Hematology: Block 1 Flashcards

1
Q

What are the roles of the three proteins found in plasma?

What are the components of solutes?

A

Albumin: retain fluids in blood
Globulin: simple proteins; clotting and infections
Fibrinogen: clotting, converted by thrombin

WHEN EV
Waste Hormone E+ Nutrient
Enzyme Vit

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2
Q

All cell sizes are compared to a ‘normal’ RBC size which is ?

Define RBC

A

6-8um (one millionth of a meter; one thousandth of millimeter)

Biconcave anuclear disc, eliminated by liver/spleen

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3
Q

Define WBC

What are the life spans of the different types of WBCs

A

Differentiated nucleated motile cell

Typical: few hrs (infxn)
T/B Cell: years

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4
Q

What is the general rule to WBC concentration locations?

Define Platelet

A

Less in circulation, more in tissues

Biconvex anuclear discoid w/ lens shape if inactivated

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5
Q

How are platelets activated

How long do RBC, WBC and Platelets live

A

Pass blood vessel w/ disrupted endothelium
Activation causes pseudopodia, adhere to form clots

RBC: 120 days
WBC: hrs to years
Platelet: 5-9 days

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6
Q

Define Hematopoiesis and what are the two forms

Extra medullary poiesis mainly occurs in what 2 areas

A

Production-maturation of all blood cells

Spleen- 53%
Liver- 25%

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7
Q

What issues can trigger extra medullary poiesis to kick in?

This form of production accounts for ? of all hematopoiesis

A

BI HALL
Breast Ca Infection HIV Anemia Leukemia Lymphoma

3%

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8
Q

What are the four locations of prenatal blood development

Define Pluripotential Stem Cell

A

Yolk* Liver Spleen
Bone marrow closer to delivery

Stem cells w/ potential to develop into any tissue/cell in body

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9
Q

What are the 3 properties held by pluripotential cells

Upon replication, they first divide into ?

A

Proliferation potential
Multiple lineages
Self renewal

Myeloid/Lymphoid Stem Cells

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10
Q

What are the 3 hematopoietic Growth Factors, development site and role

A

EPO- kidneys; RBC production

TPO- liver; thrombocyte production

Cytokines (CSF, IL)- many; WBC production

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11
Q

Where are Colony Stim Factors and Interleukins released from for WBC production

What are the cell transformations in the line of erythropoiesis

A

ME My BFS
Macrophage Endothelial Mast B/T cell Fibroblasts Stromal

Potential Myeloid Progenitor Blast (per) RBC

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12
Q

When kidneys detect low O2 and release EPO, it triggers ? cell to continue hematopoiesis

What are the cells in sequence of thromboblast development

A

Proerythroblasts

Potential Myeloid Progenitor Megakaryocyte Platelet

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13
Q

What is the sequence of thrombocyte pseudopodia formation

Leukopoiesis can develop from what two cells into ? cells

A

C-TPO + endothelial damage= internalization/pseudopodia

Myeloid: BENM
Lymphoid: B/T/NKC

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14
Q

What is the only cell myeloid stem cells can NOT differentiate into?

RBC has ? many Hbg units and what do those units contain

A

Lymphocyte

280M units
Each unit= 2A 2B chains

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15
Q

What is the best measurement of blood’s O2 carrying capacity

What part of the RBC contains the pigment

A

Hgb

Heme w/ Fe

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16
Q

One RBC w/ 300M hgb molecules can hold ? O2 molecules

One Hgb contains _O2, _Fe, and _hem

A

1 billion

4 4 4

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17
Q

Define RBC count

Define Hct

A

Total number of RBCs

Percent of blood volume that’s RBCs

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18
Q

Define Hgb

Define RBC Indicies

A

Amount of Hgb in blood

MCV: average RBC size/volume

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19
Q

Define MCH

Define MCHC

A

Mean Cell Hgb; average quantity of RBC Hgb

Mean Cell Hgb Concentration; Hgb inside RBC w/ size/vol factored in

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20
Q

Define RDW

A high RDW means theres a greater difference in size/vol, defined as ? and seen ?

A

Red Cell Distribution Width; estimated range of RBC volume and size

Aniscocytosis- post-RBC transfusion

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21
Q

Define Reticulocyte count

What is this measurement the best indicator of?

A

Number of immature RBCs in circulation

RBC production

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22
Q

When would a reticulocyte count be high or low?

What is the most important function of the Reticuloendothelial System?

A

High: anemia
Low: marrow failure

Phagocytosis

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23
Q

What are the two locations the RES functions

A

Intravascular: RBC contents released into circulation
Hgb binds to Haptoglobin- taken to liver

Extravascular: in spleen; heme broken into bili/Fe
Fe to transferrin, to marrow
Bili to albumin, to liver

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24
Q

RBC recycling is dependent up on ? cell in the body

What are the two categories of WBCs

A

Macrophage

Granular: BEN
Agranular: LM

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25
# Define WBC w/ Diff What is the MC WBC
Number of WBCs and %s of each types Neutrophils- granules are pale lilac, pus
26
If neutrophils are elevated, what does this suggest? What other stressors can cause them to be elevated
Bacterial infection Burn Stress Inflammation
27
Eosinophil granules are ? color and are elevated ? Basophil granules are ? color and are elvated ?
Red/orange Allergic reaction Prasite Autoimmune Purple/blue Allergy Leukemia/Ca Hypothyroid
28
Lymphocytes are ? color and present ? Lymphocytes produce ? two types of immunity
Sky blue rim around nucleus Infection Leukemia Mono Cell Mediated: cytokines Humoral: Abs production
29
What are the 3 cells included under lymphocytes? What are the 5 types of cytokines released during CMImmunity
B cells- destroy bacteria, humoral activity T cells- destroy virus, fungi, transplant/Ca cells NKSc- destroy infectious/malignant cells IFN Chemokine Lymphokines IL TNF
30
How do monocytes appear? When are they elevated
Horse shoe shaped, blue/gray nucleus Viral/fungal infection TB Leukemia (some) Chronic Dzs
31
What lab technique offers the best method to view and identify blood cells and components CBCs measure ? 3 things
Peripheral smear RBC WBC Platelet
32
Define the following prefixes: A/An Aniso Pan Poly Poikilo
W/out Unequal All Many/variable Irregular shaped
33
Define the following suffixes: -cytopenia - cytosis - emia - osis - penia
Absent/deficient cells Cells Condition/Dz of blood Process/condition Lack/deficiency
34
# Define Erythrocytosis and the two types What type would be seen if PT was dehydrated
Inc of RBCs, > 5.5 Absolute: high RBC mass Relative: normal RBC mass, low plasma Relative
35
What conditions can cause erythrocytosis? Define erythrocytopenia
HD DR CPC Hypoxia Dehydrate Drugs Renal dz Congenital Polycythemia vera Ca Dec of RBCs <3.5
36
What conditions can cause erythrocytopenia What conditions can cause leukocytosis
``` Ha MR H Hemolytic anemia Marrow failure Renal Dz Hemorrhage ``` I LICS Infection Leukemia Inflammation CCS Stress
37
What conditions can cause Leukopenia What can cause thrombocytosis
B12/Folate deficient Infxn Aplastic/AutoImm Spleen over stimulation Infection/Tissue damage Chronic inflammation/infxn Malignancy
38
What can cause thrombocytopenia
Dec production: marrow failure, malnutrition Inc destruction: AutoImm, transfusion reaction
39
What are the criteria for anemia in men/women What is the MC cause of anemia and what is the MC cause of this MC cause
M: Hct <41%, Hgb <13.5 W: Hct <36%, Hgb <12 Fe deficiency Bleeding
40
How is anemia classified What labs are ordered for the work up process?
Pathophysiologic process: Dec production Inc destruction/loss ``` Retic count CBC Peripheral smear CMP Marrow aspirate/biopsy ```
41
When working up a case of suspected anemias, when is a marrow sample acquired? What are the 3 common yet severe Sxs of anemia
Lab eval fails to ID source Syncope Angina MI
42
How do PTs present on PE if they have anemia What are severe Sxs of anemia that would be seen on PE?
Dyspnea Tachy Fatigued Palpitations Smooth tongue Brittle/spoon nails Pica- craving non-nutritional materials >1mon Cheilosis- B12 deficient causing cracked lips
43
MCV results indicates what part of an anemia? What are the possible etiologies of microcytic anemia, MCV <80
Classificaiton of an anemia ``` Thalassemia Anemia, chronic Dz Iron deficient** Lead poison Sideroblastic ```
44
What are the two functions of Ferritin What lab result measures the blood's capacity to bind Fe w/ transferrin
Storage protein for Fe in cells Fe carrier in serum Total Iron Binding Capacity TIBC
45
What lab result would indicate the amount of stored Fe in the body What hormone regulated Fe storage
Serum ferritin Hepcidin- hormones produced by liver, inc during inflammation/infections (dec absorption)
46
Function of Erythroferrone Function of Ferroportin
Inhibits hepcidin synthesis Fe transporter across intestinal lumen
47
Importance of Hemosiderin How much Fe is balanced in day to day homeostasis
Insoluble form of Fe ONLY found in cells Formed when body has excess Fe 1-2mg in/out
48
Where is Fe absorbed in the GI system Where/how is it distributed through out the body?
Duodenal enterocytes ``` 3-4gm between: RBCs 1800-2000mg Liver parenchyma 1000mg Spleen 600mg Marrow, reticuloendothelial macrophages 300mg Muscles 300mg Plasma transferrin 3mg ```
49
What lab result is not Dx of Fe Deficient Anemia Under normal conditions, how much of transferring is occupied by Fe
Low serum Fe 1/3
50
What are the 7 parts of an Fe panel during an anemia work up
Serum Fe: amount in liquid part of blood TIBC: amount of Fe that an be protein bound Ferritin- amount of stored Fe in blood Transferrin- amount in blood Transferrin Saturation: % saturated by Fe sTfR: functional Fe status sTfR/Log: differentiator between anemia chronic dz and Fe deficient anemia
51
What part of the Anemia Fe Panel is used during investigating Fe Deficient Anemia Why is this result used?
sTfR sTfR not affected by acute phase reactions
52
What are the 3 mechanisms of developing FeDA What is the most important part of FeDA management
Inc loss Dec intake/absorption Identifying the cause
53
What is the first consideration in male >50y/o OR post-menopause female w/ newly onset FeDA How is this consideration investigated
Occult GI Ca EGD, Colonoscopy
54
What are the 4 stages in the development of FeDA? How is mild/mod FeDA Tx
1: dec ferritin 2: hypochromic RBCs 3: Hgb dec 4: MCV/morphology dec 1st line: PO replacement Ferrous Sulfate 325mg
55
How are severe cases of FeDA Tx When using Ferrous Sulfate for FeDA Tx, what drug interactions have to be avoided?
IV Fe therapy w/ IM/Hematology provider Levothyroxin- taken 4hrs apart
56
What is considered an appropriate response to Fe Tx for FeDA How long is Tx continued
Hct return to half of normal w/in 3wks Return to normal by 2mon Hgb/Hct (serum fe) corrected by 6-8wks 3-6mon after return to norm
57
Micro Anemia Anemia of Chronic Dz is characterized by ? How is it Tx
Etiology Pathophysiology Tx underlying cause RBC transfusion Recombinant EPO
58
What extra lab result is needed in order to accurately Dx the type of thalassemia Define Thalassemia
Hgb Electrophoresis Hereditary defected synthesis of globin chains= dec Hgb synthesis
59
What are the different types of thalassemias This condition is usually seen in ? PT populations w/ ? finding
A: gene deletion, dec A/inc of unstable B chains B: point mutation Mediterranean African Asian- Microcytosis
60
What are the hallmark lab results of Microcytic Anemia Thalassemia Based off of these findings, this type of anemia is AKA ?
``` Low MCV- small RBCs Low MCH- pale RBCs Low Hct and Hgb- anemia Norm/Inc RBC count Inappropriate Norm/Inc RTC ``` Microcytic Hypochromic Anemia
61
Normal adults have ? type of Hgb What is a variant that can be seen?
A2B2 A2D2
62
What is Hgb F made of What types of Hgb cross and switch roles at birth
A2G2 Gamma- 2nd highest in vitro B- 2nd highest in adulthood
63
What is the make up of Hgb H What is the make up of Hgb Bart's
4B 4G
64
What is the Hgb make up of Hydrops Fetalis After FeDA is excluded, what is the next Dx due to clinical suspicion
0A MAT
65
What therapeutic Txs will be needed in PTs w/ MAT What are 3 abnormal morphologies that would be seen on a peripheral smear of MAT?
Transfusions- Fe overload and high RBC turn over Microcytes Acanthocytes- Greek, thorn Codocytes- target cells
66
# Define Thalassemia Trait Define Thalassemia Intermedia
Lab features w/ no clinical impact Occasional transfusion required, moderate clinical impact
67
# Define Thalassemia Major What is the make up of MAT silent carrier
Life threatening, transfusion dependent AA/A-
68
What are the two genotypes of Alpha Thalassemia Minor and are on what chromosome? Alpha thalassemia intermedia is AKA ? and has ? globulin make up
Homozygous: AA/-- Heterozygous: A-/A- Chrom 16 Hgb H Dz: A-/--
69
? form of A-thalassemia affects Asians or Blacks How are PTs w/ Hgb H Dz managed
Asian: Hetero A-/A- Black: Homo A-/-- Daily folate Avoid supplemental Fe Possible transfusions during infections/stress
70
PTs w/ MAT Hgb H Dz need to avoid what drug classes?
``` Antimalarials Sulfa Antibacterials TB Analgesics ``` ``` Fe supplements Pyrimethamine Methylene blue Napthalene (moth balls) Fava bean ```
71
What is the underlying issue in MAT B-Thalassemia What can the missing Alpha chains be substituted with here?
Dec B production causes excessive A chains W/ D chains= HbA2 W/ G chains= HbF
72
What happens when there is an excess in A chains during MAT B-T MAT B-T is more common in PTs w/ ? genotype
Unstable and incapable for forming tetramers, cause RBC membrane damage Homozygous for impaired B-globin synthesis
73
What are the 3 types of B-thalassemias What lab result may be seen in B-thalassemia minor that is not present in the Alpha-thalassemias
Minor: B+/B Intermedia: B+/B+ Major: B+/B+, RBCs have nucleus (Cooley's Anemia) Basophilic stippling
74
What do the electrophoresis results look like when testing for thalassemias? What are the only two heterozygous B-globin B-thalassemias
A: normal Hgb H: inc Hgb H B: +Gamma= Hgb F - Delta= Hgb A2 Minor Intermedia
75
How are cases of mild A or B thalassemia Tx How are severe cases Tx
None, ID'd to avoid repeat evaluation or Fe deficiency B-I/Major: spleenectomy Marrow transplant- TxOC for major
76
What is an adverse outcome that can occur when Tx severe thalassemias w/ too many transfusions? What causes sideroblastic anemia?
Iatrogenic Fe overload syndrome Mitochondria mishandle serum Fe= no Fe in Hgb by RBC precursors
77
What happens to RBCs that have too much Fe in Sideroblast anemia What does the presence of basophilic stippling represent in a Sieroblastic anemia?
Ring sideroblasts- Fe loaded mitochondria seen by Prussion blue stain (Perls reaction) in perinuclear ring of blue granules Basophil granules dispersed in RBC cytoplasm seen on peripheral smears Indicates disturbed erythropoiesis, not Fe
78
What are the causes of Sideroblastic anemia
Acuqired: MC, drugs/toxins Inherited Ethanol INH Cycloserine Chloramphenicol Busulfan Copper chelators: PCN TTD-chloride Toxins: Lead Zinc Auto-Abs
79
How is Sideroblastic anemia Tx How does lead cause anemia?
D/c offender PRBC transfusion PO pyridoxine, Vit B6 Impaired heme synthesis, inc rate of hemolysis
80
How is lead poisoning induced anemia Dx How is timing of the poisoning deduced?
Inc free erythrocyte protporphyrin Inc lead Inc Lead, N FEP= past 2-6wks Inc lead, Inc FEP= chronic/ongoing exposure
81
What would be seen on a peripheral blood smear in lead poison anemia? What follow on test is conducted?
Basophil stippling Hypochromic microcytic RBC Prussian Blue stain for Sideroblastic
82
How is lead poisoning induced anemia Tx What are the two DDx for Macrocytic anemias?
1st: Succimer 2nd: Penicillamine Severe: EDTA, crosses BBB (crosses neuro/learning issues in kids) Megaloblastic Non-megaloblastic
83
What are the causes of megaloblastic anemia What are the causes of non-megaloblastic anemia
B12/folate deficient Drug induced ``` BEHEADS Bone marrow d/o ETOH Hypothyroid Emphysema Accelerated erythropoiesis Drug induced Surface area, inc on RBC ```
84
Megaloblastic anemia etiology lies w/in ? cell in hematopoiesis? Define megaloblastic anemia
Erythroblast stage Anemia w/ megalobasts in bone marrow
85
What peripheral smear result is indicative of megaloblastic anemia Define this result
Hyper segmented neutrophils- indicates megaloblasts in marrow Neurtophil hypersegmentation- presence of neutrophil w/ 6+ nuclei lobes or, +3% neutrophils w/ 5 lobes
86
Why is Cobalamin deficiency rare How is B12 digested and absorbed
B12 in all foods of animal origin ``` Hcl stims release of pepsin Pepsin separates B12 from food Parietal cells release IFactor B12 binds to IF, absorbed in terminal ileum Transported to, stored in liver ```
87
What is the carrier protein of B12 How much B12 is normally stored in the liver
Transcobalamin II (WBCs) 2-5K mcg
88
How much B12 is lost per day? How long does it take for a deficiency to develop
3-5mcg/day >3yrs after absorption stops
89
Why is B12 so important to the body A lack of the vitamin will be seen as ?
Cofactor in DNA synthesis- converts m-CoA to s-CoA in mitochondria Methymalonic acid (MMA)- most sensitive test for B12 deficiency at cellular level Macrocytosis Hypersegmented neutrophils- asynchronous cell, nuclear and cytoplasm maturation
90
Lack of B12 affects w/ DNA synthesis how? What are 3 causes of dec IF leading to B12 deficiency
Mitosis- cells acquire abnormal shapes w/ large/immature nuclei surrounded by more mature cytoplasm Atrophic gastritis Gastric bypass Pernicious anemia, AutoImm
91
Prolonged use of ? meds can lead to B12 deficiency What are some miscellaneous causes?
``` Metformin >4mon PPIs >12mon H2 blockers >12mon Colchicine Meomycin Sulfate ``` ``` NO abuse (+spine degeneration) Imerslund-Grasbeck Syndrome ```
92
What is the last Sx to present in Initial, Mid and first Sx to show in Late B12 deficiency B12 is important for the production of ? in the NS
Peripheral neuropathy- hand parasthesia Ataxia Dementia Myelin
93
What are the 5 P's of B12 deficiency What is the autoimmune form of Vit B12 deficiency
``` Pancytopenia Peripheral neuropathy Papillary atrophy of tongue Post spine neuropathy Pyramidal tract signs ``` Pernicious Anemia- atrophy of parietal cells w/ strong predominance in Caucasians
94
Slide 36 How much B12 is used to Tx deficiency?
Deck #3 ASx: PO 1000mcg/day IM 1000mcg/mon Sxs and urgency: Cyanocobalamin- 1000mcg every other day x 2wks, then monthly or, Hydroxocobalamin- every 2-3mon
95
When Tx B12 deficiency, what route of administration is not recommended? How is pernicious anemia Tx
Nasal Parenteral B12 for life 1mg IM/Deep SC initial q4wks, then 1000mcg/mon
96
If PT has Lichtheim Dz and has deficient folate and B2, which is replaced first? How long does it take for improvement to be seen and where is it seen?
B12 to prevent spinal degeneration Erythropoiesis- norm in 2 days Hypersegmented neutrophils disappear in 14 days Neuro S/Sx reverse <6mon old
97
Where is folate found and where is it absorbed in the body? What process can destroy folic acid
Fruit Veggies Animal protein Proximal small intestine Cooking
98
How much of a stored reserve does the body have? What meds can induce folate deficient macrocytic anemia
2-3mon, 5000mcg Biguanide OCP Diuretic Anti-parasitic convuls biotic HIV
99
How is folate deficient anemia Tx If Folate Tx leads to PTs developing neuro Sxs, what's next
PO 1000mcg PO x 1-4mon Add B12 after folic acid Tx
100
What is a derm Sx associated w/ megaloblastic anemia? What are the typical/classic findings of megaloblastic anemia that are absent in macro non-megalo anemia
Premature graying of hair Oval macroctye Inc RDW
101
If PT has non-megaloblastic macrocytosis that is unexplained, what is the next step? What is the lab result of a marrow sample from a PT w/ non-megalo macro anemia
Marrow exam and cytogenic analysis to r/o myelodysplasia Proerythroblasts- megaloblast precurors
102
What are the causes of Non-Megalo Macro Anemia What were the two medications pointed out that can cause this?
Hypothryoid Inc RBC membrane surface ara= inc cholesterol/lipids deposition Benzenes HIV anti-virals
103
# Define Myelodysplastic Syndromes 80% of these cases are caused by ?
Marrow d/o w/ ineffective erythropoieses/dysplasia due to mutation in hematopoietic stem cell Idiopathic
104
What are the secondary causes of MDS This Dx is suspected if w/ ? RBC indicies result
Benzen meds Chemo/Rad therapy MCV ? 105
105
Third of MDS PTs develop ? How does MDS present in clinic
AML- pre-leukemia syndrome Pallor Anemia w/ severe fatigue Neutropenia
106
What lab results are going to indicate PT has MDS What are two key components during the MDS work up
Inc RDW Oval macrocytosis Peripheral smear Unilateral marrow biopsy
107
What are 3 condition presentations that mimic MDS and need to be ruled out? How is MDS Tx
HIB B12/Folate deficiency ``` Symptomatic RBC Transfusion if Hgb <7 Platelete transfusion if: <50K before surgery <20K and bleeding <10K and ASx Chemo Marrow transplant ```
108
PTs w/ MDS can die as quickly as ? due to infections What are the DDx for Normocytic Anemia
4hrs ``` Chronic Dz: inflammation CLDz Hemolytic Apalstic CKDz Endocrinopathies Acute blood loss ```
109
What are the 3 types of hemolytic anemia that can cause normocytic anemia What is the 2nd MC anemia and how long does it take to develop
Hereditary spherocytosis G6PD Sickle Cell Dz Normocytic over 1-2mon
110
Why/how does chronic inflammation lead to anemia What is the only difference in lab results between microcytic and normocytic anemia?
Dec Fe availability in marrow dec erythropoiesis and RBC survival Inc hepcidin= dec absorption or release from cells MCV size: Micro <80 Normo 80-100
111
What is the role of the IL-6 protein in Normocytic anemia What is the sole etiology of normocytic anemia
Released by macrophages due to inflammation Stims hepatocytes to release hepcidin Inflammation
112
How do endocrinopathies lead to normocytic anemia How is anemia of chronic Dz Tx
Metabolic imbalance influences EPO and thyoid stimulation of erythroid colonies Fe supplements if deficiency exists Transfusion if severe ESA in PTs w/ Sxs and Hgb <10 (CA w/ chemo, GFR <30, HIV Tx w/ myelosuppression)
113
In PTs w/ CKD and anemia, what is the goal of therapy? Define Aplastic Anemia
Hgb of 11, HCt >33% Hypoproliferative anemia characterized by marrow failure resulting in pancytopenia
114
What are the two types of aplastic anemia This is an anticipated result during ? Tx course
Primary: IgG auto-Ab against marrow stem cells Secondary: acquired Ca Tx
115
What drug reactions can cause Aplastic Anemia What are 3 toxins that can cause this?
``` Anti-seizure ABX Arsenics NSAIDS Anti-thyroid Gold ``` Benzene Solvents Glue
116
What viral infections can cause aplastic anemia What is the bimodal distribution this condition presents in w/ ? Sxs?
EBV, HIV, HSV Sero - non-A-G Hepatitis 15-25y/o and >60y/o S: Pallor Purpura Petechiae Sxs: Thrombocytopenia Anemia Neutropenia
117
What PE findings should NOT be present in aplastic anemia What is the hallmark?
Hepato/Spleenomegaly or Lymphadenopathy Pancytopenia
118
How is aplastic Tx What are 3 intrinsic etiologies that can cause hemolytic anemias
Young w/ functional status: marrow transplant Older: ImmSupp Membrane defect: hereditary spherocytosis Oxidation: G6PD Hemoglobinopathies: Sickle, Thalassemia
119
When would Bite or Helmet cells be seen on a smear? What are 3 infections that can cause this issue
Normocytic Hemolytic anemia Malaria Clostridium Borrelia
120
lab results of Normo Hemolytic What is the MC intrinsic hemolytic anemia
Lect 4 Slide 46 Hereditary spherocytosis- abnormal spectrin/actin that provide structure to RBC membrane causing rupture and trapped in spleen
121
What type of genetic passage occurs w/ hereditary spherocytosis Three unique lab findings that will be seen
Autosoma dominant- only one dominant trait needed for Dz Inc MCHC Inc osmotic fragility test Neg Coombs test
122
How is Normocytic Anemia Tx What is the MC enzymatic d/o of RBCs
Support RBC transfusion if <7 Folic supplement Splenectomy G6PD- leads to hemolysis when RBCs undergo oxidative stress
123
G6PD is passed along ? and is common in ? PT populations Oxidative stress in the body can create radicals that damage ?
X-linked recessive SE Asia Mediterranean AfAm Severe deficiency in Mediterranean may have chronic hemolytic anemia DNA Lipids Proteins
124
What lab result/cell type is pathognemonic for G6PD? Define Favism and who expresses it
Heinz body- oxidized Hgb damages RBC membranes, forms bite cells during spleen passage. Crystal violet stains reveal Hemolytic response to fava beans; all PTs w/ favism have G6PD, not all G6PD PTs have favism
125
What 3 drug classes can induce oxidative stress and a G6PD episode When is splenectomy a Tx consideration
Taking these @ elevation: Antimalarial ABX: Dapsone High dose ASA Severe episodic/chronic hemolysis
126
What type of genetic issue is Sickle Cell How is Sickle Cell anemia Dx
Autosomal recessive: Point mutation on 6th AA of B-globin gene First year of life when Hgb F drops and reveals the Hgb S
127
What influences the rate of sickling blood cells? PTs w/ sickle cell trait may experience what 2 adverse outcomes due to the mutation
Intracorpuscular concentration of deoxygenated Hgb(dehydration, hypoxia, acidosis, altitudes Sudden cardiac death Rhabdo
128
Chronic Sickle cell anemia can present w/ ? Ortho finding? What underlying issues usually lead to death in adults?
Ulceration over lower anterior tibia Pulm and Renal failure
129
What causes an aplastic crisis in Sickle Cell PTs What are the MC sites for vaso-occlusice crisis in Sickle PTs?
Infection or Folate deficiency Back- spine/kidney Long bones Lungs
130
# Define Acute Chest Syndrome Sickle PTs w/ osteomyelitis acquire this from ? MC organism
Angina Hypoxemia Pulm infiltrates on CXR Salmonellae
131
What are the hallmarks of hyposlenism seen on peripheral smears of Sickle Cell PT blood How is Sickle Cell Anemia Tx
Howell-Jolly bodies Target cells PO Folate 1mg/day Omega 3- reduce occlusive episodes ACEI- PTs w/ microalbuminuria Hydroxyurea and L-glutamine for painful crisis
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What are the 5 times Sickle Cell anemia PTs will get an exchange transfusion When are they admitted?
``` Severe occlusive crisis Intractable pain crisis Acute chest syndrome Priapism Stroke ``` Acute chest syndrome Aplastic crisis Unresponsive pain crisis
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What is a definitive Tx for Sickle if done early What happens during acute blood loss
Stem Cell Transplant in kids prior to organ damage Baroreceptor stim release of vasopressin, extra to intracellular fluid shift Hemodilution changed hypovolemia to anemia Marrow inc RTC in 5-7 days
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# Define Massive Transfusion Doing these procedures can cause ? metabolic shifts/issues
>50% of PTs blood volume within 24hrs >10 units of PRBCs in 24hrs FFP ``` Metabolic acidosis Hypo Ca/thermia HyperK Coagulopathy Dilutional thrombocytopenia ```
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What is the MC transfusion reaction How can the chances of this reaction be reduced How is it Tx
Leukoagglutinin- reaction to Abs on WBCs; febrile non-hemolytic transfusion reaction, F/c w/in 12hrs of receipt Leukopore filters- filters donor WBCs out prior to storage Diphenhydramine Acetaminophen CCS
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What causes acute hemolytic transfusion reactions What is the MC cause of this reaction When do the most severe reactions occur
Mismatched ABO/Rh blood is given, causes massive intravascular hemolysis Clinical error Surgery under general anesthesia
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What are the S/Sxs of AHTR What are the S/Sxs in PTs under anesthesia
Fever/rigor HOTN HA/back pain Oliguria, generalized bleeding
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How long for DHTR to present What unique Ab is seen in this type of reaction What is the name of the reaction
5-10 days Alloantibody- Ab produced against foreign tissues Anamnestic responses- enhanced immune response
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Blood product contamination is usually by ? type of microbe infecting ? blood cell How would PTs present if the not MC microbes infected their product
Gram neg Platelets, can't be refrigerated Gram Pos= Fever, Bacteremia Rarely sepsis
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How does kidney damage occur from blood product contamination? Fresh Whole Blood
Gram neg microbes causes transfusion endotoxins RBCs Plasma WBCs and platelets Kept for <24hrs Use: cardiac surgery/massive hemorrhage, +10 units in 24hrs Exact blood match- MUST
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PRBC Define CMV negative, leukocyte reduced filtered RBC
MC transfused product 1 unit inc Hgb x 1g and Hct x 4% 1 unit= 300ml vol inc Dec leukocyte blood to prevent transmission of CMV, causes post-transplant I/G/H Dz
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When are CMV neg, WBC reduced RBCs used How long can frozen RBCs be stored?
Hx febrile non-hemolytic transfusion reactions Cardiovascular surgery Transplant candidates Chronically transfused PTs 10yrs- expensive, reserved for rare blood types
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# Define Irradiated RBCs What part of blood contains coagulation factors
Completely removes WBCs Used for ImmComp PTs Plasma
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When is FFP used 1 unit of apheresis platelets= + units of whole blood platelets
PTs w/ active bleeding or at high risk for bleeds 6 units 1 unit= inc platelets by 5-10K w/in 1hr
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Define Platelet refractoriness
Platelets don't inc w/in 1hrs after transfusion Non-Imm MC cause: sepsis Imm: Abs against HLA Ags are primary cause
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What is the use of Freeze Dried Plasma Who is universal donor and recipient
Only avail for SF, sterile water reconstitute, ready in minutes D: O- R: AB+
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Erythroblastosis Fetalis How is it prevented
Immune Hydrops or Rh Dz of Pregnancy Mother Imm system destroys Rh+ cells in baby ``` ImmGlob 300mcg @ 28wks W/in 72hrs of terminated pregnancy Post-any vaginal bleed Amniocentesis or chorionic villi sample Avoid manual removal of placenta ```