Disorders of hematopoietic function-JZ

Red Blood Cells (RBCs)

• responsible for oxygen delivery & CO2 removal

• aka erythrocytes

• life span: 120 days

• normal RBC: 4.5-6.0 million cells/L

• hematocrit: 37%-52%

RBC Production = erythropoiesis

• RBC are produced in bone marrow

• erythropoietin stimulates bone marrow to produce RBCs

• erythropoietin - produced by the kidneys

Hemoglobin (Hgb)

• RBCs primarily made of hemoglobin

• ~ 250 million hemoglobin molecules per RBC

• hemoglobin is how RBC carry O2 to CO2

• 1 hemoglobin molecule can carry 4 O2 or CO2 molecules

RBC destruction

• facilitated by spleen, liver, bone marrow, lymph nodes

  • hemoglobin

    • heme

      • iron

      • bilirubin

    • globulin

      • amino acids

RBC disorders

• anemia

Anemia

• RBC disorders = anemias

  • blood loss

  • iron-deficiency

  • pernicious

Cause of Anemia

• dependent on the type of anemia

Dev. of Anemia

• decreased circulating RBCs

• decreased hemoglobin (Hgb)

• abnormal Hgb

or

• impaired production

• increased destruction

• blood loss

resulting in:

• decreased oxygen carrying capacity

Signs & Symptoms of Anemia

• weakness

• fatigue

• pallor

• syncope

• dyspnea

• tachycardia

Diagnosis of Anemia

• lab testing

  • complete blood count (CBC)

Treatment of Anemia

• dependent on the type of anemia

Cause of Blood Loss Anemia

• bleeding - chronic or acute

Signs & Symptoms of Blood Loss Anemia

• orthostatic hypotension

• increased HR, decreased BP

• decreased LOC

• decreased urine output

• melena

• hematemesis

Treatment of Blood Loss Anemia

• stop bleeding

• blood transfusion (if Hgb <7)

• iron supplementation

• increasing dietary iron

Cause of Iron Deficiency Anemia

• decreased Hgb production d/t lack of iron (Fe)

• common in women of childbearing age, children <2, & elderly

• inadequate intake of iron

Signs & Symptoms of Iron Deficiency Anemia

• hair loss

• cheilitis

• glossitis

• pica

• splenomegaly

Treatment of Iron Deficiency Anemia

• iron supplementation

• increasing dietary iron

Cause of Pernicious Anemia

• vitamin B12 deficiency

  • inadequate intake of B12

  • lack of intrinsic factor

Signs & Symptoms of Pernicious Anemia

same as general anemia

• weakness

• fatigue

• pallor

• syncope

• dyspnea

• tachycardia

Platelets

• responsible for hemostasis (blood clotting)

• aka thrombocytes

• normal platelet count: 150,000-450,000 cells/mL

Hemostasis 3 types

• physiological process that stops bleeding at site of injury

3

• vasoconstriction

• platelet plug formation

• coagulation

Vasoconstriction

• initiated by endothelial injury

• vessel spasm = vasoconstriction

  • decreased blood flow

  • increased BP

Platelet Plug Formation

• activation

  • collagen & von Willebran factor

    • change from smooth disk to spiny spheres

• adhesion

  • to vessel wall

• aggregation

  • clumping together

Coagulation

• intrinsic pathway

  • damage to vessel wall

  • slow process

• extrinsic pathway

  • trama to vessel

  • faster process

• common

  • prothrombin to thrombin

  • fibroingen to fibrin

Clot Dissolution

• clot retraction

  • occurs 20-60 min after clot formation

  • platelets squeeze serum from clot

    • pulling vessel edges together

• fibrinolysis

  • aka dissolution

  • plasminogen in converted to plasmin

    • plasminogen activators

  • digests fibrin strands & clotting factors

Platelet Disorders

• thrombocytosis

• thrombocytopenia

Thrombocytosis

• disorder of too many platelets

  • hypercoagulability, increased clotting risk

Cause of Thrombocytosis

• primary (essential) - increased platelet production

• secondary (reactive) - caused by another condition (ie: infection / infection)

Dev. of Thrombocytosis

• platelets < 450,000

Signs & Symptoms of Thrombocytosis

• deep vein thrombosis (DVT)

• pulmonary embolism (PE)

Diagnosis & Treatment of Thrombocytosis

• lab testing

  • CBC

• medications

• plateletpheresis (donation)

Thrombocytopenia

• too few, not enough, platelets

  • bleeding risk, also considered a coagulation disorder

Cause of Thrombocytopenia

• decreased production

• increased destruction

  • drug induced (heparin, aspirin)

  • immune induced - ITP

Dev. of Thrombocytopenia

• platelets < 150,000

Signs & Symptoms of Thrombocytopenia

• fatigue

• petechiae

• purpura

• ecchymosis

• bleeding - nose, mouth, GI tract

Diagnosis & Treatment of Thrombocytopenia

• lab testing

  • CBC

• medications

Coagulation Disorders

• factor V liden

• hemophilia & von Williebran disease

Factor V Liden

• thrombophilic genetic disorder that causes hypercoagulability

  • genetic mutation, increased risk of thrombosis/clot formation

Cause of Factor V Liden

• genetic mutation

• increases clotting risk x7

Dev. of Factor V Liden

• impacts the factor V in the clotting cascade causing more coagulation

• thrombi form most commonly in the venous system

• resulting in DVT or PE

Signs & Symptoms of Factor V Liden

• deep vein thrombosis (DVT)

• pulmonary embolism (PE)

Diagnosis & Treatment of Factor V Liden

• lab testing

  • genetic screening

• anticoagulant therapy

Hemophilia & von Williebrand Disease

• genetic coagulation disorder that causes bleeding

  • genetic mutation, bleeding risk

Cause of Hemophilia & von Williebrand Disease

• genetic mutation

• different types (A, B…)

• lack of clotting factors needed to form clots

Dev. of Hemophilia & von Williebrand Disease

• w/ out all clotting factors blood clots are unable to form

• resulting in bleeding that does not stop

Signs & Symptoms of Hemophilia & von Williebrand Disease

• bleeding

  • trauma/injury

  • GI

• bruising

Diagnosis & Treatment of Hemophilia & von Williebrand Disease

• lab testing

  • genetic screening

• medication

  • factor replacement

  • desmopressin (DDAVP)

Bleeding Complications

• Systemic Symptoms of Bleeding

• Tachycardia

• Hypotension

• Pallor

• Syncope

• Changes in LOC (hypoxia)

• Disseminated Intravascular Coagulation (DIC)

• Disorder of both clot formation and bleeding that is seen in critically ill patients

• Caused by: high volume blood loss, sepsis, trauma (burns)

• Can lead to organ failure

• Patho: abnormal production of clotting factors and fibrinolysis factors

• Clinical Manifestations:

• Signs of bleeding: oozing around IV sites, petechiae, bleeding gums, spontaneous GI bleeding

• Development of microthrombi: disrupting blood flow to heart, lungs and kidneys

• Treatment: blood products, fluids, critical care

• Prognosis: 20% - 50% mortality rate

Lab values

Hematology:

Complete Blood Count (CBC)

• Red Blood Cells (RBC): 4.5-6.0 million cells/L

• Hematocrit (Hct): 37&-52%

• Hemoglobin (Hgb): 12-18g/dL

• Platelets: 150,000-450,000