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• Hematology encompasses key terms used in the field
• Visual morphology aids in the identification of blood cells
• Thrombosis and hematopoiesis both have distinct physiological processes
• Key historical signs and physical characteristics manifest with anemia

Anemia

• It requires understanding of the pathophysiology, diagnosis, and treatments, including but not limited to; Iron-deficiency anemia, anemia of chronic disease, vitamin B12 deficiency, and hemolytic/aplastic anemia
• Anemias are categorized as microcytic, macrocytic, or normocytic
• Hemophilia shares similar symptoms/signs with anemia
• Complete blood count and iron studies can help determine a patient’s anemia
• Treatment and referral plans should also be developed for patients with anemia or hemophilia
• Educating patients is key regarding the emergency department precautions, the expected course, and management of disease

Hemoglobinopathies

• Requires understanding the clinical presentation, pathophysiology, diagnosis, and treatment regarding Glucose-6-phosphate dehydrogenase deficiency, sickle cell anemia, thalassemias, and hemochromatosis.

Bleeding Disorders

• Requires understanding the clinical presentation, pathophysiology, diagnosis, and treatment regarding Hemophilia A/B, and Von Willebrand Disease

Key Terms

• Hematopoiesis involves the production of blood cells in the bone marrow, including erythrocytes, granulocytes and platelets
• Stem cells are the progenitors with dual properties of self-renewal and lineage commitment through mitosis
• Blasts the are most immature cells which contain large nuclei of a lineage
• Large amount of blasts on the smear can indicate acute leukemia or fresh proliferation
• "Left Shift" indicates numerous immature cells in the blood, suggestive of proliferation
• Numeruous neutrophils in a patient with "Left Shift" indicates the body is fighting an infection
• "Left Shift" refers to the earlier differential counting machines to count cells on a slide
• A CBC (complete blood count) gives important information for hematologic and organ systems
• A CBC includes; RBC (red blood cell) count, hemoglobin, hematocrit, RBC indices, WBC (white blood cell) count, WBC differential count, platelet count, and mean platelet volume.
• RBC count for males is 4.7 to 6.1 million cells/µL, females it is 4.2 to 5.4 million cells/µL
• Hemoglobin: Males are 14-18 g/dL, females 12-16 g/dL
• Hematocrit: Males are 42%-52%, and females are 37%-47%

RBC Indices

• Mean Corpuscular Volume (MCV): 80-100 femtoliter
• Microcytic MCV is low
• Macrocytic is high
• Normocytic is normal
• Mean Corpuscular Hemoglobin (MCH) amount: 27-31 pg
• Mean Corpuscular Hemoglobin Concentration (MCHC): concentration average of Hgb
• Normochromic MCHC is normal
• Hypochromic MCHC is low: iron deficiency anemia
• Hyperchromic MCHC is high and consider B12/folate deficiency
• Red Cell Distribution Width (RDW): 11% - 14.5%
• It is the standard deviation of RBC volume by the MCV
• Indicates greater variability in the size of red blood cells when the number is high
• White Blood Cell Count range: 5000 -10,000/μL
• WBC Differential Count includes: Neutrophils, lymphocytes, monocytes, eosinophils & basophils
• Neutrophil range: 2500-8000/μL or 55%-70%
• Lymphocytes range: 1000-4000/μL or 20%-40%
• Monocytes range: 100-700/μL or 2%-8%
• Eosinophils range: 50-500/µL or 1%-4%
• Basophils range: 25-100/μL or 0.5%-1%
• Platelet Count range: 150,000-400,000/μL
• Mean Platelet Volume (MPV) range: 7.4-10.4 femtoliter
• Anemia is when the RBC hemoglobin, hematocrit are low
• Polycythemia (erythrocytosis) means that the RBC, hemoglobin, hematocrit are high
• Leukopenia means there the WBC is decreased
• Neutropenia has low neutrophils
• Lymphopenia has low lymphocytes
• Monocytopenia has low monocytes
• Leukocytosis has increased WBC
• Neutrophilia has increased neutrophils
• Lymphocytosis has increased lymphocytes
• Monocytosis has increased monocytes
• Thrombocytopenia means there are low platelets
• Thrombocytosis means increased platelets
• Pancytopenia is a combination of neutropenia, anemia, thrombocytopenia
• Aplasia: marrow isn't making enough cells
• Dysplasia: marrow isn't making cells that function properly
• Reticulocyte: immature young RBC indicating the marrow's ability to respond to anemia. (Adult normal range: 0.5% - 2% of total # of RBCs)
• Peripheral Blood Smear: Microscopic examination of a patient's blood, giving info relating to the three hematologic cell lines – erythrocytes (RBCs), platelets and leukocytes (WBCs)
• Circulating vs Marginated Neutrophils: The number of neutrophils seen in a blood is representative
• Demargination is when the neutrophils that are marginated are the released

Cells

• Red blood cell, erythrocyte: carries oxygen and has a life cycle of 120 days
• Erythropoiesis occurs in the bone marrow under influence of erythropoietin
• Platelets, thrombocytes are a major factor in coagulation and a lifespan of about 10-12 days
• Neutrophils has a lifespan of about 3-5 hours
• Lymphocytes cells can live for decades with T cells that travel to tissues
• B cells produce antibodies

Substances

• Ethylenediaminetetraacetic acid: EDTA, anticoagulant present in the “purple top
• Erythropoietin: it raises the RBC production in marrow, released by kidneys in response to hypoxia
• Prothrombin: aka factor II
• Fibrinogen: the precursor
• Plasminogen: precursor to plasmin
• Fibrinolysis: the action of breaking down the fibrin
• D-dimer: fibrin degradation product.

Visual Morphology

• Includes erythrocytes, platelets, neutrophils lymphocytes, monocytes Eosinophils & Basophils

Thrombosis

• A clot occurs when the endothelium is damaged exposing collagen
• Circulating Von-Willebrand factor connects to the unrolled collagen binding for platelets
• Platelets turn from discs to spheres with pseudopodia that activate the release of enzymes
• The Prothrombin Activator Complex (Xa and Va) uses calcium ions to convert prothrombin to the active form, thrombin (II to IIa)
• IIA converts fibrinogen to fibrin, where fibrin forms strands

Extrinsic Pathway

• Factor VII flows abudantly in blood triggering Tissue Factor (III) in damaged tissue
• Tissue Factor stimulates factor VII to its active state, eventually activating calcium to convert factor X to its active state
• The extrinsic pathway is quicker because of feedback loops built in

Intrinsic Pathway

• Occurs when triggered by inflammation
• Factor XII activates Factor XI and uses other cofactors, eventually activating Factor IX where it combines with VIII using calcium ions, activating Factor X

Plasminogen

• Is converted to plasmin by u-PA and t-PA: breaking down into fibrin degradation products (FDPs) in FIBRINOLYSIS

Hematopoiesis

• Bone marrow is a conveyer belt that releases them into blood stream in normal conditions
• Stress can accelerate the process
• Severe anemia causes more reticulocytes
• Bacterial infection causes a “left shift" of the neutrophil line
• Hematologic neoplasias

Anemia

• Decrease in RBC measurements from a CBC test
• Manifestation is from an underlying disorder
• Hematocrit is usually three times more than the hemoglobin
• Patients who are short on blood cells might display a variety of neurological symptoms from fatigue, dizziness, and headaches

Anemia Compensations

• Body compensates by constricting peripheral vasculature
• Body fails to compensate starts causing heart tissues to fail
• Kidneys monitor levels of oxygen and releases erythropoietin in response
• Checking for underlying health issues can reveal cause

Anemia Questions

What is the nutritional status of your patients?

• Has the patient received blood transfusions or had liver damage?
• What questions might you want to ask a patient in whom you suspect anemia?
• Recent surgeries or injuries
• Bleeding
• History of anemia
• Symptoms
• Menstrual history

Physical Examination for Anemia

• Palpebral conjunctiva-pallor: The most useful physical location to detect
• Digital rectal exam and guaiac testing for more sensitive skin tone
• Petechiae or purpura suggest thrombocytopenia

Anemia Laboratory Tests

• Labs to consider: CBC and differential or type to prepare a possible transfusion
• When concerned about bleeding consider measuring; PT/INR, PTT, and fibrinogen
• Chemistry and LFT for why
• Order a Coombs test for auto immune
• Consider a pregnancy test
• Size helps classify it
• Treatment may differ, and it stems from other conditions
• It falls into three categories of anemia:
• Reduced production if marrow decreases from failure
• Reduced survival from extrinsic factors

Immune Hemolysis

• Transfused blood cells can cause problems
• Autoimmune, lupus etc may have splenomegaly
• Drug induced may be cephalosporins and levofloxacin
• Infectious which can be malarial
• HUS happens as the most commonly due to E. coli
• Defects in hemoglobin/structural surface may result in Acute bleeding
• Volume affects result/dehydratation affects anemia

Cell Volume

• Dehydration creates high hemacrit
• Acute hemorrhaging takes time administered (IV) for Hgb Hct recognition
• Cirrhosis, CHF, or volume overload shows Hgb Hct low results
• Hemolysis which flows freely can exhibit jaundice
• MCV: between 80 and 100 fL (femtoliters) is average red blood cell volume
• Microlytic has low MCV
• Macrolytic has high MCV
• Normolytic: average of between 30 and 35
• Normochromatic levels normal MCHC
• Hypo iron deficiency: low MCHC
• Reticule: immature blood cell

Red Cell Distribution

• High numbers mean high variance
• Anemia is a sign that should not be ignored
• Bleeding or increased RBC destruction indicates bone marrow
• Lab findings reveal CBC smear and increases or decreases based on disease
• Micro <80

Anemia Pearls

• Iron deficiency anemia can indicate; reduced ferritin, increased TIBC and low serum
• Chronic inflammation indicates decrease in serum, lower TIBC and increased ferritin levels
• Anemia is a subject that is lecture intensive

Iron-Deficiency Anemia

• Bloodloss/menstrual trauma can cause anemia
• Unexplainable or excessive loss can lead to endoscopy or even lower or upper endoscopy
• 10%-15% of American average in their iron intake
• Ascorbic assists absorption
• Intestinal tracts are home base
• iron absorption from gut may be reduced from transfers
• Transferrin binds to iron
• Store house protein indication can show available protein

Symptoms and Signs of Storage Problems

• Fatigue, headaches, irritability and palpitations
• Can also result in smooth tongue or brittle nails

Iron levels

• Under 30 is not consistent with iron deficiency
• High transferrin is common
• Can also occur the same with inflammation treatment can vary
• Bone marrow biopsy happens rarely
• Chronic disease or toxicity has to differ from

Guidelines to CDC protocol

• Screen adolescents and girls or people every one to five
• If low, screen for iron deficiency anemia
• Stratified by pregnancy for criterion
• Has proven nonbeneficial with postmenopausal women and routine men.
• Replacements include; ferrous sulfate
• Stomach sides can cause lack of compliance
• Better with vitamin V and dosing to test

Oral replacments

• Other options are ferris and fumarate
• Take iron for a few months longer due to some stomach sensitivities Parenteral replacements are also required to tolerate replacement.
• Can present those who are immune to the same as inflammatory problems
• Transfusion treatments may lead to many health problems Hgb>7 g/DL is normal. Referral when suspected or not responding

Problems Linked to Anemia

• Chronic; mild, noramylic
• Increased transferrin, ferrin or reduced transferrin
• Underlying chronic diseases caused by inflammatory infections or malignancy
• Low gut and marrow productions causes a decline
• Caustic condition is dependent

Organ Failure

Occurs in patients with chronic kidney disease (CKD), liver failure, and endocrine gland failure ↓ erythropoietin release resulting in↓ RBC production Serum iron is low in CKD but is normal in liver and endocrine gland failure In CKD there is↓ clearance of hepcidin from the kidney resulting in increased degradation of ferroportin and decreased absorption of iron from the gut Signs and Symptoms:

Dependent upon the causative condition Suspected in patients with known chronic diseases If patient is significantly anemic, may have coexistent iron deficiency or folic acid deficiency Patients undergoing hemodialysis lose both iron and folic acid

Laboratory Findings

Hct rarely falls below 60% of baseline MCV is normal to slightly reduced RBC

Megaloblastic Anemia

Due to deficiency to b12 or if a smear is used

B12 Deficiency

• needed for a multitude of functions
• daily average intake: 2-5mcg
• Can be from a variety of diseases
• Moderate issues with a range of B12
• Possible for the patients symptoms to be ignored
• Anorexia and diarrhea
• Complicated neuro condition occurs
• low serum counts
• Red blood cells may increase
• Bioposy results may not show
• Inactive patients may need injections Neurological symptoms can be lessened in the first 6 months or potentially a referral

Folica Acid

• High intake in fruits/vegges
• body requires: 50-100mcgs 5mgs
• Deficiency may stem from pregnancy complications as well
• Neurological abnormalities and changes in cells
• treatment requires: 1mcg per day

Hemolytic Anemias

Reticytye cells increase with hemolysis

• intracupuslar defects : defects that are rare
• membranes that affect
• extra problems that affect the entire membrane
• infections affecting body lysis happens within systems , can harm bodies, and deplete them Hgb degrades

Anemia Approach

• rapid spread in the skin, splem, lungs and consult heme is critical with problems

lab features that assist

• haptoglobin
• increase counts assist with treatment related conditions

Alpha Thalessimia

Hemoglobinuria

• Rare condition that are complications Treatment is mild treatment options

Autoimmune Hemolytic Anemia

Hemolysis

• leads to the macrophages eating
• auto immune can be threatening

Clinical signs for problems

• fatigue and dyspnea
• splem and jauduce results
• severe problems can result in multiple infections to the bone marrows issues

Bone problems

• bone marrow and autoimmune attacks
• weakness and fatigue in joints
• allogeneic sibling treatment can have curative results
• can be chronic if unassisted
• heme problems may occur and be painful for many

Hemablobin

• inherited blood disorders
• difficulty breathing
• can be fatal

Early Detection

• 6- phosphate dehydrogenase deficiency : examples of treatment types for thalassemia
• 9- phosphate linked can be recessive
• medication with an increase
• treat with oxidant and avoid known drugs
• rare to skip fava beans

Alpha cell

• genetics and disorders stemming from cells with origins that affect the cells
• single chain results in unstable blood with electrophoresis
• blockage is normal with some side problems

Treatment for Pain-related Disorders

• transplantation of Hematopoiesis, can assist with oxygen intake avoid drugs, and may require blood transfusion

Thalassimia

• decreased protein that affects body's protein level
• can also cause mild anemia

beta or alpha effects the chain

• the affected chain may determine the issue

Genetic Effects

• genetics can be recessive and determined by tests
• Beta thalassemia is often transfused or known as coopers anemia
• Life may be difficult with problems but treatment leads to transplant options

Hemochromatosis HFE and recessive genes genetic testing is useful

Treatements

• avoud alcohol intake

Hemoplilia

Bleeding symptoms occur and factor deficiency results females are carriers

Thrombin

• genetic problems can make it difficult for bodies to clot
• minor genetic problems can persist for hours

Diagnoses Results

Low levels of Factor VIII can prevent clotting

• platelet is normal until platelet count occur

Tests DDAVP can assist, Anti drugs or Gene therapy

Von Willebrand

Made of endothelial tissue Synthesized and stored in the endothelium of blood vessels linked through platelet injury von Willebrand is prevalent disorder problems are activity problems and deficiency

The chart can change with results

The chart can vary can take for months and requires maintenance