Hematology: Block 1 Flashcards
What are the roles of the three proteins found in plasma?
What are the components of solutes?
Albumin: retain fluids in blood
Globulin: simple proteins; clotting and infections
Fibrinogen: clotting, converted by thrombin
WHEN EV
Waste Hormone E+ Nutrient
Enzyme Vit
All cell sizes are compared to a ‘normal’ RBC size which is ?
Define RBC
6-8um (one millionth of a meter; one thousandth of millimeter)
Biconcave anuclear disc, eliminated by liver/spleen
Define WBC
What are the life spans of the different types of WBCs
Differentiated nucleated motile cell
Typical: few hrs (infxn)
T/B Cell: years
What is the general rule to WBC concentration locations?
Define Platelet
Less in circulation, more in tissues
Biconvex anuclear discoid w/ lens shape if inactivated
How are platelets activated
How long do RBC, WBC and Platelets live
Pass blood vessel w/ disrupted endothelium
Activation causes pseudopodia, adhere to form clots
RBC: 120 days
WBC: hrs to years
Platelet: 5-9 days
Define Hematopoiesis and what are the two forms
Extra medullary poiesis mainly occurs in what 2 areas
Production-maturation of all blood cells
Spleen- 53%
Liver- 25%
What issues can trigger extra medullary poiesis to kick in?
This form of production accounts for ? of all hematopoiesis
BI HALL
Breast Ca Infection HIV Anemia Leukemia Lymphoma
3%
What are the four locations of prenatal blood development
Define Pluripotential Stem Cell
Yolk* Liver Spleen
Bone marrow closer to delivery
Stem cells w/ potential to develop into any tissue/cell in body
What are the 3 properties held by pluripotential cells
Upon replication, they first divide into ?
Proliferation potential
Multiple lineages
Self renewal
Myeloid/Lymphoid Stem Cells
What are the 3 hematopoietic Growth Factors, development site and role
EPO- kidneys; RBC production
TPO- liver; thrombocyte production
Cytokines (CSF, IL)- many; WBC production
Where are Colony Stim Factors and Interleukins released from for WBC production
What are the cell transformations in the line of erythropoiesis
ME My BFS
Macrophage Endothelial Mast B/T cell Fibroblasts Stromal
Potential Myeloid Progenitor Blast (per) RBC
When kidneys detect low O2 and release EPO, it triggers ? cell to continue hematopoiesis
What are the cells in sequence of thromboblast development
Proerythroblasts
Potential Myeloid Progenitor Megakaryocyte Platelet
What is the sequence of thrombocyte pseudopodia formation
Leukopoiesis can develop from what two cells into ? cells
C-TPO + endothelial damage= internalization/pseudopodia
Myeloid: BENM
Lymphoid: B/T/NKC
What is the only cell myeloid stem cells can NOT differentiate into?
RBC has ? many Hbg units and what do those units contain
Lymphocyte
280M units
Each unit= 2A 2B chains
What is the best measurement of blood’s O2 carrying capacity
What part of the RBC contains the pigment
Hgb
Heme w/ Fe
One RBC w/ 300M hgb molecules can hold ? O2 molecules
One Hgb contains _O2, _Fe, and _hem
1 billion
4 4 4
Define RBC count
Define Hct
Total number of RBCs
Percent of blood volume that’s RBCs
Define Hgb
Define RBC Indicies
Amount of Hgb in blood
MCV: average RBC size/volume
Define MCH
Define MCHC
Mean Cell Hgb; average quantity of RBC Hgb
Mean Cell Hgb Concentration; Hgb inside RBC w/ size/vol factored in
Define RDW
A high RDW means theres a greater difference in size/vol, defined as ? and seen ?
Red Cell Distribution Width; estimated range of RBC volume and size
Aniscocytosis- post-RBC transfusion
Define Reticulocyte count
What is this measurement the best indicator of?
Number of immature RBCs in circulation
RBC production
When would a reticulocyte count be high or low?
What is the most important function of the Reticuloendothelial System?
High: anemia
Low: marrow failure
Phagocytosis
What are the two locations the RES functions
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
RBC recycling is dependent up on ? cell in the body
What are the two categories of WBCs
Macrophage
Granular: BEN
Agranular: LM
Define WBC w/ Diff
What is the MC WBC
Number of WBCs and %s of each types
Neutrophils- granules are pale lilac, pus
If neutrophils are elevated, what does this suggest?
What other stressors can cause them to be elevated
Bacterial infection
Burn Stress Inflammation
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
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
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
How do monocytes appear?
When are they elevated
Horse shoe shaped, blue/gray nucleus
Viral/fungal infection
TB
Leukemia (some)
Chronic Dzs
What lab technique offers the best method to view and identify blood cells and components
CBCs measure ? 3 things
Peripheral smear
RBC WBC Platelet
Define the following prefixes:
A/An
Aniso
Pan
Poly
Poikilo
W/out
Unequal
All
Many/variable
Irregular shaped
Define the following suffixes:
-cytopenia
- cytosis
- emia
- osis
- penia
Absent/deficient cells
Cells
Condition/Dz of blood
Process/condition
Lack/deficiency
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
What conditions can cause erythrocytosis?
Define erythrocytopenia
HD DR CPC
Hypoxia Dehydrate Drugs Renal dz Congenital Polycythemia vera Ca
Dec of RBCs <3.5
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
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
What can cause thrombocytopenia
Dec production: marrow failure, malnutrition
Inc destruction: AutoImm, transfusion reaction
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
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
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
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
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
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
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)
Function of Erythroferrone
Function of Ferroportin
Inhibits hepcidin synthesis
Fe transporter across intestinal lumen
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
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
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
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
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
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
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
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
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
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
Micro Anemia Anemia of Chronic Dz is characterized by ?
How is it Tx
Etiology
Pathophysiology
Tx underlying cause
RBC transfusion
Recombinant EPO
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
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
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
Normal adults have ? type of Hgb
What is a variant that can be seen?
A2B2
A2D2
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
What is the make up of Hgb H
What is the make up of Hgb Bart’s
4B
4G
What is the Hgb make up of Hydrops Fetalis
After FeDA is excluded, what is the next Dx due to clinical suspicion
0A
MAT
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
Define Thalassemia Trait
Define Thalassemia Intermedia
Lab features w/ no clinical impact
Occasional transfusion required, moderate clinical impact
Define Thalassemia Major
What is the make up of MAT silent carrier
Life threatening, transfusion dependent
AA/A-
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-/–
? 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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
Megaloblastic anemia etiology lies w/in ? cell in hematopoiesis?
Define megaloblastic anemia
Erythroblast stage
Anemia w/ megalobasts in bone marrow
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
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
What is the carrier protein of B12
How much B12 is normally stored in the liver
Transcobalamin II (WBCs)
2-5K mcg
How much B12 is lost per day?
How long does it take for a deficiency to develop
3-5mcg/day
> 3yrs after absorption stops
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
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
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
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
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
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
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
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
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
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
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
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
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
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
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
What are the secondary causes of MDS
This Dx is suspected if w/ ? RBC indicies result
Benzen meds
Chemo/Rad therapy
MCV ? 105
Third of MDS PTs develop ?
How does MDS present in clinic
AML- pre-leukemia syndrome
Pallor
Anemia w/ severe fatigue
Neutropenia
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
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
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
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
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
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
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)
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
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
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
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
What PE findings should NOT be present in aplastic anemia
What is the hallmark?
Hepato/Spleenomegaly or Lymphadenopathy
Pancytopenia
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
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
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
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
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
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
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
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
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
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
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
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
Define Acute Chest Syndrome
Sickle PTs w/ osteomyelitis acquire this from ? MC organism
Angina Hypoxemia Pulm infiltrates on CXR
Salmonellae
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
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
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
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
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
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
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
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
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
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
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
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
Define Irradiated RBCs
What part of blood contains coagulation factors
Completely removes WBCs
Used for ImmComp PTs
Plasma
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
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
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+
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
