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IPAP > Hematology: Block 1 > Flashcards

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
Q

Define WBC w/ Diff

What is the MC WBC

A

Number of WBCs and %s of each types

Neutrophils- granules are pale lilac, pus

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26
Q

If neutrophils are elevated, what does this suggest?

What other stressors can cause them to be elevated

A

Bacterial infection

Burn Stress Inflammation

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27
Q

Eosinophil granules are ? color and are elevated ?

Basophil granules are ? color and are elvated ?

A

Red/orange
Allergic reaction Prasite Autoimmune

Purple/blue
Allergy Leukemia/Ca Hypothyroid

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28
Q

Lymphocytes are ? color and present ?

Lymphocytes produce ? two types of immunity

A

Sky blue rim around nucleus
Infection Leukemia Mono

Cell Mediated: cytokines
Humoral: Abs production

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29
Q

What are the 3 cells included under lymphocytes?

What are the 5 types of cytokines released during CMImmunity

A

B cells- destroy bacteria, humoral activity
T cells- destroy virus, fungi, transplant/Ca cells
NKSc- destroy infectious/malignant cells

IFN Chemokine Lymphokines IL TNF

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30
Q

How do monocytes appear?

When are they elevated

A

Horse shoe shaped, blue/gray nucleus

Viral/fungal infection
TB
Leukemia (some)
Chronic Dzs

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31
Q

What lab technique offers the best method to view and identify blood cells and components

CBCs measure ? 3 things

A

Peripheral smear

RBC WBC Platelet

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32
Q

Define the following prefixes:
A/An

Aniso

Pan

Poly

Poikilo

A

W/out

Unequal

All

Many/variable

Irregular shaped

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33
Q

Define the following suffixes:
-cytopenia

  • cytosis
  • emia
  • osis
  • penia
A

Absent/deficient cells

Cells

Condition/Dz of blood

Process/condition

Lack/deficiency

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34
Q

Define Erythrocytosis and the two types

What type would be seen if PT was dehydrated

A

Inc of RBCs, > 5.5
Absolute: high RBC mass
Relative: normal RBC mass, low plasma

Relative

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35
Q

What conditions can cause erythrocytosis?

Define erythrocytopenia

A

HD DR CPC
Hypoxia Dehydrate Drugs Renal dz Congenital Polycythemia vera Ca

Dec of RBCs <3.5

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36
Q

What conditions can cause erythrocytopenia

What conditions can cause leukocytosis

A 
Ha MR H
Hemolytic anemia 
Marrow failure
Renal Dz 
Hemorrhage

I LICS
Infection Leukemia
Inflammation CCS Stress

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37
Q

What conditions can cause Leukopenia

What can cause thrombocytosis

A

B12/Folate deficient
Infxn
Aplastic/AutoImm
Spleen over stimulation

Infection/Tissue damage
Chronic inflammation/infxn
Malignancy

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38
Q

What can cause thrombocytopenia

A

Dec production: marrow failure, malnutrition

Inc destruction: AutoImm, transfusion reaction

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39
Q

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

A

M: Hct <41%, Hgb <13.5
W: Hct <36%, Hgb <12

Fe deficiency
Bleeding

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40
Q

How is anemia classified

What labs are ordered for the work up process?

A

Pathophysiologic process:
Dec production
Inc destruction/loss

Retic count
CBC
Peripheral smear
CMP
Marrow aspirate/biopsy
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41
Q

When working up a case of suspected anemias, when is a marrow sample acquired?

What are the 3 common yet severe Sxs of anemia

A

Lab eval fails to ID source

Syncope Angina MI

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42
Q

How do PTs present on PE if they have anemia

What are severe Sxs of anemia that would be seen on PE?

A

Dyspnea Tachy Fatigued Palpitations

Smooth tongue
Brittle/spoon nails

Pica- craving non-nutritional materials >1mon

Cheilosis- B12 deficient causing cracked lips

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43
Q

MCV results indicates what part of an anemia?

What are the possible etiologies of microcytic anemia, MCV <80

A

Classificaiton of an anemia

Thalassemia
Anemia, chronic Dz
Iron deficient**
Lead poison
Sideroblastic
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44
Q

What are the two functions of Ferritin

What lab result measures the blood’s capacity to bind Fe w/ transferrin

A

Storage protein for Fe in cells
Fe carrier in serum

Total Iron Binding Capacity TIBC

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45
Q

What lab result would indicate the amount of stored Fe in the body

What hormone regulated Fe storage

A

Serum ferritin

Hepcidin- hormones produced by liver, inc during inflammation/infections (dec absorption)

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46
Q

Function of Erythroferrone

Function of Ferroportin

A

Inhibits hepcidin synthesis

Fe transporter across intestinal lumen

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47
Q

Importance of Hemosiderin

How much Fe is balanced in day to day homeostasis

A

Insoluble form of Fe ONLY found in cells
Formed when body has excess Fe

1-2mg in/out

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48
Q

Where is Fe absorbed in the GI system

Where/how is it distributed through out the body?

A

Duodenal enterocytes

3-4gm between:
RBCs 1800-2000mg
Liver parenchyma 1000mg
Spleen 600mg
Marrow, reticuloendothelial macrophages 300mg
Muscles 300mg
Plasma transferrin 3mg
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49
Q

What lab result is not Dx of Fe Deficient Anemia

Under normal conditions, how much of transferring is occupied by Fe

A

Low serum Fe

1/3

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50
Q

What are the 7 parts of an Fe panel during an anemia work up

A

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

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51
Q

What part of the Anemia Fe Panel is used during investigating Fe Deficient Anemia

Why is this result used?

A

sTfR

sTfR not affected by acute phase reactions

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52
Q

What are the 3 mechanisms of developing FeDA

What is the most important part of FeDA management

A

Inc loss
Dec intake/absorption

Identifying the cause

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53
Q

What is the first consideration in male >50y/o OR post-menopause female w/ newly onset FeDA

How is this consideration investigated

A

Occult GI Ca

EGD, Colonoscopy

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54
Q

What are the 4 stages in the development of FeDA?

How is mild/mod FeDA Tx

A

1: dec ferritin
2: hypochromic RBCs
3: Hgb dec
4: MCV/morphology dec

1st line: PO replacement
Ferrous Sulfate 325mg

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55
Q

How are severe cases of FeDA Tx

When using Ferrous Sulfate for FeDA Tx, what drug interactions have to be avoided?

A

IV Fe therapy w/ IM/Hematology provider

Levothyroxin- taken 4hrs apart

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56
Q

What is considered an appropriate response to Fe Tx for FeDA

How long is Tx continued

A

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

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57
Q

Micro Anemia Anemia of Chronic Dz is characterized by ?

How is it Tx

A

Etiology
Pathophysiology

Tx underlying cause
RBC transfusion
Recombinant EPO

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58
Q

What extra lab result is needed in order to accurately Dx the type of thalassemia

Define Thalassemia

A

Hgb Electrophoresis

Hereditary defected synthesis of globin chains= dec Hgb synthesis

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59
Q

What are the different types of thalassemias

This condition is usually seen in ? PT populations w/ ? finding

A

A: gene deletion, dec A/inc of unstable B chains
B: point mutation

Mediterranean African Asian-
Microcytosis

60
Q

What are the hallmark lab results of Microcytic Anemia Thalassemia

Based off of these findings, this type of anemia is AKA ?

A 
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
Q

Normal adults have ? type of Hgb

What is a variant that can be seen?

A

A2B2

A2D2

62
Q

What is Hgb F made of

What types of Hgb cross and switch roles at birth

A

A2G2

Gamma- 2nd highest in vitro
B- 2nd highest in adulthood

63
Q

What is the make up of Hgb H

What is the make up of Hgb Bart’s

A

4B

4G

64
Q

What is the Hgb make up of Hydrops Fetalis

After FeDA is excluded, what is the next Dx due to clinical suspicion

A

0A

MAT

65
Q

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?

A

Transfusions- Fe overload and high RBC turn over

Microcytes
Acanthocytes- Greek, thorn
Codocytes- target cells

66
Q

Define Thalassemia Trait

Define Thalassemia Intermedia

A

Lab features w/ no clinical impact

Occasional transfusion required, moderate clinical impact

67
Q

Define Thalassemia Major

What is the make up of MAT silent carrier

A

Life threatening, transfusion dependent

AA/A-

68
Q

What are the two genotypes of Alpha Thalassemia Minor and are on what chromosome?

Alpha thalassemia intermedia is AKA ? and has ? globulin make up

A

Homozygous: AA/–
Heterozygous: A-/A-
Chrom 16

Hgb H Dz: A-/–

69
Q

? form of A-thalassemia affects Asians or Blacks

How are PTs w/ Hgb H Dz managed

A

Asian: Hetero A-/A-
Black: Homo A-/–

Daily folate
Avoid supplemental Fe
Possible transfusions during infections/stress

70
Q

PTs w/ MAT Hgb H Dz need to avoid what drug classes?

A 
Antimalarials
Sulfa 
Antibacterials
TB
Analgesics
Fe supplements
Pyrimethamine
Methylene blue
Napthalene (moth balls)
Fava bean
71
Q

What is the underlying issue in MAT B-Thalassemia

What can the missing Alpha chains be substituted with here?

A

Dec B production causes excessive A chains

W/ D chains= HbA2
W/ G chains= HbF

72
Q

What happens when there is an excess in A chains during MAT B-T

MAT B-T is more common in PTs w/ ? genotype

A

Unstable and incapable for forming tetramers, cause RBC membrane damage

Homozygous for impaired B-globin synthesis

73
Q

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

A

Minor: B+/B
Intermedia: B+/B+
Major: B+/B+, RBCs have nucleus (Cooley’s Anemia)

Basophilic stippling

74
Q

What do the electrophoresis results look like when testing for thalassemias?

What are the only two heterozygous B-globin B-thalassemias

A

A: normal
Hgb H: inc Hgb H
B: +Gamma= Hgb F
- Delta= Hgb A2

Minor
Intermedia

75
Q

How are cases of mild A or B thalassemia Tx

How are severe cases Tx

A

None, ID’d to avoid repeat evaluation or Fe deficiency

B-I/Major: spleenectomy
Marrow transplant- TxOC for major

76
Q

What is an adverse outcome that can occur when Tx severe thalassemias w/ too many transfusions?

What causes sideroblastic anemia?

A

Iatrogenic Fe overload syndrome

Mitochondria mishandle serum Fe= no Fe in Hgb by RBC precursors

77
Q

What happens to RBCs that have too much Fe in Sideroblast anemia

What does the presence of basophilic stippling represent in a Sieroblastic anemia?

A

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
Q

What are the causes of Sideroblastic anemia

A

Acuqired: MC, drugs/toxins
Inherited

Ethanol INH Cycloserine Chloramphenicol Busulfan

Copper chelators:
PCN
TTD-chloride

Toxins:
Lead Zinc Auto-Abs

79
Q

How is Sideroblastic anemia Tx

How does lead cause anemia?

A

D/c offender
PRBC transfusion
PO pyridoxine, Vit B6

Impaired heme synthesis, inc rate of hemolysis

80
Q

How is lead poisoning induced anemia Dx

How is timing of the poisoning deduced?

A

Inc free erythrocyte protporphyrin
Inc lead

Inc Lead, N FEP= past 2-6wks
Inc lead, Inc FEP= chronic/ongoing exposure

81
Q

What would be seen on a peripheral blood smear in lead poison anemia?

What follow on test is conducted?

A

Basophil stippling
Hypochromic microcytic RBC

Prussian Blue stain for Sideroblastic

82
Q

How is lead poisoning induced anemia Tx

What are the two DDx for Macrocytic anemias?

A

1st: Succimer
2nd: Penicillamine
Severe: EDTA, crosses BBB (crosses neuro/learning issues in kids)

Megaloblastic
Non-megaloblastic

83
Q

What are the causes of megaloblastic anemia

What are the causes of non-megaloblastic anemia

A

B12/folate deficient
Drug induced

BEHEADS
Bone marrow d/o
ETOH
Hypothyroid
Emphysema
Accelerated erythropoiesis
Drug induced
Surface area, inc on RBC
84
Q

Megaloblastic anemia etiology lies w/in ? cell in hematopoiesis?

Define megaloblastic anemia

A

Erythroblast stage

Anemia w/ megalobasts in bone marrow

85
Q

What peripheral smear result is indicative of megaloblastic anemia

Define this result

A

Hyper segmented neutrophils- indicates megaloblasts in marrow

Neurtophil hypersegmentation- presence of neutrophil w/ 6+ nuclei lobes or,
+3% neutrophils w/ 5 lobes

86
Q

Why is Cobalamin deficiency rare

How is B12 digested and absorbed

A

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
Q

What is the carrier protein of B12

How much B12 is normally stored in the liver

A

Transcobalamin II (WBCs)

2-5K mcg

88
Q

How much B12 is lost per day?

How long does it take for a deficiency to develop

A

3-5mcg/day

> 3yrs after absorption stops

89
Q

Why is B12 so important to the body

A lack of the vitamin will be seen as ?

A

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
Q

Lack of B12 affects w/ DNA synthesis how?

What are 3 causes of dec IF leading to B12 deficiency

A

Mitosis- cells acquire abnormal shapes w/ large/immature nuclei surrounded by more mature cytoplasm

Atrophic gastritis
Gastric bypass
Pernicious anemia, AutoImm

91
Q

Prolonged use of ? meds can lead to B12 deficiency

What are some miscellaneous causes?

A 
Metformin >4mon
PPIs >12mon
H2 blockers >12mon
Colchicine
Meomycin Sulfate
NO abuse (+spine degeneration)
Imerslund-Grasbeck Syndrome
92
Q

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

A

Peripheral neuropathy- hand parasthesia
Ataxia
Dementia

Myelin

93
Q

What are the 5 P’s of B12 deficiency

What is the autoimmune form of Vit B12 deficiency

A 
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
Q

Slide 36

How much B12 is used to Tx deficiency?

A

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
Q

When Tx B12 deficiency, what route of administration is not recommended?

How is pernicious anemia Tx

A

Nasal

Parenteral B12 for life
1mg IM/Deep SC initial q4wks, then 1000mcg/mon

96
Q

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?

A

B12 to prevent spinal degeneration

Erythropoiesis- norm in 2 days
Hypersegmented neutrophils disappear in 14 days
Neuro S/Sx reverse <6mon old

97
Q

Where is folate found and where is it absorbed in the body?

What process can destroy folic acid

A

Fruit Veggies Animal protein
Proximal small intestine

Cooking

98
Q

How much of a stored reserve does the body have?

What meds can induce folate deficient macrocytic anemia

A

2-3mon, 5000mcg

Biguanide OCP Diuretic
Anti-parasitic convuls biotic HIV

99
Q

How is folate deficient anemia Tx

If Folate Tx leads to PTs developing neuro Sxs, what’s next

A

PO 1000mcg PO x 1-4mon

Add B12 after folic acid Tx

100
Q

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

A

Premature graying of hair

Oval macroctye
Inc RDW

101
Q

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

A

Marrow exam and cytogenic analysis to r/o myelodysplasia

Proerythroblasts- megaloblast precurors

102
Q

What are the causes of Non-Megalo Macro Anemia

What were the two medications pointed out that can cause this?

A

Hypothryoid
Inc RBC membrane surface ara= inc cholesterol/lipids deposition

Benzenes
HIV anti-virals

103
Q

Define Myelodysplastic Syndromes

80% of these cases are caused by ?

A

Marrow d/o w/ ineffective erythropoieses/dysplasia due to mutation in hematopoietic stem cell

Idiopathic

104
Q

What are the secondary causes of MDS

This Dx is suspected if w/ ? RBC indicies result

A

Benzen meds
Chemo/Rad therapy

MCV ? 105

105
Q

Third of MDS PTs develop ?

How does MDS present in clinic

A

AML- pre-leukemia syndrome

Pallor
Anemia w/ severe fatigue
Neutropenia

106
Q

What lab results are going to indicate PT has MDS

What are two key components during the MDS work up

A

Inc RDW
Oval macrocytosis

Peripheral smear
Unilateral marrow biopsy

107
Q

What are 3 condition presentations that mimic MDS and need to be ruled out?

How is MDS Tx

A

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
Q

PTs w/ MDS can die as quickly as ? due to infections

What are the DDx for Normocytic Anemia

A

4hrs

Chronic Dz: inflammation  
CLDz
Hemolytic
Apalstic
CKDz
Endocrinopathies 
Acute blood loss
109
Q

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

A

Hereditary spherocytosis
G6PD
Sickle Cell Dz

Normocytic over 1-2mon

110
Q

Why/how does chronic inflammation lead to anemia

What is the only difference in lab results between microcytic and normocytic anemia?

A

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
Q

What is the role of the IL-6 protein in Normocytic anemia

What is the sole etiology of normocytic anemia

A

Released by macrophages due to inflammation
Stims hepatocytes to release hepcidin

Inflammation

112
Q

How do endocrinopathies lead to normocytic anemia

How is anemia of chronic Dz Tx

A

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
Q

In PTs w/ CKD and anemia, what is the goal of therapy?

Define Aplastic Anemia

A

Hgb of 11, HCt >33%

Hypoproliferative anemia characterized by marrow failure resulting in pancytopenia

114
Q

What are the two types of aplastic anemia

This is an anticipated result during ? Tx course

A

Primary: IgG auto-Ab against marrow stem cells
Secondary: acquired

Ca Tx

115
Q

What drug reactions can cause Aplastic Anemia

What are 3 toxins that can cause this?

A 
Anti-seizure
ABX
Arsenics
NSAIDS
Anti-thyroid
Gold

Benzene Solvents Glue

116
Q

What viral infections can cause aplastic anemia

What is the bimodal distribution this condition presents in w/ ? Sxs?

A

EBV, HIV, HSV
Sero - non-A-G Hepatitis

15-25y/o and >60y/o
S: Pallor Purpura Petechiae
Sxs: Thrombocytopenia Anemia Neutropenia

117
Q

What PE findings should NOT be present in aplastic anemia

What is the hallmark?

A

Hepato/Spleenomegaly or Lymphadenopathy

Pancytopenia

118
Q

How is aplastic Tx

What are 3 intrinsic etiologies that can cause hemolytic anemias

A

Young w/ functional status: marrow transplant
Older: ImmSupp

Membrane defect: hereditary spherocytosis
Oxidation: G6PD
Hemoglobinopathies: Sickle, Thalassemia

119
Q

When would Bite or Helmet cells be seen on a smear?

What are 3 infections that can cause this issue

A

Normocytic Hemolytic anemia

Malaria Clostridium Borrelia

120
Q

lab results of Normo Hemolytic

What is the MC intrinsic hemolytic anemia

A

Lect 4
Slide 46

Hereditary spherocytosis- abnormal spectrin/actin that provide structure to RBC membrane causing rupture and trapped in spleen

121
Q

What type of genetic passage occurs w/ hereditary spherocytosis

Three unique lab findings that will be seen

A

Autosoma dominant- only one dominant trait needed for Dz

Inc MCHC
Inc osmotic fragility test
Neg Coombs test

122
Q

How is Normocytic Anemia Tx

What is the MC enzymatic d/o of RBCs

A

Support
RBC transfusion if <7
Folic supplement
Splenectomy

G6PD- leads to hemolysis when RBCs undergo oxidative stress

123
Q

G6PD is passed along ? and is common in ? PT populations

Oxidative stress in the body can create radicals that damage ?

A

X-linked recessive
SE Asia Mediterranean AfAm
Severe deficiency in Mediterranean may have chronic hemolytic anemia

DNA Lipids Proteins

124
Q

What lab result/cell type is pathognemonic for G6PD?

Define Favism and who expresses it

A

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
Q

What 3 drug classes can induce oxidative stress and a G6PD episode

When is splenectomy a Tx consideration

A

Taking these @ elevation:
Antimalarial
ABX: Dapsone
High dose ASA

Severe episodic/chronic hemolysis

126
Q

What type of genetic issue is Sickle Cell

How is Sickle Cell anemia Dx

A

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
Q

What influences the rate of sickling blood cells?

PTs w/ sickle cell trait may experience what 2 adverse outcomes due to the mutation

A

Intracorpuscular concentration of deoxygenated Hgb(dehydration, hypoxia, acidosis, altitudes

Sudden cardiac death
Rhabdo

128
Q

Chronic Sickle cell anemia can present w/ ? Ortho finding?

What underlying issues usually lead to death in adults?

A

Ulceration over lower anterior tibia

Pulm and Renal failure

129
Q

What causes an aplastic crisis in Sickle Cell PTs

What are the MC sites for vaso-occlusice crisis in Sickle PTs?

A

Infection or Folate deficiency

Back- spine/kidney
Long bones
Lungs

130
Q

Define Acute Chest Syndrome

Sickle PTs w/ osteomyelitis acquire this from ? MC organism

A

Angina Hypoxemia Pulm infiltrates on CXR

Salmonellae

131
Q

What are the hallmarks of hyposlenism seen on peripheral smears of Sickle Cell PT blood

How is Sickle Cell Anemia Tx

A

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

132
Q

What are the 5 times Sickle Cell anemia PTs will get an exchange transfusion

When are they admitted?

A 
Severe occlusive crisis
Intractable pain crisis
Acute chest syndrome
Priapism
Stroke

Acute chest syndrome
Aplastic crisis
Unresponsive pain crisis

133
Q

What is a definitive Tx for Sickle if done early

What happens during acute blood loss

A

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

134
Q

Define Massive Transfusion

Doing these procedures can cause ? metabolic shifts/issues

A

> 50% of PTs blood volume within 24hrs
10 units of PRBCs in 24hrs
FFP

Metabolic acidosis
Hypo Ca/thermia
HyperK
Coagulopathy
Dilutional thrombocytopenia
135
Q

What is the MC transfusion reaction

How can the chances of this reaction be reduced

How is it Tx

A

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

136
Q

What causes acute hemolytic transfusion reactions

What is the MC cause of this reaction

When do the most severe reactions occur

A

Mismatched ABO/Rh blood is given, causes massive intravascular hemolysis

Clinical error

Surgery under general anesthesia

137
Q

What are the S/Sxs of AHTR

What are the S/Sxs in PTs under anesthesia

A

Fever/rigor
HOTN
HA/back pain

Oliguria, generalized bleeding

138
Q

How long for DHTR to present

What unique Ab is seen in this type of reaction

What is the name of the reaction

A

5-10 days

Alloantibody- Ab produced against foreign tissues

Anamnestic responses- enhanced immune response

139
Q

Blood product contamination is usually by ? type of microbe infecting ? blood cell

How would PTs present if the not MC microbes infected their product

A

Gram neg
Platelets, can’t be refrigerated

Gram Pos=
Fever, Bacteremia
Rarely sepsis

140
Q

How does kidney damage occur from blood product contamination?

Fresh Whole Blood

A

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

141
Q

PRBC

Define CMV negative, leukocyte reduced filtered RBC

A

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

142
Q

When are CMV neg, WBC reduced RBCs used

How long can frozen RBCs be stored?

A

Hx febrile non-hemolytic transfusion reactions
Cardiovascular surgery
Transplant candidates
Chronically transfused PTs

10yrs- expensive, reserved for rare blood types

143
Q

Define Irradiated RBCs

What part of blood contains coagulation factors

A

Completely removes WBCs
Used for ImmComp PTs

Plasma

144
Q

When is FFP used

1 unit of apheresis platelets= + units of whole blood platelets

A

PTs w/ active bleeding or at high risk for bleeds

6 units
1 unit= inc platelets by 5-10K w/in 1hr

145
Q

Define Platelet refractoriness

A

Platelets don’t inc w/in 1hrs after transfusion
Non-Imm MC cause: sepsis
Imm: Abs against HLA Ags are primary cause

146
Q

What is the use of Freeze Dried Plasma

Who is universal donor and recipient

A

Only avail for SF, sterile water reconstitute, ready in minutes

D: O-
R: AB+

147
Q

Erythroblastosis Fetalis

How is it prevented

A

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

IPAP (212 decks)

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