332 Lec. 3 - hematologic systems: alterations and treatments

Hematopoesis definition

how all blood cells are created

controlled by hormone like factors - colony stimulating factors (Cytokines)

erythropoeisis definition

how RBCs are made only

the sequence of red blood cell development

start: progenirator cell

proerythroblast

erythroblast/normoblast

reticulocyte

erythrocyre

at each step HgB increase, nucleas decreases

regulation of red cell development

controlled by feedback loop spun by erythropoietin

erythropoeitin inc. RBC production in response to hypoxia

what does a CBC cover

overall bloodhealth, RBC, WBC, HgB, plateletes

what does a CBC w/ differentials cover

all info from regular CBC plus covering other types of WBC

provides % neutrophils, lymphocytes, monocytes, basophils, eosinophils

purpose of CBC w/ differential

helps diagnose different conditions by analyzing immune response, identifying infxns or allergies

polycythemia

high RBC count

erythrocytopenia

low RBC count

leukopenia

low WBC

leukocytosis

high WBC count

thrombocytopenia

low platelets

thrombocytosis

high plateletes

pancytopenia

reduciton or absence of all 3 blood cell types

what are segmented neutrophils

mature neutrophils

high lvls indicate a bacterial infxn or inflammation in the body

low lvls indicate bone marrow disorders or severe infxn

what are banded neutrophils

immature neutrophils

higher band count (left shit) indicates acute inflammation or infxn

decreased band count indicates bone marrow disorders of severe infxn

Mean corpuscular volume (MCV) meaning

average size of RBC

microcytic, normocytic, macrocytic

Mean corpuscular Hemoglobin (MCH)

average weight of hemoglobin

mean corpuscular hemoglobin concentration (MCHC)

average % of HGB in a RBC

hypochromic or normochromic

Red Blood Cell dirstribution width (RDW)

variation in size among RBCs

types of anemias - types of macrocytic normochromic anemia (megaloblastic anemia)

pernicious anemia

folate deficiencet anemia

types of anemias - types microcytic hypochromic anemia

iron deficiency anemia

types of anemias - types of normocytic normochromic anemia

aplastic anemia

hemolytic anemia

anemia of chronic dz - inflammation anemia

post hemmorrhagic anemia (acute or chronic) - blood loss anemia

macrocytic normochromic anemias (megaloblastic anemias) identifiers

inc. MCV, MCH, RDW, normal MCHC

abnormally large stem cells (megaloblasts) produce abnormally large RBCs (macrocytic)

ineffective DNA synthesis - Vitamin B12, follic acid deficiencey

hemoglobin (MCHC) count normal in RBC (normachromic)

eryptosis dec. RBC count

macrocytic-normochromic anemia - folate deficiencey anemia - manifestations

cheilosis - sores/inflammation of mouth corners

stomatits - sores in mouth

diarrhea

macrocytic-normochromic anemia - folate deficiencey anemia - treatment

supplementation with improvement occuring after 1-2 weeks

macrocytic-normochromic anemia - folate deficiencey anemia - causes

deficient in dietary follic acid

small intestinal malabsorption

limited liver stroage

can occur with pernicious anemia

macrocytic-normochromic anemia - pernicous anemia - manifestations

vitamin B12 deficiency

nervous system involvment

irreversible myelin degen

parasthesia

gait disturbances

spastic

neurocognitive disorders

fatigue

stomatitis

macrocytic-normochromic anemia - pernicious anemia - how is the absence of intrinsic factor related

secreted by the parietal cells it is the glycoprotein that is required for absorption of B12, without this glycoprotien absorption doesnt occur causing B12 deficiency

causes: congenital (at birth), gastric bypass, chronic gastritis

macrocytic-normochromic anemia - pernicious anemia - treatment

untreated = death by high output heart fail

to treat:

replace vitamin B12 (takes 5-6 weeks to correct) w/ subQ injections that are continuous over a lifetime

microcytic hypochromic anemia (iron deficient)

dec. MCV, MCH, MCHC, inc. RDW

most common type of anemia

microcytic hypochromic anemia (iron deficient) - causes

lack of intake

inability to recycle lost ____

inability to metabolize ____

increased metabolic demand (preganant, high endurance athletic training)

chronic blood loss

microcytic hypochromic anemia (iron deficient) - signs and symptoms

fatigue

SOB, palpitations, tachycardia

pallor

activity intolerance

difficulty swallowing

formation of inflammatory cells over esophagus

koilonychia (spoon nails)

normocytic normochromic anemia

dec RBC, normal MCV, MCh, MCHC

types of normocycitic normochromic anemia - aplastic anemia

hematopoietic failure or bone marrow aphasia

reduced production of matured cells

pancytopenia production or absence of all 3 blood cell types

causes: stem cell deficiency, AE from meds, autoimmune, CKD consequences

symptoms: Classic CV adn pulmonary manifestations

types of normocycitic normochromic anemia - anemia of chronic dz

results from dec. erythropoietin and imparied iron utilization in chronic dz and inflammation

dec erythrocyte life span

suppressed production of erythropoietin

ineffective bone marrow response

altered iron metabolism in macrophages

common in hospitalized pts

treatement aimed at underlying disorder

if infxn/inflammation symptoms not found screen for malignancies

erythropoiesis - epoetin alfa

types of normocycitic normochromic anemia - post hemorrhagic anemia

acute - traumas, motor vehicle accidents

chronic - GI bleed gynecological disturbances

treatment - stop the bleeding

types of normocycitic normochromic anemia -hemolytic anemia

premature accelerated destruction of erythrocytes

can be hereditary or acquired

caused by G6PD deficiencey, sickle cell dz, infxn, DIC, hemolytic blood transfusion rxn, iatrogenic blood transfusion hemolysis, prosthetic cardiac valves

Iron supplementation pharmacology - Ferrous sulfate (Slow Fe, Ferrousul)

NC:

take with plenty of water

eggs, milk, dairy products, caffine, spinach, fiber, antacids = dec. absorption

orange juice = inc. absorption

take w/ snack/meal = dec. GI upset, but dec. absorption

sit upright for 30 mins after to dec. GI upset

AE:

black tarry stool, ABD pain, Nausea, vomitting, diarrhea, constipation

iron supplementation pharmacology - iron sucrose (Venofer)

preffered for iron deficiencey and renal dz

NC:

rapid admin inc. risk of Hypotension

<200 mg diluted over 15 min

300 mg diluted over 1.5 hrs

400 mg diluted over 2.5 hrs

500 mg diluted over 3.5-4 hrs

may be given w/ hemodialysis

less incidence of anaphylaxis

Iron dextran (Infed)

less used than iron sucrose

BBW:

anaphylaxis like rxn

appropriate use

NC:

orthostatichypotnsn w/ rapid admin

befor full admin give test dose to see possible anaphylaxis.

Blood transfusions

occur when HgB <7g/dl

depends on age, illness, risk factors and surgery performed

acute massive blood loss replaced with whole transfusion

anemias treated with RBC concentrates w/ needed components

Having D antigen = Rh+ positive type

No Dantigen = Rh- negative type

blood tranfusions - tranfusion rxns

sensations of heat along IV line

flushing of the face

urticaria, HA, pain in lumbar area

chills, fever, constricting pain in chest

cramping pain in abd

NV

tachycardia, hypotn, dyspnea

Blood products - what do they do ?

improve tissue oxygenation

inc. oncotic pressure

plasma expansion

blood products - what should you assess before giving

history of transfusions, rxns and hemotolgy issues

possibility of volume overload

consent for transfusion of products

cross math - making sure blood is compatible to reviever

blood products - implementation - PRBC (packed RBC)

admin only w/ normal saline

inc HCT and dec plasma

for hypoxia, bleeding (loss of 25% blood volume, HGB <8)

Blood products - implementation - FFP (fresh frozen plasma)/Cryopercipetates/plasma protein fraction(PFF)

admin with normal saline

adjunctive therapy (support therapy for primary therpay being used already)

clotting factor to reverse bleeding, antidote to anticoagulants and thrombolytics

Blood products - implementation - platelets

used to replace lost platelets

used in slow bleeding events

polycythemia definition

an inc concentration of HgB in the blood through an inc in RBCs

polycythemia types - primary polycythemia (polcythemia vera)

causes: genetic mutations, EX: JAK2 mutation

symptoms: dizzy, HA, redness of skin, fatigue, bluish skin tone

treatment options: phlebotomy, meds(hydroxyurea), manage underlying condition

types of poycythemia - Secondary polycythemia

cause: low O2, kidney dz, hormone replacement therapy, tumors

symptoms: dizzy, HA, redness of skin, bluish skin tone, fatigue

treatments: phlebotomy, meds(hydroxyurea), manage underlying condition

consumptive thrombohemmorhagic disorder - dissemenated intravascular coagulation

the widespread activation of coagulation

causes the consumption of platelets and clotting factors

medium, small or microobstructions

bloocks blood flow to organs causing single of multiple organ failure

errouneous bleeding (irregular uteran bleeding)

severity depends on: the intensity of the stimulus

ex: spesis (gram negative bacteria), systematic inflammatory response to trauma, solid tumors, preganacy complications, hemolytic blood tranfusions

dissemenated intravascular coagulation - pathway

1. triggering event - sepsis, trauma, cardiac arresst, production of conception, hemolytic transfusion

2. endothelial damage - inflammation and platelete aggregation

3. clotting cascade activation

4. microvascular thrombosi

5. vascular occlusion - ischemia, PLT consumption, clotting factors consumption, bleeding

Heparin

deactivates clotting factors 2,10,11 preventing fibrinogen turning into fibrin

prolongs intrinsic pathway: 4-6hrs

affects are immediate and short lived

available in multiple strengths - 10u/1mL or 40,000u/1mL - requires 2 RN check

associated w/ med errors

prophylactic = subQ form = no need to monitor aPTT

complications with heparin therapy - Heparin induced thrombocytopenia (HIT2)

immune sys mediated drug response - binds to proteins on inactivated plateletes forming complex

platelets are destroyed w/ 50% reduction (type 2) - you need to stop infusion

paradoxical thrombus formation occurs and is fatal (5-15%)

complications with heparin therapy - Heparin induced thrombocytopenia (HIT2) s/sx

epistaxis, hematuria, petechia, melenea

complications with heparin therapy - Heparin induced thrombocytopenia (HIT2) treatment

stop the infusion as it has a short half life

argatroban (direct thrombin inhibitor) which has immediate onset

protamine sulfate (reversal agent for active bleeding)

clot prevention vs clot busters

(Clot prevention)

antiplateletes - aspirin, clopidogrel

anticoagulants - heparin (PTT), warfrin (Pt, INR), antifactor Xa or Rivoroxaban

(Clot busters)

thrombolytics - alteplase

clot prevention vs clot busters - labs

PT (prothrombin time) - measures how long it takes for blood to clot, used to monitor warfrin therapy \

PTT/aPTT (partial thromboplastin time) - assess the intrinsic pathway of coagulation, used to monitor heprin therapy, diagnosis hemophilia, clottign disorders

INR (international normalized ration) - assess how well blood is clotting - evaluates affects of antiplateletes (warfrin)

normal range 0.8-1.1

target range: 2-3.5

>3.5 risk for bleeding

Coagulation Labs - aPTT and PTT (activated partial thromboplastin time and Partial thromboplastin time)

evaluation of intrinsic system and common pathway

measures ability of clotting factors 1,2,5,8,9,10,11,12

normal times: aPTT = 30-40sec, PTT= 60-70sec

critical times: aPTT >70 sec, PTT > 100 sec

anticoag rx = 1.5-2.5x the value

Coagulation Labs - aPTT and PTT (activated partial thromboplastin time and Partial thromboplastin time) - increased levels

acquired or cogenital clotting factor deficiencies

cirrhosis of the liver

vitamin K deficiency

disseminated intravascular coagulation

heparin admin

Coagulation Labs - aPTT and PTT (activated partial thromboplastin time and Partial thromboplastin time) - decreased levels

early stages of disseminated intravascular coagulation

extensive cancer

coagulation labs: anti-factor Xa

evaluation of the intrinsic pathway

heparin interferes with clotting by accelerating inhibition of factos Xa and thrombin

therapeutic range = 0.3-0.7

EBP - measurement of levels may give more accurate reading of coagulation then aPTT /PTT w/ heparin

increased MCV

liver dz

antimetabolite therapy

acloholism

pernicious anemia (vit B12 deficient) (macrocytic normochromic anemia ~ megaloblastic)

folic acid anemia (macrocytic normochromic anemia ~ megaloblastic)

Decreased MCV

iron deficiency anemia (microcytic hypochromic anemia)

thalassemia

anemia from chronic illness (normocytic normochromic anemia

increased MCH

macrocytic anemia

decreased MCH

microcytic and hypochromic anemia

Increased MCHC

spherocytosis

intravascular hemolysis

cold agluttinins

decreased MCHC

iron deficiency anemia

thalassemia

increased RDW

iron deficiency anemia

folate or B12 deficiency anemia

sickle cell dz

hemolytic anemias

posthemorrhagic anemias