DPT 604: Hematologic System

transportation, regulation and protection

3 key functions of blood

oxygen, carbon dioxide, nutrients, hormones, waste products

key components that blood transports

pH, body temperature and fluid balance

what does blood help regulate?

Erythrocytes

Red blood cells responsible for oxygen transport.

leukocytes

White blood cells involved in immune response and fighting infections

thrombocytes or platelets

blood cell type responsible for assisting with clotting

hematopoiesis

formation of blood cells

bone marrow

where are blood cells made?

spoon nail

this nail deformity can be an indication of iron deficiency and anemia

little to no trauma, lasting longer than normal, present in stool or emesis

when does bleeding/bruising become a concern?

petechiae

smallest, flat, pin-point red/purple spot

purpura

larger flat spots

ecchymoses

AKA bruise or contusion; flat area of discolored skin

hematoma

large, localized collection of blood (often clotted) outside vessels; usually raised and palpable. can be painful

thrombus

solid mass of clotted blood within a blood vessel or heart chamber

embolus

mass of solid, liquid or gas that travels to lodge at distant site

congestion

accumulation of blood within blood vessels of organs or tissues

infarction

region of necrosis caused by reduction of arterial perfusion

edema

accumulation of fluid in interstitial tissues or body cavities

lymphedema

accumulation of lymph fluid in interstitial space

lymphadoenopathy

disease of lymph nodes

splenomegaly

enlargement of the spleen

shock

circulatory system unable to maintain adequate pressure to perfuse organs

etiology and pathophysiology of anemia

RBC cannot transport the sufficient amount of oxygen to meet the need of the body; lack of iron, lack of RBC. can be due to loss of too much blood or abnormal cell shape/function

symptoms of anemia

paleness/yellowing of skin

fatigue/dizziness

Changed stool color

SOB

Weak mm

Low BP

Decreased exercise tolerance

Severe: fainting, chest pain, angina, MI

diagnosis and treatment for anemia

Dx: CBC

Tx: diet, iron supplements, transfusion if severe. It all depends on cause

etiology and pathophysiology of polycythemias

The increased RBC increase blood viscosity and blood volume

Primary: polycythemia vera (neoplastic disease of bone marrow)

Secondary: due to underlying medical conditions/external factors (altitude change, smoking)

Increase # of RBC

signs and symptoms of polycythemias

SOB

Fatigue

Blurred vision

Fullness in head

Weight loss

Bruising

etiology and pathophysiology of Hemochromatosis

Genetic condition (mutation)

Body cannot break down iron in blood, iron overload

Increased iron absorption of the small intestine

signs and symptoms of Hemochromatosis

Skin bronzing, joint pain (iron fist sign), weakness, fatigue, abdominal pain

diagnosis and treatment for Hemochromatosis

Dx: blood test/genetic screening

Tx: therapeutic phlebotomy

etiology and pathophysiology of Thrombocytopenia

Inadequate production of bone marrow

Increased platelet destruction

Splenic sequestration

Causes: medications/supplements, cancer, bone marrow issues, alcohol use

Low platelet count

signs and symptoms of Thrombocytopenia

Bleeding

Easy bruising

Mennoragia

diagnosis and treatment for Thrombocytopenia

Dx: CBC

Tx: treat underlying cause, platelet transfusion

etiology and pathophysiology of Thrombocytosis

High platelet count

Primary & secondary

signs and symptoms of Thrombocytosis

clotting/thrombosis

Asymptomatic until higher

diagnosis and treatment of Thrombocytosis

Dx: CBC

Tx: treat underlying cause, platelet transfusion

etiology and pathophysiology of Leukopenia

Infections, autoimmune disorders, bone marrow failure/suppression, diseases, medications

Low WBC count

signs and symptoms of Leukopenia

⬆️infection risk, fever, fatigue, mouth ulcers

diagnosis and treatment of Leukopenia

Dx: CBC w/ differential, eval leukocyte count. Maybe bone marrow biopsy

Tx: depends on underlying cause

etiology and pathophysiology of Leukocytosis

Inflammation, infection, severe stress/pain, steroids

Increased WBC count

signs and symptoms of Leukocytosis

Often asymptomatic; fever, infection signs, inflammation,

diagnosis and treatment of Leukocytosis

Dx: CBC w/ differential, eval leukocyte count. Maybe bone marrow biopsy

Tx: depends on underlying cause

etiology and pathophysiology of Leukemias

Originate in blood forming tissue

Risk factors: radiation exposure, chemical exposure, genetic disorders, family hx

Abnormal proliferation of WBC

Acute: accumulation of immature cells; aggressive, higher death rate

Chronic: prolonged course; accumulation of more mature

signs and symptoms of leukemias

Anemia

bleeding/bruising

Bone pain

diagnosis and treatment of leukemias

Dx: CBC, blood smear, bone marrow biopsy

Tx: varies by type: chemo, HCT

etiology and pathophysiology of Multiple Myeloma

Cancer originating in plasma cells in the bone marrow

Idiopathic

Risk factors: old age, chemical exposures, genetic mutations

B-lymphocytes differentiated

Malignant cells invade bone marrow

signs and symptoms of Multiple Myeloma

CRAB

Calcium increase (confusion, constipation, weakness)

Renal failure

Anemia

Bone lesion

diagnosis and treatment of Multiple Myeloma

Dx: CBC and bone marrow biopsy

Tx: chemo, targeted therapy, stem cell transplant, corticosteroids, radiations for bone lesions

etiology and pathophysiology of Myeloproliferative Neoplasms/Disorders

Excess of certain type of blood cell

Too many cells in the body

Chronic myeloid leukemia (CML); too many WBC

Polycythemia: too many RBC

Essential thrombocythemia (ET): too many platelets

Primary myelofibrosis (MF): fibrosis/scar tissue of bone marrow

signs and symptoms of Myeloproliferative Neoplasms/Disorders

Increased thrombosis/hemorrhage

Fatigue

Weight loss

Itching

Bone pain

diagnosis and treatment of Myeloproliferative Neoplasms/Disorders

Dx: biopsy

Tx: oral medication, chemo, HCT

etiology and pathophysiology of Von Willebrand Disease (vWD)

Autosomal inheritance

Deficiency/dysfunction of von willebrand factor (vWF)

signs and symptoms of Von Willebrand Disease (vWD)

Easy bruising

mucocutaneous bleeding

Heavy periods

Excessive bleeding

diagnosis and treatment of Von Willebrand Disease (vWD)

Dx: CBC, coagulation factors, genetic testing

Tx: factor replacement therapy, antifibrinolytics

etiology and pathophysiology of Hemophilia

X linked inheritance; mainly affects males

Deficiency in clotting factors

signs and symptoms of Hemophilia

Easy bruising

mucocutaneous bleeding

Heavy periods

Excessive bleeding

Hematoma*

Hemoarthrosis*

Bleeding in deep muscles*

Bleeding into internal organs*

*unique to hemophilia

diagnosis and treatment of Hemophilia

Dx: CBC, coagulation factors, genetic testing

Tx: factor replacement therapy, gene therapy, antifibrinolytics

etiology and pathophysiology of Disseminated Intravascular Coagulation (DIC)

Acquired syndrome with widespread coagulation/clotting

Disruption of steady state

signs and symptoms of Disseminated Intravascular Coagulation (DIC)

Bleeding, organ dysfunction

diagnosis and treatment of Disseminated Intravascular Coagulation (DIC)

Dx: labs

Tx: treat underlying cause, supportive care

etiology and pathophysiology of Sickle Cell Disease

Inherited, geographic influence

Sickle shaped RBC that prevents the RBC from functioning properly

signs and symptoms of Sickle Cell Disease

Anemia

Fatigue

SOB

Infections

Slowed growth

Jaundice

Dark urine

Splenomegaly

Bone deformities

Pain episodes*

Swelling*

stroke*

Acute chest syndrome*

Organ damage*

*unique

diagnosis and treatment of Sickle Cell Disease

Dx: genetic testing, prenatal testing, CBC, hemoglobin tests, newborn screening

Tx: transfusions, HCT, gene therapy

etiology and pathophysiology of Thalassemias

Inherited disorder affecting the hemoglobin chain

Structurally impaired defective hemoglobin synthesis

signs and symptoms of Thalassemias

Anemia

Fatigue

SOB

Infections

Slowed growth

Jaundice

Dark urine

Splenomegaly

Bone deformities

diagnosis and treatment of Thalassemias

Dx: genetic testing, prenatal testing, CBC, hemoglobin tests, newborn screening

Tx: transfusions, HCT, gene therapy

hemoglobin

number 1 lab value for PTs to keep an eye on

general s/s of hematologic disorders

Fever

Chills

Pallor

Fatigue

Weakness

Chest pain

Palpitations

Dizziness

Dyspnea

Changes to nails

Tissue inflammation

Syncope

Spoon/clubbing nails