Hematology Flashcards
1
Q
Three basic pathophysiological causes of anemia
A
- Decreased RBC production - bone marrow, renal failure, low B12 or folate
- RBC loss - hemorrhage, hemolysis
- Production of defective RBCs
2
Q
Clinical definition of anemia
A
Hemoglobin less than 12 in women and 13 in men
3
Q
Symptoms of anemia
A
fatigue, dizziness, tachycardia, pallor, orthostatic HTN, exertional dyspnea
4
Q
What are reticulocytes?
A
immature RBCs
5
Q
What is MVC measure?
A
volume of average RBC in sample
6
Q
MCV of microcytic and macrocytic anemia?
A
Microcytosis = MCV less than 80 (small cells) Macrocytosis = MCV +100 (big cells)
7
Q
How to determine blood cell morphology?
A
peripheral blood smear
8
Q
What measures are included in CBC?
A
WBC, RBC, hematocrit, platelet count, retic count, MCV, RDW, MCHC
basophil, eosinophil, lymphocyte, monocyte, neutrophil %s
9
Q
What is the significance of low reticulocyte count?
A
means poor RBC production
seen in all microcytic anemias
10
Q
Most common cause of microcytic anemia
A
iron deficiency
11
Q
Causes of iron deficiency
A
GI bleeds (think NSAIDs) Decreased iron intake Decreased iron absorption
12
Q
Signs of iron deficiency anemia
A
fatigue mouth ulcers palpitations pallor brittle nails angular cheilitis dysphagia pica
13
Q
What is ferritin and transferrin?
A
Ferritin = iron stores
Transferrin = transport protein for iron or iron binding capacity
14
Q
What lab is diagnostic of iron deficiency anemia?
A
plasma ferritin < 10 in women and < 30 in men
15
Q
Treatment of iron deficiency anemia
A
ferrous sulfate 325 TID x 6 months
16
Q
Side effects of iron supplementation
A
lots of GI issues
17
Q
Epidemiology of alpha and beta thalassemia
A
alpha - Southeast Asia, Middle East, African
beta - Mediterranean
18
Q
minor vs major thalassemia
A
Minor = gene inherited from one parent; few or no symptoms
Major = gene inherited from both parents
- Alpha major = Bart’s Hgb
Still born or death soon after birth (hydrops fetalis typical outcome)
- Beta major = Cooley’s anemia
Born without a problem, but develop severe anemia over the first year and typically don’t survive past 30 yo
19
Q
Symptoms of thalassemia
A
bone issues, splenomegaly, jaundice, severe anemia, osteopenia, fatigue
20
Q
How to definitively dx thalassemia
A
HgB electrophoresis
21
Q
Treatment of thalassemia
A
Folic acid
Serial blood transfusions but watch for iron overload
Genetic counseling
Splenectomy
22
Q
Basophilic stippling on peripheral blood smear seen in what 3 hematologic diseases?
A
beta thalassemia
lead poisoning and sideroblastic anemia
23
Q
Diagnostic test of choice for sideroblastic anemia
A
Prussian blue staining of bone marrow - shows ringed sideroblasts
24
Q
How can sideroblastic anemia be acquired?
A
alcoholism
lead poisoning
myelodysplasia
25
Heinz bodies seen in what anemia?
alpha thalassemia
| G6PD deficiency anemia
26
What are Howell-Jolly bodies? in what diseases?
DNA remnants seen with macrocytic anemia or damaged spleen
B12 or folate deficiency anemia, sickle cell anemia, celiac disease
27
Hemolysis of macrocytic anemia can progress to ______ dysfuction.
spleen
28
Microcytic anemias
iron deficiency
thalassemia
lead poisoning
29
Macrocytic anemias
folic acid deficiency
B12 deficiency
Sickle cell
G6PD deficiency
30
hypersegmentation of PMN nuclei on peripheral blood smear =
folic acid or B12 deficiency
31
stocking glove paresthesias =
B12 deficiency or diabetic neuropathy
32
bite cells =
G6PD deficiency
33
Clinical presentation of folic acid deficiency
sore/swollen tongue
GI issues - diarrhea
mouth and peptic ulcers
anemia sx's
34
Pathophysiology of pernicious anemia
inability of GI tract to absorb B12 due to missing transport protein called intrinsic factor
35
Causes of B12 deficiency
- Pernicious anemia (#1 cause)
- Vegan diet
- Bowel surgery
- Crohn's disease
36
B12 absorbed where in body?
terminal ileum
37
Clinical presentation of B12 deficiency
neuro sx's: stocking glove paresthesias, ataxia, dementia, depression
anemia sx's
38
How is B12 deficiency differentiated from folic acid deficiency?
Neurologic sx's
Low LDH
Low indirect bilirubin
* Low serum B12 is NOT diagnostic
39
Schilling test
Determines cause of B12 deficiency; pernicious anemia or terminal ileum disease (bacteria Crohns, sprue)
1. IM B12 injection to saturate B12 receptors in ileum
2. Patient given oral radioactive B12
Normal = B12 in urine; B12 binds to intrinsic factor and absorbed into bloodstream
Pernicious anemia = B12 in urine only if patient also given intrinsic factor
40
B12 deficiency treatment
Treat underlying cause if possible
Intramuscular B12
High dose of oral B12 will allow some to pass through small intestine by passive diffusion without intrinsic factor
Oral intrinsic factor may be given, but if patient has intrinsic factor antibodies this method will not be effective.
Neurological symptoms which are treated within the first 6 months do well. Starting treatment after 6 months of symptoms may result in permanent damage.
41
Etiology and Epidemiology of Sickle cell anemia
autosomal recessive
| predominantly African American
42
What is a sickle cell crisis?
Life-threatening; acute painful episode due to ischemia somewhere in body
Increased risk associated with dehydration, acidosis, and hypoxemia
43
How do carriers of sickle cell trait present?
Asymptomatic unless under stress (dehydration, acidosis, hypoxia)
44
Sickle cell anemia makes patients at increased risk for what other conditions?
splenomegaly (pneumococcal infections), non-healing ulcers, strokes, retinopathies, osteomyelitis, necrosis of femoral head
45
Elevated retic count anemia
```
Sickle Cell
G6PD deficiency (during hemolytic episode)
```
46
Definitive diagnosis method for Sickle cell anemia
electrophoresis - HbS in red blood cells
47
Sickle cell treatment
No specific treatment
Folic acid supplement and pneumococcal vaccination
Acute painful episode – fluids, pain meds, and oxygen
Exchange transfusion may be necessary in severe crisis
48
Drugs that increase risk of hemolytic episode
```
Antimalarials
Sulfonamides
Methylene blue
Aspirin
Fava beans
Menthol
```
49
I came in to see my physician assistant because of…
Prolonged jaundice
Hemolytic crises secondary to illness, certain medications or foods.
G6PD deficiency anemia
50
Treatment of G6PD deficiency anemia
Supportive; episodes are self-limiting
| Prevention is key
51
Inheritance of G6PD deficiency anemia
X-linked recessive
52
Signs of anemia + thrombocytopenia + decreased WBCs =
Normocytic anemia
53
Normocytic anemia treatment
```
Treatment of underlying disease
Bone marrow transplant
Immunosuppression
Erythropoietin
Blood transfusions
```
54
Define hemophilia
a clotting factor deficiency
55
Utility of measuring PTT and aPTT
- Assess intrinsic (12, 11, 9, 8) and common coagulation pathways (10, 5, 2, 1)
- Monitor Heparin therapy
*PTT longer name than PT, and intrinsic pathway more coagulation factors
56
Causes of delayed PTT include...
Heparin use, hemophilia, sepsis secondary to coagulation factor consumption
57
Utility of measuring PT and INR
- Assess extrinsic pathway of coagulation (VII = tissue factor)
- Monitor Warfarin therapy
- Check for liver damage
58
Causes of delayed PT/INR
Warfarin use, liver damage
59
Bleeding time used to assess _______.
platelet function
60
Most common inherited clotting disorder
von Willebrand disease
61
Functions of von Willebrand's factor (vWF)
Helps platelets stick to blood vessel wall and bind to factor VIII
Increases half-life of circulating factor VII
62
Symptoms of bleeding disorders
easy bruising/bleeding
frequent bloody noses
bleeding gums
heavy menses
63
Results of following tests for von Willebrand disease:
PT/INR
PTT
PT/INR normal
PTT usually prolonged
64
von Willebrand disease treatment
Desmopressin
Factor VII concentrate
Platelet concentrates may be given in severe cases
65
Hemophilia A is deficiency in factor ____ and Hemophilia B is deficiency in factor ____.
VIII
| IX
66
Inheritance of hemophilia A and B
X-linked recessive
67
Results of following tests for Hemophilia A and B:
PT/INR
Bleeding time
PTT
PT/INR are normal
Bleeding time is normal
PTT is prolonged
68
What type of hemophilia primarily occurs in Ashkenazi Jews?
Hemophilia C
69
Hemophilia C is deficiency in what?
coagulation factor XI
70
Treatment of Hemophilia A, B, and C
Mild cases of A and B: Desmopressin to encourage factor VII release
Severe cases of A and B: factor concentrate 2-3x/week; patients may develop antibody to factor VIII and IX with treatment
C: tx usually unnecessary unless severe trauma or surgery; fresh frozen plasma, platelets, Desmopressin
71
Congenital cause of hypercoagulable states
Factor V Leiden
72
Acquired causes of hypercoagulable states
```
Antiphospholipid syndrome (autoimmune disease where antibodies against cell membranes)
Poor blood flow (sedentary, Afib, Sickle cell disease, Polycythemia vera)
Cancer
Pregnancy
Nephrotic
Inflammatory bowel diseases
Estrogen/birth control
Obesity
Recent surgery
```
73
What is Polycythemia vera?
excess production of red blood cells
74
type of clots
DVT
Pulmonary embolism
Superficial vein thrombosis (SVT)
75
How to manage a hypercoaguble state?
Treat underlying cause if possible
Long term warfarin – risk of major bleed is 3% per year
76
How should pregnant woman with hypercoaguability be treated?
Warfarin is teratogenic so women who may become pregnant should use low molecular weight heparin
77
________ defined as platelet count less than 50,000.
thrombocytopenia
78
What distinguishes petechia and purpura from other rashes?
do not blanch
79
Megathrombocytes
immature platelets recently released from bone marrow
80
Petechiae and purpura are found on the skin and mucous membranes
Low platelet count with no apparent cause
ITP
81
ITP treatment
Avoid aspirin and NSAIDS
Typically no treatment is necessary for either acute or chronic disease
Steroids
Splenectomy
Platelet transfusion in cases of severe trauma
82
Petechiae and purpura
Low platelets
Labs tests showing less than 5% normal ADAMTS13 protein
DX?
TTP
83
Symptoms of TTP
```
Petechiae and purpura
Malaise
Fever
Bleeding from the nose and gums
Neuro sx's including hallucinations and altered mental status
Kidney failure
```
84
TTP treatment
Plasma exchange
Steroids
Without treatment 95% of patients will not survive. With treatment this number drops to around 10%
85
Similarities and differences between TTP and DIC
Both have hemolysis and thrombocytopenia
TTP has abnormal renal function but normal PT/PTT
DIC has normal renal function but prolonged PT /PTT causing increased clotting
86
DIC treatment and prognosis
Platelet transfusion
Fresh frozen plasma
Anticoagulation may be necessary??
10-50% of patients will not survive
87
Disease characterized by overproduction of WBCs
leukemia
88
What are PE findings of leukemia?
swollen lymph glands, enlarged spleen
89
How is chronic and acute leukemia differentiated in lab?
acute - cells immature from fast increase in WBC production
chronic - slower buildup of WBC so mature cells; cells still abnormal and non-functioning
90
Myelogenous vs lymphocytic leukemia
Lymphocytic - overproduction of lymphocytes
Myelogenous- overproduction of RBCs as well as some WBCs and platelets
91
Symptoms of leukemia
```
Symptoms of anemia
Weakness
Fatigue
Fever/infection
Decrease in appetite
Bone and joint pain
Enlarged lymph nodes
Petechia
```
92
Leukemia in ages 2-5 with excessive lymphoblasts
ALL
93
Bone marrow biopsy of ALL and AML
hypercellular
94
Most common type of leukemia, majority of men over 50 yo
CLL
95
Auer rods are pathonomonic for ______.
ALL
96
Leukemia treatment
Chemotherapy
Radiation
Steroids
Bone marrow transplant
97
Which leukemias can present as asymptomatic?
CLL, CML
98
Which leukemia can be managed with tyrosine kinase inhibitors?
CML
99
Unlike leukemia, __________ is a solid tumor.
Hodgkins lymphoma
100
Staging 1-4 for lymphoma
Stage 1: single site
Stage 2: two or more lymph nodes on same side of diaphragm
Or one extralymphatic site and one lymph node on same side
Stage 3: Affected lymph nodes on both sides of diaphragm and involvement of spleen or another extralymphatic site
Stage 4: Presence of B symptoms (fever, weight loss, night sweats) or diffuse extra lymphatic involvement
101
Lymph node biopsy showing ________ is diagnostic for Hodgkins lymphoma.
Reed-Sternberg cells
102
Differences between Hodgkins and non-Hodgkins lymphoma?
- Both cancer of WBCs (lymphomas)
- Non Hodgkins more common
- Hodgkins has Reed-Sternberg cell on microscopy
103
Build up of plasma cells in the bone marrow interfering with normal production
Multiple Myeloma
104
Findings of Multiple Myeloma on skeletal survey
XR of skull shows punched on lesions
Lytic lesions
105
Why is hypercalcemia seen in multiple myeloma?
excessive bone resorption
106
Multiple Myeloma treatment and prognosis
No treatment other than treating end organ damage
Chemo with stem cell transplant
5 yr survival rate 35%
107
Philadelphia Chromosome is pathognomonic for _____.
CML
108
Bence-Jones proteins and rouleaux formation on peripheral smear
multiple myeloma
109
hematopoiesis
development of blood cells
110
How is fetal hemoglobin different than adult?
greater affinity for oxygen
111
What is hematocrit measure?
percent of blood volume composed of RBC
112
Normal range for WBC
4.5K to 11K
113
Normal range for platelets
150K to 400K
114
What is the spleens role in hematologic system?
- filters out malformed, damaged, or old RBCs
| - hematopoiesis in fetal period
115
Site of hematopoiesis
bone marrow in adults
| spleen in fetus
116
What is aplastic anemia?
loss of hematopoietic cells leading to pancytopenia (decrease in all blood cell types)
117
Pathologies of the hemoglobin
Sickle cell
| Thalassemias
118
Lab findings of hemolysis
```
increased indirect bilirubin
increased LDH
increased reticulocytes
decreased free haptoglobin
schistocytes
```
119
Type I hypersensitivity reaction
Anaphylaxis
IgE mediated
Allergies (food, dust, insects)
120
Type II hypersensitivity reaction
Cytotoxic
| mediated by IgG, IgM antibodies
121
Hypersensitivity reaction with antigen-antibody complexes
Type III
122
Cell mediated or delayed hypersensitivity reaction
Type IV
123
What cells produce antibodies?
lymphocytes
124
_________ is proliferation of WBCs in bone marrow and _________ is proliferation in lymphatic tissue.
Leukemia
| Lymphoma
125
Where is lymphatic tissue?
lymph nodes and spleen
126
Causes of elevated WBC
```
infection or inflammation
autoimmune disease
hematologic malignancy (leukemia, lymphoma)
```
127
Cells that arrive at site of inflammation first
neutrophils
128
Coagulation factors that need Vitamin K for synthesis?
Mneumonic: 1972
X, IX, VII, II
129
What does abnormal PT and normal PTT mean?
problem with extrinsic pathway of clotting cascade
130
MOA of Coumdadin and how is it monitored
anticoagulation; interferes with Vit K-dependent clotting factors (10, 9, 7, 2)
monitored by PT
131
MOA of Heparin and how is it monitored
anticoagulation; increases antithrombin III activity and decreases amount of fibrin
monitored by PTT (intrinsic)
132
What helps platelets stick together when forming a clot?
fibrinogen (factor I)
133
Pathologies that cause platelet loss or destruction?
ITP
TTP
HUS
DIC
134
Byproduct of RBC breakdown
Bilirubin, iron, and amino acids
135
When _____ is negative, this rules out PE in a low risk patient.
D-dimer
