AS) show on blood smear?
No sickled Cells
94
What is the most accurate test for Sickle Cell?
Hemoglobin Electrophoresis
95
What is found on Peripheral Blood Smear in Sickle Cell?
Howell-Jolly bodies: precipitated remnants of nuclear material inside the RBC's
96
How do you begin treatment of acute crisis in Sickle Cell?
Oxygen + Hydration + Analgesia
97
What do you give Sickle Cell patients in acute crisis with fever or high WBC?
Antibiotics: Ceftriaxone, Levofloxacin, Moxifloxacin
98
What Abx are given to Sickle cell patients in acute crisis with fever or high WBC?
Ceftriaxone Levofloxacin Moxifloxacin
99
How do you manage chronic Sickle Cell Disease?
Replace folic acid prn Pneumococcal vaccine: for autosplenectomy Hydroxyurea: prevent recurrence
100
What are the 2 treatment strategies that lower mortality in sickle cell disease?
Hydroxyurea in prevention and Antiobiotics with fever
101
What drugs can be given in sickle cell disease to increase oxygen-carrying capacity?
Voxelotor and Crizanlizumab IgG
102
How does Voxelotor work?
Increases oxygen carrying capacity by decreasing hemoglobin S polymerization
103
How does crizanlizumab IgG work?
decreases platelet aggregation and promotes oxygen-carrying capacity by binding to P-Selectin
104
When is exchange transfusion used in sickle cell?
Severe vaso-occlusive crisis
105
How does severe vaso-occlusive crisis present?
Acute chest syndrome Priapism Stroke Visual disturbance from retinal infarction
106
What infection can trigger aplastic crisis in sickle cell?
Parvovirus
107
What is the most accurate test for Parvovirus B19?
PCR for DNA
108
What is the best initial treatment for Parvovirus B19?
IVIG
109
What is sickle cell trait?
Patient is heterozygous for sickle gene (AS)
110
What is the only manifestation of sickle cell trait?
defective ability to concentrate urine "isothenuria"
111
How do you treat sickle cell trait?
you don't
112
What is Hereditary Spherocytosis?
Defect in the cytoskeleton of RBC leading to abnormal round shape and loss of normal flexibility
113
What are the common symptoms of Hereditary Spherocytosis?
Recurrent Hemolysis, intermittent jaundice Splenomegaly, Family hx of anemia or hemolysis Bilirubin Gallstones
114
What is the most accurate test for Hereditary Spherocytosis?
Eosin-5-Maleimide flow cytometry
115
What other diagnostic test features are seen in Hereditary Spherocytosis?
Low MCV Increased Mean Corpuscular Hemoglobin Concentration (MCHC) Negative Coombs Test
116
How do you treat Hereditary Spherocytosis?
Chronic Folic acid replacement: aid RBC production Splenectomy: stops hemolysis but doesn't eliminate spherocytes
117
What is another name for Autoimmune Hemolysis?
Warm or IgG Hemolysis
118
What are commonly known causes of Warm Autoimmune Hemolysis?
Chronic Lymphocytic Leukemia (CLL) Lymphoma Systemic Lupus Erythematosus (SLE) Penicillin, Alpha-methyldopa, Rifampin, Phenytoin
119
What cell shape is associated with autoimmune Hemolysis?
Microspherocytes
120
What is the mechanism autoimmune hemolysis?
Autoab remove small amounts of the RBC membrane leading to a smaller membrane which forces the cell to become round
121
What is the most accurate test for warm autoimmune hemolysis?
Coombs Test: detects IgG Ab on the surface of RBC
122
Where is the site of hemolysis in Warm Autoimmune hemolysis?
In the spleen or liver: not in the vessels
123
What are cold Agglutinin?
IgM Ab against RBC developing in association with EBV, Waldnstrom macroglobulinemia or Mycoplasma Pneumoniae
124
How do you treat Warm Autoimmune Hemolysis?
Glucocorticoids -cyclophosphamide, cyclosporine, azathioprine, myocphenolate mofetil Splenectomy for recurrent episodes
125
What is the best initial treatment for Warm Autoimmune Hemolysis?
Prednisone
126
Where do symptoms occur in Cold Agglutinin Disease?
Colder parts of the body: nose, ears, fingers, toes -resolve with warming
127
What is the most accurate test for Cold Agglutinin Disease?
Cold Agglutinin Titer
128
What is shown on a direct coombs test in Cold Agglutinin Disease?
Complement
129
How do you treat Cold Agllutinin Disease?
Keep warm Rituximab or Sutimlimab: inhbit C1s and stop IgM RBC destruction Plasmapheresis Cyclphosphamide, Cyclosporine etc to stop Ab production
130
What is Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency?
XLR disorder leading to inability to generate glutathione reductase and protect the RBC from oxidant stress
131
What is the most common Oxidant stress in G6PD deficiency?
Infection
132
What are common causes of Oxidant stress in G6PD deficiency?
Infection Dapsone, Quinidine, Primaquine, Nitrofurantoin Fave beans
133
How does G6PD deficiency commonly present?
Sudden anemia and Jaundice in an African American or Mediterranean man with infection or using one of the commonly associated drugs
134
What is the best initial test for G6PD deficiency?
Heinz Bodies and Bite Cells
135
What is the most accurate test for G6PD deficiency?
G6PD level: 2-3 months after an acute episode of hemolysis -G6PD will be normal right after hemolytic event
136
What is seen on peripheral Smear in G6PD deficiency?
Heinz bodies and bite cells
137
What stain is used to see Heinz bodies on peripheral Smear?
Methylene Blue or Crystal Violet
138
How do you manage G6PD Deficiency?
No acute treatment Avoid oxidant stress Caution with Rasburicase which provokes hemolysis in G6PD
139
What are both Thrombotic Thrombocytopenic Purpura (TTP) and Hemolytic Uremic Syndrome (HUS) associated with?
Intravascular hemolysis Renal Insufficiency Thrombocytopenia <30,000
140
What are common etiologies of TTP?
HIV, Cancer, Cyclosporine and Clopidogrel
141
What are common etiologies of HUS?
Children, E. Coli O157:H7 and Shigella
142
What additional clinical features are associated with TTP?
Neurological symptoms Fever
143
What cell types on peripheral blood smear are suggestive of Intravascular hemolysis?
Schistocytes Helmet Cells Fragmented RBC
144
What happens to PT and aPTT levels in TTP and HUS?
They are normal
145
A low level of what supports the diagnosis of TTP?
ADAMTS13 level
146
What is the only indispensable finding to establish the diagnosis of TTP?
Intravascular hemolysis
147
How do you treat TTP and HUS?
Urgent Plasmapheresis for TTP and severe HUS
148
What do you use for TTP and Severe HUS if Plasmaphoresis is not available?
Fresh Frozen Plasma (FFP)
149
How do you treat severe, atypical HUS?
Eculizumab
150
What is Atypical HUS?
Occurs when Complement erroneously attacks RBC, not as a result of infection
151
What is the mechanism of Eculizumab in HUS?
Blocks Complement C5a
152
What is needed before using Eculizumab?
Vaccination against Meningococcus
153
What additional treatments aid in TTP?
Steroids Caplacizumab Rituximab and Ocrelizumab
154
Why are steroids helpful in TTP and not HUS?
TTP has antibodies against ADAMTS13
155
What is the Mechanism of Caplacizumab in TTP treatment?
Ab against VWF: acts like ADAMTS13
156
What is the mechanism of Rituximab and Ocrelizumab in TTP treatment?
Anti-CD20 drug: stop the production of Ab to ADAMTS13
157
What is Paroxysmal Nocturnal Hemoglobinuria (PNH)?
Clonal stem cell defect with increased sensitivity of RBCs to complement in acidosis
158
What is the Mechanism of PNH?
Deficiency of C' regulatory proteins CD55 and CD59 (Decay-accelerating factor) lead to C' overactivation
159
What gene is defective in PNH?
Phosphatidylinositol Class A (PIG-A): leads to hemolysis and thrombosis in PNH
160
What is the most common form of death in PNH?
Hemolysis and Thrombosis
161
What are common symptoms of PNH?
Episodic dark urine Pancytopenia and iron deficiency Clots in unusual places
162
What is the most accurate test for PNH?
Decreased levels of CD55 and CD59
163
What does a CBC commonly show in PNH?
Pancytopenia and Anemia
164
What is the best initial therapy for PNH?
Prednisone
165
What are the other treatment options for PNH?
Eculizumab Folic acid replacement and transfusions as needed Allogenic bone marrow transplant
166
What is the only curative treatment in PNH?
Allogenic Bone Marrow Transplant
167
What is the mechanism of Eculizumab in PNH?
Inactivates C5 and Decreases RBC destruction -Complement inhibitor: treats hemolysis and thrombosis
168
What vaccine must be given prior to use of Eculizumab?
Meningococcal vaccine
169
What is Aplastic Anemia?
Pancytopenia of unclear etiology
170
What is pancytopenia?
Low levels of all 3 blood cell types; RBC, WBC and PLT
171
What is the cause of Aplastic Anemia?
Autoimmune disorder where T-Cells attack the patient's own bone marrow
172
What are common etiologies associated with aplastic anemia?
Cancer Radiation Toxins: Toluene, Insecticide (DDT), Benzene Sulfa drugs, phenytoin, carbamazepine, chloramphenicil, alcohol Chemotherapy SLE, PNH HIV, Hepatitis, CMV, EBV B12 and Folate Deficiency PTU and Methimazole
173
What are common symptoms of Aplastic Anemia?
Fatigue, infections and bleeding
174
What is the most accurate test for Aplastic Anemia?
Bone marrow biopsy
175
How do you start treatment of Aplastic Anemia?
Manage the underlying cause with supportive therapy -Abx for infection -transfusions for anemia -platelets for bleeding
176
What is a consideration for treatment in a young patient with true aplastic anemia?
Allogenic Bone Marrow Transplant
177
How do you treat aplastic anemia if Bone marrow transplant isn't an option?
Antithymocyte Globulin Cyclosporine or Tacrolimus Alemtuzumab
178
What is the Mechanism of Alemtuzumab in Aplastic Anemia treatment?
Anti-CD52 agent that suppresses T Cells
179
Why do T-Cell inhibitors treat Aplastic Anemia?
They bring the marrow back to life
180
What drugs can you use to increase the marrow response when treating Aplastic Anemia?
Trhombopoietins: Eltrombopag or Avatrombopag

Master the Boards: Hematology (7 decks)

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