The Child with a Hematological Alteration Flashcards
Hematologic System - Hematology
* Study of blood and blood-forming tissues
* Hematopoiesis occurs initially in ___ of all bones
> Stem cells
- Red blood cells (RBC)/erythrocytes
- White blood cells (WBC)/leukocytes
- Platelets
bone marrow
Red Blood Cells (RBCs) or Erythrocytes
* Biconcave
* Transport O2 to tissue
* Need hemoglobin (Hgb) for O2 transport
* Hemoglobin needs IRON (Fe)
* Production stimulated by decreased circulating O2
* Kidneys secrete ___
* RBC precursors produced in bone marrow
erythropoietin
Red Blood Cells (RBCs)
Average size RBCs = ___
- Macrocytic
- Microcytic
- Normocytic
MCV (mean corpuscular volume)
Red Blood Cells (RBCs)
Average amount of Hgb in RBCs = ability to transport O2 into cells = ___
- Hypochromic
- Normochromic
MCH (mean corpuscular hemoglobin)
Red Blood Cells (RBCs)
Average amount of Hgb per volume of RBCs (concentration/ratio) of Hgb in RBCs (amount of Hgb small r/t RBC size) = ___
MCHC (mean corpuscular hemoglobin concentration)
Red Blood Cells (RBCs)
Anemia occurs:
↓ # RBCs
↓ Hgb content
↓ volume packed RBCs
Anemia classified according to:
↓ MCV (size of RBCs)
↓ MCH (amount Hgb in RBCs)
↓ MCHC (concentration of Hgb in RBCs)
?
↑ # RBCs
Polycythemia
White Blood Cells (WBCs) or Leukocytes
* Produced in bone marrow and lymphatic tissue
Protect body by:
- Phagocytosis
- Production of antibodies
Disorders of WBCs:
- Rate of WBC production = lymphocytosis or lymphopenia
- Alteration in cell function
Types of WBCs
Granulocytes
- Neutrophils
- Eosinophils
- Basophils
Agranulocytes
- Monocytes/macrophages
- Lymphocytes
- T-cells
- B-cells
- Natural Killer (NK) cells
Platelets
* Promote hemostasis
* Bone marrow - ?
* Smaller fragmented cells - ?
* Disorders occur when bone marrow cannot produce for demand
Megakaryocytes
Platelets
?
Most common cause of anemia in children
Causes
- ↓ Fe intake
- ↑ Fe or blood loss
- Rapid growth rate
Diagnostic Evaluation
* Nutritional assessment
* Laboratory work-up
> CBC
- Hgb, MCV, MCH ↓
- Reticulocyte count - normal or ↑
- Serum ferritin and serum Fe ↓
- TIBC ↑
Iron deficiency anemia (IDA)
Iron deficiency anemia (IDA) - Manifestations
* Extreme pallor
* Pale mucous membranes and conjunctiva
* Tachycardia, tachypnea, lethargy, fatigue, irritability
Therapeutic regimen
> Dietary changes
- Iron-rich foods
- Vitamin C (increases iron absorption)
> Iron supplementation
- Ferrous sulfate, ferrous gluconate, ferrous fumarate
* 3-6 mg/kg/day in 1 or 2 doses
* Empty stomach with Vitamin C
* DO NOT GIVE WITH CALCIUM (milk formula or iron-fortified cereal)
* Side effects = teeth staining (use straw); dark stools; constipation
Sickle Cell Disease (SCD)
* Genetic disorder = autosomal ___ condition
* Affects African, Mediterranean, Indian, and Middle Eastern descent
* Group of hemoglobinopathies characterized by production of sickle hemoglobin, chronic hemolytic anemia, ischemic tissue injury
recessive
Caused by:
* Low O2 concentration
* Acidosis
* Dehydration
* Stress
Which in children with SCD leads to RBCs assuming sickle shape
> Lifespan 10-20 days as opposed to 100-120 days
> Clumping together causes occlusions in small and large vessels
- Tissue ischemia, infarct, organ damage
- Lungs, spleen, and brain most affected
Sickle Cell Disease (SCD) - Manifestations
__ __ __
> Anemia, pallor, jaundice, fatigue, cholelithiasis, delayed growth in puberty, avascular necrosis of the hips and shoulders, renal dysfunction and retinopathy
Acute exacerbations or __ __ __
> Triggered by infection, dehydration, hypoxia, trauma, stress
Chronic hemolytic anemia
SICKLE CELL CRISIS
Sickle Cell Disease (SCD)
Complication of SCD = Sickle Cell Crisis can be…
?
* Increased destruction or decreased production of RBCs
* Pallor, lethargy, headache, syncope
* Tx - RBCs transfusions
Aplastic
?
* Most common PAIN
> Bone and joint pain
* Less common
> Acute chest syndrome, dactylitis, priapism, CVA
* Treatment
> oral analgesics
> oral and IV opioids, NSAIDs, hydration
> aggressive incentive spirometry (10x q2 hrs while awake)
Vaso-occlusive
?
* Obstructed blood flow from organs = engorged organs
- Engorged liver and spleen
- Pallor, irritability, and tachycardia
- ↓ Hgb
- Hypovolemic shock
* Leads to acute anemia
* When in lungs leads to acute chest syndrome
> s/s - chest pain, fever, cough, abd pain
> Tx - IV hydration, antibiotics, O2, RBC transfusion, analgesics
* Treatment in general
> IV fluid with crystalloid and colloid infusion
> Transfusion TX
> Splenectomy
Acute Sequestration
Sickle Cell Disease (SCD) - Therapeutic Management
* Preventing infections
> Prophylactic daily penicillin V therapy (BID) until age 5
* Immunizations
> Pneumococcal polyvalent vaccine PCV-13 (ages 2-4-6 and 12-15 months)
> H-influenzae
> Hepatitis B (birth - 2 mos - 4 mos)
> Annual influenza vaccine
> Meningococcal vaccine after age 2
* Preventing exacerbations
* Pain management
> Opioids (morphine is opioid of choice) and NSAIDs
* Identify complications
> Assess for fever - prompt tx if > 101.3
> Prevent strokes
> Avoid extreme cold/extreme heat
* Supportive care
> Hydration
> Oxygenation
> Analgesia
> RBC transfusion
Thalassemia
* Genetic disorder
* Thalassemia minor = 1 parent inherited gene
* Thalassemia major = 2 parent inherited genes = severe
* Primarily Mediterranean, Asian, African American descent
* Reduction or abnormal Hgb
↓ circulating O2 ⇒
Triggers production of erythrocytes ⇒
To meet demands - extramedullary hematopoiesis ⇒
Chronic state of production and destruction of erythrocytes ⇒
Inadequate amount of normal Hgb
Beta-thalassemia Manifestations
- Pallor
- Growth retardation
- Pubertal delay
- Severe anemia
- Characteristics facies (enlarged head, frontal and parietal bossing, severe maxillary hyperplasia, wide-set eyes with a flattened nose, malocclusion)
- Hepatosplenomegaly
- Bronze skin tone (yellow/green)
Diagnostic evaluation
- CBC
- Microcytic, hypochromic anemia
Therapeutic Management - Beta Thalassemia
* Erythrocyte transfusions - packed RBCs
> Goal to maintain Hgb at __ g/dL
* Chelation therapy
- Iron builds up from byproduct of hemolysis of RBCs
- Iron builds up from transfusions
> Inability for intestines to absorb large quantities of Fe
> ___ - excess deposits of Fe in tissues (major complication of long-term transfusion therapy)
CHELATION Therapy - removes excess amounts of Fe
> Deferoxamine (Desferal), Deferasirox (Exjade)
* Splenectomy
* Bone marrow transplant/stem cell transplant (only cure)
11 g/dL
HEMOSIDEROSIS
Hemophilia
* Hereditary - X-linked autosomal __ disorder (no cure)
* Female carriers pass onto males
* Lack of coagulation “factor”
* Varies - % in blood
> Hemophilia A - lack factor ___ (classic)
> Hemophilia B - lack factor ___ (Christmas disease)
> Hemophilia C - lack factor ___ (mild)
recessive
VIII
IX
XI
Hemophilia - Manifestations
* Bleeding
- After surgery or serious trauma
* Easily bruising
* Episodes of epistaxis and hematuria
* Bleeding in muscles and joints - ___
- Especially knees
- Causes swelling, pain, bleeding, stiffness
hemarthrosis
Hemophilia - Therapeutic Management
* Individualized and depends on severity
Goal ⇒ Prevent excessive bleeding and tissue damage by supplying the body with the missing factors - VIII or IX
* Monoclonal products
* Prophylactic therapy
> For mild hemophilia A - desmopressin acetate (DDAVP) intranasal spray - vasoconstrictor action
* Prophylactic factor - prior to surgery or dental procedures
* Prevent injury
> Pad corners, cribs, and extra padding for bones
> Soft brittle toothbrushes
> Avoid contact sports
> Protective helmets
* Injury
> Monitor area of injury qh/24 hours
> Measure circumference
> Head trauma = LOC hourly
> Treat = RICE
> Apply gentle pressure 10-15 min (venipunctures)
* Avoid rectal temps
* Administer factor replacement
* Do not give acetylsalicylic acid (aspirin or aspirin-containing products)
?
- Most common inherited bleeding disorder
- Autosomal inherited disorder with dominant and recessive variants
Type __ (defect in __ __ factor)
Type __ (mild-moderate)
Von Willebrand Disease
* Underproduction or dysfunction of Von Willebrand protein
II (Von Willebrand)
I
Von Willebrand Disease - Manifestations
* Epistaxis
* Bleeding from the gums
* Prolonged bleeding from cuts
* Excessive bleeding after surgery or trauma
* Menorrhagia (excessive menstrual bleeding)
Diagnostic evaluation
- Family history
- Bleeding history
- Lab tests - bleeding time, clotting time (PT)
- ELIZA
Von Willebrand Disease - Therapeutic Management
Goal is to control bleeding episodes and replace missing or dysfunctional factor in the blood
* Transfusion of anti-hemolytic factors or FFP
* Treatment of choices - DDAVP - synthetic derivative of ADH vasopressin
* Adolescent females with menorrhagia -
> Anti-fibrinolytics -
- Oral aminocaproic acid (Amicar) or tranexamic acid (Lysteda)
Education
- Prophylactic therapy
- Medic alert bracelet
- Limit contact sports
- Avoid OTC ASA, NSAIDs
Immune Thrombocytopenic Purpura (ITP)
* Acquired bleeding disorder
* Characterized by thrombocytopenia due to immune destruction (platelets <150,000 mm3); purpura rash; normal bone marrow production
* Acute or chronic
* Etiology - unknown
* Autoimmune - antibodies attack platelets and destroy (macrophages) in liver and spleen
Manifestations
- Sudden onset of bruising and petechiae (mucous membranes and gums)
Diagnostic evaluation
- Family history
- CBC
> Low platelet count <50,000 mm3
- Physical exam - bruising and petechiae
- Bone marrow aspirate - rule out cancer
Therapeutic management
* Goal is to prevent real life-threatening bleeding disorders such as intracranial bleeding
- Restore platelet count above 20,000
- Oral steroids and IV immunoglobulin (IVIG)
- Splenectomy
Family education
- Maintaining IV access for IV steroids or IVIG
- Restricting activity
- Use extra soft bristle toothbrush if platelet count <20,000
- Educating on side effects of steroids - mask infection
- Avoid administering IM injections; aspirin-containing products; and taking temperatures rectally
?
* Acquired hemorrhagic syndrome
Characterized by uncontrolled formation in deposition of fibrin thrombi
Causes
- trauma
- hypoxemia
- necrotizing enterocolitis
- shock
- liver disease
- overwhelming viral or bacterial infections
- acute promyelocytic leukemia
Disseminated Intravascular Coagulation (DIC)
Disseminated Intravascular Coagulation (DIC) - Manifestations
- Changes in platelet count and fibrinogen levels
- Excessive bruising and petechiae
- Oozing from puncture sites
- Oozing from sites of mild tissue damage
- Mild GI bleeding
Disseminated Intravascular Coagulation (DIC) - As disease progresses:
- purpuric rash
- worsening of bleeding
- hemoptysis
- hypoxemia
- oliguria
- renal failure
- organ failure
- intracranial bleeding
Disseminated Intravascular Coagulation (DIC) - Diagnostic evaluation
* Decreased RBC count
* Low platelet count
* RBC fragments on smear
* Prolonged PT
* Decreased fibrinogen level
* Elevated levels of fibrin degradation products (fdps) and D-dimer
Disseminated Intravascular Coagulation (DIC) - Therapeutic management
Goal is to identify and treat underlying conditions then treat symptoms
* Directed at replenishing consumed coagulation factors
- FFP
- RBCs and platelet transfusions
- Vitamin K
- Heparin
Aplastic Anemia
Characterized by cessation of hematopoiesis = granulocytes, erythrocytes, and megakaryocytes
⇒ peripheral pancytopenia
↓ WBC ↓ RBC ↓ Platelets
* Etiology = congenital, acquired, or idiopathic
* Mild, moderate, severe (depends on counts)
Diagnosis of severe aplastic anemia requires 2 of the following:
- granulocyte count <500 mm3
- platelet count <20,000 mm3
- reticulocyte count below 1%
- Also, bone marrow biopsy specimen must contain <25% of the normal cellularity
Aplastic Anemia - Manifestations
* Petechiae
* Ecchymosis
* Pallor
* Epistaxis
* Fatigue
* Tachycardia
* Anorexia
* Infection
Aplastic Anemia - Therapeutic management
* Treat symptoms
* Platelet and erythrocyte transfusions
* Antibiotic therapies and blood cultures
* Immunosuppressive medication - cyclosporin and antithymocyte globulin (ATG)
* Bone marrow or stem cell transplantation
* Private room preferably
* Meticulous hand hygiene
* No injections
* No rectal temperatures, medications, or examinations
* Extra soft bristle toothbrush
* Abstain from any contact sports
* Platelet count maintained > 20,000 mm3
* Hemoglobin level maintained > 7 g/dL
