MIDTERM Flashcards
1
Q
3 Etiologies of ANEMIA
A
Blood LOSS
DEC Production
INC Destruction
2
Q
Acute vs. Chronic Etiologies Blood Loss
A
Acute Trauma/hemorrahage
Chronic: GI/GYN lesions, malignancies
3
Q
Myelophthisic anemia, aplastic anemia and pure red cell dysplasia are due to problems in what?
A
Problem with Bone Marrow
4
Q
What proinflammatory cytokine is seen in anemia
A
IL-6
5
Q
= negative regulator of Fe metabolism
A
HEPCIDIN = inhibitor
6
Q
RELATIVE causes
Reduction of plasma volume leading to hemoconcentration
Ex: Vomiting, Diarrhea and Polyuria
A
Polycythemia – Erythrocytosis
7
Q
Polycythemia Absolute Causes: PRIMARY OR SECONDARY?
abnormal proliferation of myeloid stem cells with normal or low EPO levels
Ex: Polycythemia Vera
A
PRIMARY
problem at bone marrow
8
Q
Polycythemia Absolute Causes: PRIMARY OR SECONDARY?
proliferation of myeloid stem cells in the setting of elevated EPO levels
A
SECONDARY
no problem at bone marrow – too much stimulus
9
Q
Where is EPO produced and what does it stimulate?
A
Produced by kidneys, stimulates RBC synthesis in bone marrow
10
Q
What can cause elevated EPO?
A
LOW O2
* chronic lung disease
* cyanotic heart disease
* high alt living
* EPO secreting tumors
* intake of EPO (athletes)
11
Q
Hematopoietic stem cell –> Myeloid stem cell –> proerythroblast –> reticulocyte –> RBC
A
HEMATOPOIESIS
12
Q
immature RBCs
too many of these means the body is losing RBCs faster than they can make them
Hemolysis hemorrhage
A
RETICULOCYTES
13
Q
What is the function of HEPCIDIN?
A
inhibit Fe absorption from GI, sequesters Fe in liver and macrophages
release of IL6 –> Hepcidin release from liver –> DEC serum Fe
14
Q
measures volume percentage of RBC in blood
A
HEMATOCRIT (HCT)
15
Q
measure of the amount of Hgb per cell
A
MCH
mean corpuscular hb
16
Q
measure of the concentration of Hgb in each RBC
A
MCHC
mean corpscular hb concentration
17
Q
What protein STORES iron and what protein TRANSPORTS iron?
A
Stores – Ferritin
Transport – Transferrin
TIBC = how much Fe can be bound to transferrin
18
Q
IRON Studies
A
19
Q
Hemoglobin Electrophoresis
Order the Hb travelled from furthest to least travelled
Hb = neg charge – travells to anode
A
HbA –> HbF –> HbS –> HbC
20
Q
Characterization based on morphology
small and pale RBCs
less than normal amt of Hb
MCV = < 80 fL
A
Hypochromic/Microcytic
Hb prob – iron def. anemia, thalassemia, anemia of chronic disease
21
Q
Characterization based on morphology
normal color and size RBCs
MCV = 80 – 100 fL
A
Normocytic/Normochromic
NOT ENOUGH CELLS
hereditary spherocytosis, sickle cell, acute blood loss, myelofibrosis
22
Q
Characterization based on morphology
cells are larger than usual
MCV = > 100fL
A
MACROYTIC
DNA PROBLEM
B12 deficiency anemia, folate deficiency
23
Q
Where is TPO made ?
A
LIVER
24
Q
Petechiae are a sign of what?
non-blanching, red lesions less than 2cm
A
THROMBOYCTOPENIA
25
# Thrombocytopenia - which microangiopathic hemolytic anemia
Etiology: **GI infection**, pregnancy, autoimmune
TRIAD: Thrombocytopenia, Hemolysis , Kidney Disease
| TREATMENT = plasmapheresis
Hemolytic Uremic Syndrome
26
# Thrombocytopenia - which microangiopathic hemolytic anemia
Etiology: **ADAMTS 12 deficiency**
PENTAD: fever, anemia (hemolysis), thrombocytopenia, Renal failure, neurologic symptoms (**encephalopathy**)
| FAT RN
Thrombotic Thrombocytopenic Purpura
TTP
27
# thrombocytopenia
CAUSE = **autoab against platelets cause thrombocytopenia**
Assoc. Dx = SLE, infection, HIV
Signs/Symptoms = epistaxis, menorrhagia, petechiae
Immune Thrombocytopenic Purpura (ITP)
28
What causes an issue in coagulation?
Decreased or dysfunctional clotting factors
29
Where are clotting factors synthesized?
LIVER
30
What is an important cofactor of the coagulation cascade?
VITAMIN K
31
# What disease?
Autosomal Dominant
Defect in **Factor 8** & **vWF complex**
Symptoms: excessive bleeding, easy brusing, menorrhagi, prolonged bleeding
| Treatment = DESMOPRESSIN
Von Willebrand disease
| labs show DEC ristocetin cofactor assay
32
# What disease?
X-Linked Recessive
Factor 8 deficiency
| Treatment = Factor 8
Hemophilia A
| **Hemophilia B = Factor 9 deficiency **
33
**Mononucleosis** is caused by what?
| **lymphocytosis of activated, CD8+ T cells (atypical lymphocytes**)
**EBV**
34
severe neutropenia =
Risk of infection : **< 500 cells /uL**
| treatment = G-CSF
AGRANULOCYTOSIS
35
inherited intrinsic defect in RBC membrane (ankyrin,
spectin, band3)
heriditary spherocytosis
36
inherited defect in the hemoglobin molecule B globin chain
Sickle cell anemia
37
inherited disorders in **globin genes** that **decrease the synthesis of alpha or beta globin**
Thalassima
38
acquired mutation in the PIGA gene
| Dark Tea Urine, hemoglobinuria, blood clots in VEINS
paroxysomal nocturnal hemoglubinuria
39
caused by defects that increase destruction of RBCs by **phagocytes in the spleen**
Hyperbilirubinemia and jaundice
EXTRAVASCULAR
40
RBC explode within circulation due to injury of the RBC membrane
Hemoglobinemia, Hemoglobinuria and **HEMOSIDERINURIA**
| Loss of iron
INTRAVASCULAR HEMOLYSIS
41
RBCs look more rounded with **missing central pallor**
Hereditary Spherocytosis
42
Screening test for hereditary Spherocytosis
Osmotic fragility
43
Pathogenesis: (HbS) **Single amino acid substitution in B globin chains** --> deoxygenated hemoglobin leading to distrotion of RBC shape
| (HbC) **LYSINE** residue in place of **GLUTAMIC** ACID -- only trait
SICKLE CELL ANEMIA
hemoglobinopathy/hereditary
44
Severe Hemolytic Anemia is due to what?
Severe membrane damage
45
Deoxygenation causes sickling →
Microvascular Occulusion
46
this disease is due to abnormally low production of alpha or beta globin chain
THALASSEMIA
47
Diagnosis of Alpha vs. Beta thalassemia
Alpha -- small RBC
Beta -- hemolytic anemia/high fetal HbF
48
Which chromosome is associated with Alpha and Beta Thalassemia
ALPHA = 16
BETA = 11
49
The severity of alpha thalassemia INCREASES based on what
3 genes --> 0 genes
MOST SEVERE = 0 genes present
| **Hb Barts**
50
Barts hemoglobin on screening newborns means what are present?
| **Hydrops fetalis (alpha thalassemia
major**
Alpha gene deletions are present
| Left Shift in Curve -- No O2 to tissues-- baby dies in utero
51
Pt. presents with **severe anemia w/ abnormal facial appearance**, upper teeth protrusion, physical and mental delays
| **LOW reticulocyte count/crew cut skull X Ray**
BETA THALASSEMIA **MAJOR**
52
Pt. Presents with **jaundice** and **splenomegaly**, Labs show **Hb and MCV decreased**
Fe/RBC are normal
Blood Smear -- **microcytosis**
Beta Thalassemia **MINOR**
53
You see **Heinz Bodies & Bite Cells** on peripheral smear
G6PD Deficiency
| G6PD reduces NADP+ to NADPH while oxd G6PD
54
You see **BURR** **cells** on peripheral smear
**Inhibits prod. of ATP** and pyruvate by blocking glycolytic pathway
Pyruvate Kinase Deficiency
55
hereditary, aut. recessive blood disorder in which large numbers of RBC are **ellipitical** in shape
RBC **membrane defect**
Most common defects are in genes for the polypeptides **alpha spectrin and beta spectrin**
ELLIPTOCYTOSIS (Ovalocytosis)
56
In patients with iron loss name two serum lab tests that will change first?
Ferritin and TIBC
57
Compared to a patient in NEG. IRON Balance, a patient with IRON DEFICINET ERYTHROPOIESIS, now has changes in what 3 lab tests?
DEC Serum Iron (SI) / % Saturation
INC RBC protoporhyrin
58
# IS IT AN UPPER OR LOWER GI BLEED
If a patients presents with Hematemesis, coffee ground emesis, nause/vomitting, epigastric pain, melena, or syncope?
UPPER GI BLEED
59
# IS IT AN UPPER OR LOWER GI BLEED
If a patient presented with any of the following: hematochezia, tenesmus, diarrhea
LOWER GI BLEED
60
What are treatment options for **anemia of chronic inflammation?**
Treat the underlying condition
* Rhematoid Arthritis: with disease modifying agents (**DMARDS**)
* **EPO** for severe and symptomatic anemia
61
what is an acute phase reactant that binds ferroportin --> **DEC dietary iron transport and macrophage recycling (from dying RBCs**)
HEPCIDIN
62
A bone marrow biopsy shows FATTY HYPOCELLULAR bone marrow cells
APLASTIC ANEMIA
63
What lab serum level is only elevated in B12 deficiency and not in folate deficiency?
Methylmalonic acid (MMA)
| homocysteine is elevated in both
64
Presence of **Anti-intrinsic factor antibodies** and anti-parietal cell antibodies are diagnostic for what?
PERNICIOUS ANEMIA
65
pernicious anemia causes an INCREASED risk for what type of cancer?
GASTRIC CANCER
66
Fe2+ is absorbed in the duodenum via what receptors?
**DMT1** and **HCP1** receptors
67
# PARENTERAL IRON Forms
What two ways can iron deficient anemia patients with malabsorption syndromes or pts. intolerant to oral iron be treated ?
Ferric hydroxide + dextran
Iron Sucrose
68
Treatment for Iron TOXCITIY
administration of **deferoxamine**
69
A **decrease** in production of what results in a**cquired penicious anemia**
INTRINSIC Factor
70
What **oral B12 therapy** is preferred because it is more **highly protein bound** and therefore remains longer in the circulation.
Hydroxocobalamin
71
What symptoms differentiates folate deficiency and Vit B12 deficiency?
VIT B 12 --> NEURO SYMPTOMS
72
What is the best treatment option for megaloblastic anemia?
| folate can mask b12 deficiency/cause irreversible neuro dysfunction
Combo of FOLATE + CYANOCOBALAMIN
73
What is the treatment for patients with **anemia and chronic renal failure**
EPO
74
What is the major side effect of EPO therapy?
ELEVATED DIASTOLIC BP
75
# gram - rod
What pathogen is responsible for **Cat-Scratch Disease?**
| Fever, malaise/swollen lymph nodes
Bartonella henselae
| transmitted via cat saliva
76
A patient presents with a **nodular lymphangitis**, says they were **gardening**
Sporothrix Schenckii
Cutaneous SPOROTRICHOSIS
| DIMORPHIC FUNGI
77
What disease is transmitted via **SANDFLY**
| protozoan parasite
Leishmania
78
**Ixodes Tick** causes what
| Protozoa
Babesia
Blood Smear --> MALTESE CROSS pattern
79
What are transfusion trigger symtoms?
serere blood loss
Collapse, air Hunger, Anuria (no urinary output)
80
cryopreciptate and desmopressin (DDAVP) provide what?
| used in tx of hemophilia A, von wilebrnad dx, hypofibrinogenemia
Factor 8, vVWF, and fibrinogen
81
Lyophilized Factor IX has freeze dried factors ??
2, 7, 9, snd 10
82
What determines the blood type?
Agglutinogens = RBC surface antigen
83
What blood type = UNIVERSAL DONOR
O neg
| univ. reciever = AB+
84
**febrile non-hemolytic reaction** can occur during a transfusion due to what?
anti-wbc or anti-platelet antibodies insenistized patient
| STOP TRANSFUSION
85
patient recieved blood transfusion, now is in **hypoxic resp distress**, has **pulm crackles w/o signs of CHF**
CXR --> **bilateral pulm edema**
White out patchy infiltrates
Transfusion Related Acute Lung Injury
86
Virchows Triad
Stasis of blood
Endothelial injury
Hypercoagable states
87
Heparin/Heparan sulfate activates what
Antithrombin 3 --> inactivates 2, 9, 10, 11, 12
88
protein C breaks down what factors
5 and 8
| the Cat ate (8) my hand (5)
89
most common inherited thrombophilia identified =
**Factor V Leiden** Mutation
factor 5 resist breakdown by protein C
90
Prothrombin G20210A
pt. mutation where adenine subs for guanine at position 20210
END RESULT = **higher circulating prothrombin**
| adenine = ADDs, guanine = GONE
91
thrombosis (recurrent) +/-
morbidity associated with preg. complications
Positive AB: ABC
Lupus Anticoagulant
Anti Beta 2 glycoprotein 1 ab
Anto Cardiolipin ab
Antiphospholipid Syndrome
92
Heparin Induced Thrombocytopenia -- two types
Type 1 -- non immune transient 30% dec in platelets - no thrombosis
Type 2 -- immune mediated (ab complex with hep and PF4)
93
# Paroxysmal Nocturnal Hemoglobinuria
What proteins inhibit the complement pathway?
CD55DAF and CD59 MIRL
94
# Paroxysmal Nocturnal Hemoglobinuria
What is the anchor protein that anchors CD55DAF and CD59 MIRL to the membrane of the myeolid cell?
GPI
if theres a deficiency in this
Complement pathway INC
| development of venous thrombosis in atypical placess
95
What is the gold standard to detect absence of CD55 and CD59?
Flow Cytometry
96
what binds exposed collagen?
vWF
97
platelet adhesion uses what receptor
Gp-1b
98
platelet aggregation uses what receptor?
| want 2 b 3
Gp2b and 3a
and thromboxane TXa2= vasoconsrtictor
99
labs show **prolonged PTT**
| intrinsive pathway: 12, 11, 9, 8
Factor VIII deficiency = Hemophilia A
| x-linked recessive
100
How can DDVAP be used as a treatment for Hemophilia A
DDAVP (vasopressin) inc factor 8 stores by stimulating vWF from endothelial cells
101
vWF is coupled with what factor?
Factor 8
| important for platelet adhesion -- bleeding time can be prolonged
102
if the only deifciency is **BLEEDING time** then the problem is with what?
problem with platelets
**BERNARD SOULIER DISEASE = defect with platelet adhesion**
103
what disorder has a defect in **platelet aggregation**?
**glanzmann's thrombastenia which decreases GP2b-3a**
104
what are the vitamin K dependent factors?>
2,7,9, 10 and C + S
105
warfarin inactivates what vitamin?
VIT K
106
Hemostatsis occurs in TWO stages
1. Platelet pLug
2. 2. stabilize the platelet plug (coag cascade)
107
platelets are granular cells derived from what?
Megakryocyets
108
primary cells responsible for hemostasis
Platelets
109
What hormone regulates the formation of platelets?
Thrombopoietin
110
What is the first factor to join the intrinsic cascade?
factor 12
111
How does factor 7 get activated in the Extrinsic pathway/?
It has to be exposed to Tissue Factor (Factor3)
112
What factor is needed for cross-linking the fibrin mesh
Factor 13
113
What factor is called the stuart prower factor
Factor 10
114
What prevents formation of embolus?
Anticoagulation
115
What cofactor is vital for coagulation?
Ca2+
116
What vitamin is used in the production of Factors 2, 7, 9, and 10
VITAMIN K
| kale, spinach, collards
## Footnote
absence of K -- leads to uncontrolled bleeding
117
What vitamin is used in the production of Factors 2, 7, 9, and 10
VITAMIN K
| kale, spinach, collards
## Footnote
absence of K -- leads to uncontrolled bleeding
118
**Vitamin K** hydroquinone acts as a cofactor to
**gamma-glutamly carboxylase**
119
how do we recyle vitamin K
vitamin K epoxide reductase
| blood thinners INHIBIT this
## Footnote
warfarin or coumadin
120
thrombodulin and thromin activate what protein
PROTEIN C
121
deficiencies of proteins C and S can cause
more clotting than you need
