Heme/Oncology

Describe the hematologic system

• Involved in the process of cellular regulation, consisting of blood and blood forming tissues of the body:

  • RBCs

  • Platelets

  • WBCs

Describe RBCs

• Transport nutrients and O2 to the body and waste products from tissue

  • Hemoglobin contains iron = good for O2 and CO2

  • Avg cycle = 120 days, bones continue producing cells

Describe platelets

• Responsible for clotting

• Irregularly shaped and have a sticky surface

Describe WBCs

• Fight infection

  • Germ present → WBC produce antibodies, surround bacteria, fight infection

  • WBC inc when infection present

  • Neutropenia: abnormal % neutrophils compared to total WBC = dec ability to fight pathologic bacteria

What are the different types of treatments for hematological disorders?

• H&H screen for anemia

• CBC w/ differential to help diagnose specific disorder

• Bone marrow

• Transfusions

Describe bone marrow and the different procedures done with it

• Bone marrow = spongy substance in center of bone that creates stem cells and produces RBCs

• Procedures:

  • Bone marrow aspiration = most conclusive test to determine aplastic anemia, leukemia

  • Bone marrow transplant = marrow is aspirated out of iliac crest, filtered, transferred to a blood bag and infused

Describe different types of transfusions given for hematological conditions

• Packed RBCs = anemia

• Platelets = platelet dysfunction

• Fresh frozen plasma = coag factors

What are the different types of reactions to transfusions?

• Hemolytic = immune response

• Allergic = resp distress and anaphylaxis

• Febrile = occurs after many transfusions

What guidelines do we follow with blood transfusions?

• NO:

  • Meds in blood

  • Blood with dextrose and water (don’t give with IV solutions)

  • Blood that’s discolored, cloudy, or unrefrigerated for more than 20 mins

What common lab and diagnostic tests are used for hematological conditions?

• Complete blood count with differential (ANC)

• Reticulocyte count

• Hemoglobin electrophoresis

• Blood type and cross-match

• Clotting studies

• Coagulating factor concentration

• Iron and lead levels

• Serum ferritin

Describe anemia and the different types

• Deficit of rbc/hgb caused by impairment of rbc production or inc in rbc destruction

• Types:

  • Iron deficiency anemia

  • Aplastic anemia

  • Sickle cell disease

  • Thalassemia

Describe iron deficiency anemia (IDA)

• Blood doesn’t have enough iron to produce hgb (poor diet/impaired absorption)

• Most common

• Occurs in children experiencing rapid growth/loss of blood

• Hgb < 8 g/dl

What signs do you see in children with IDA?

• Irritability

• Fatigue

• SOB

• Pallor

• Difficulty feeding

• Exercise intolerance

What is the treatment for IDA?

• Iron supplementation given AM on empty stomach

  • May result in dark colored stools

Describe the management for IDA for different age groups

• Infants < 12 mo = formula with iron

• Infants > 12 mo = decrease milk intake; inc solid foods

  • Solid foods introduced around 6 mo

    • Rice cereal fortified with iron

    • Pureed Vegetables, meats

• Children: iron fortified cereals, meat, green leafy vegetables, yogurt, cheeses, low/non-fat milk

• Teenagers: reduce junk food

What foods are rich in iron?

• Red meats, tuna, salmon eggs, tofu enriched grains

• Leafy green veggies

Describe aplastic anemia

• Failure of bone marrow to produce cells

• Acquired/inherited (fanoci’s anemia)

• Signs: ecchymosis, petechiae, oral ulcers, tachypnea, tachycardia

What is the treatment for aplastic anemia?

• Bone marrow transplant

• Antithymocyte globulin administered IV - 4 days.

  • Response seen within 3 mo

Describe sickle cell disease

• Dec level of O2 and hgb in rbcs

• Obstructs blood flow = congestion and tissue hypoxia

• Autosomal recessive disorder

• Complications include splenic sequestration and vaso-occlusive crisis

What are signs of sickle cell disease?

• Severe chronic anemia: pallor

• Abdominal & joint PAIN, gallstones

• Lethargy, irritability, fever, enlarged spleen

• Jaundice from excessive cell destruction, widened bone marrow

How do we manage sickle cell disease?

• Prevent vaso-occlusive episodes & infection

• Pain management

• Encourage hydration with IV fluids or inc fluid intake

Describe thalassemia

• Inherited diseases of blood that affect ability to produce hgb, chronic

• Types:

  • Cooley’s anemia = severe form of disease

  • Alpha thalassemia = synthesis of alpha chain of hgb is affected

  • Beta thalassemia = more common, 3 different categories

Describe the different categories of beta thalassemia

• Minor (β-thalassemia trait): leads to mild microcytic anemia (often no treatment req)

• Intermedia: req blood transfusion

• Major: req ongoing medication attention, blood transfusion, iron removal

How do you assess for thalassemia?

• Blood tests/family genetics study → has thalassemia or carrier

• Chronic villi sampling @ 11th week prenatal / amniocentesis @ 16 wks

• s/s: pale, listless, poor appetite, enlarged spleen, liver, heart, bones thin & brittle

• HF and infection = major cause of death

How do you manage thalassemia?

• Freq blood transfusions = hgb level near normal

• Iron chelators = rid excess iron from transfusions

• Bone marrow transplant = only possible for pt who have suitable bone marrow donor

Describe lead poisoning

• Lead in bloodstream interfering w/ enzymatic process of heme

• Exhibit s/s of anemia

• Risk factors = lead exposure in home, school environment

What are s/s of lead poisoning?

• Behavioral problems, irritability, hyperactivity, lack of ability to meet developmental milestones

• Blood level of lead > 10 μg/dL = require follow up

What is the management for lead poisoning?

• Interventions based on blood lead level

• Screen children for lead exposure

• > 44 → chelation therapy (removal of heavy metals via chelating agents)

Describe idiopathic thrombocytopenia purpura (ITP)

• Immune response following viral infection

  • Antibodies see platelets as bacteria and work to eliminate/destroy them

• Considered acute in children who’re 1-6 yrs old and chronic for 10+ yr olds

• Preceded by viral illnesses: URI, varicella, smallpox/measles vax

What are s/s of ITP?

• Random purpura

• Epistaxis, hematuria, hematemesis, menorrhagia

• Petechiae & hemorrhagic bullae in mouth

What is the management for ITP?

• Observe and reevaluate lab values

• Avoid aspirins, NSAIDS, antihistamines = anemia development

• Avoid trauma prone sports

• Severe cases → IV gamma globulin 2-5 days

• Corticosteroids enhance vascular stability

Describe hemophilia and the different types

• Sex-linked disorder = factors for blood coagulation is missing

• Passed from unaffected carrier females to affected males

• Types:

  • Hemophilia A: factor VIII deficiency

  • Hemophilia B: factor IX deficiency

  • Von Willebrand disease: 1% pop; prolonged bleeding time

What do you look for in regards to hemophilia?

• Circumcision = prolonged bleeding

• Inc age = inc incidence of bleeding from trauma

• Bleeding in joint spaces & Intracranial bleed = most dangerous

• Present symptoms, Prolonged activated PTT & dec lvl of factor VIII / IX

Describe the management for hemophilia

• FFP & cryoprecipitate (single blood donor w/ special freezing)

• 2nd gen factor VIII

• Fibrin glue (mixture fibrinogen & thrombin)

• Prevent bleeding

  • If occurs → cold compress & pressure, immobilize site of bleeding

Describe DIC (disseminated intravascular coagulation)

• Acquired coagulopathy = thrombosis & hemorrhage

• S/s:

  • Bleeding

  • Resp = hemoptysis, tachypnea, dyspnea and chest pain

  • Skin = petechiae, ecchymosis, jaundice, acrocyanosis and gangrene

How do we manage DIC?

• Avoid trauma to delicate tissue areas

• Injections & iv sites treated as arterial stick

• Administer clotting factors, platelets, and cryoprecipitate to prevent hemorrhage

• Anticoagulation therapy (heparin) = controversial in children as hemorrhage is a concern

What is the difference between child and adult cancer?

• Child:

  • Affects tissues

  • Common sites = blood, lymph, brain, kidney, muscle

  • Prevention detection = incidental/accidental

  • Extent of disease = metastasis present at diagnosis

  • Very responsive to tx

• Adult:

  • Affects organs

  • Common sites = breast, lung, prostate, bowel, bladder

  • 80% preventable

  • Early detection possible

  • Less responsive to tx

What are common types of childhood cancer?

• Blood: leukemia, hodgkin disease, non-hodgkin lymphoma

• Brain: medulloblastoma, brainstem glioma, ependymoma, astrocytoma

• Other: neuroblastoma, osteosarcoma, wilms tumor, rhabdomyosarcoma, retinoblastoma

Describe leukemia

Bone marrow disorder, normal elements are replaced with abnormal WBC

Describe acute lymphoblastic leukemia (ALL)

• Most common form of leukemia

• Immature lymphoblasts & bone marrow cant keep normal levels of components of blood =

  anemia, thrombocytopenia

• Risk factors: male 2-5 yo, sibling w/ leukemia

Describe acute myelogenous leukemia (AML)

• 2nd most common and peaks in adolescence

• Affects myeloid cells in bone marrow and creates malignant cells

• Less responsive to tx

Describe lymphomas and the different types

• Tumors of the lymph tissue (lymph nodes, thymus, spleen)

• Types:

  • Hodgkin disease

  • Non-hodgkin lymphoma

Describe Hodgkin disease

• Malignant B lymphocytes multiply and grow in lymph tissue

• Affects nodes closer to body surface (cervical, axillary, inguinal)

• Cause being researched but linked to epstein-barr virus

• S/s: enlarged lymph nodes, weight loss, night sweats, anorexia, malaise

Describe non-hodgkin lymphoma (NHL)

• B & T lymphocyte mutation = uncontrolled growth

• Affects nodes deeper in body

• Spreads by bloodstream

• Tx: chemo and bone marrow transplant

Describe medulloblastoma

• Located at cerebellum

• Invasive, highly malignant, grows rapidly

• Progresses quick to ICP

• Peak incidence = 5-10 yo

Describe brainstem glioma

• Aggressive, difficult to resect & resistant to chemo

• Spreads widely within brainstem affecting cranial nerve function

Describe ependymoma

Varying speed of growth / diagnosed before spread often cause hydrocephalus

Describe astrocytoma

• Slow course w insidious onset

• Responsive to chemo & respectable

• Low grade = removed easy / high grade = poor prognosis

What are the s/s of a brain tumor?

• N&V, headache, blurred/double vision, seizures, unsteady gait, swallowing difficulties

• Observe for strabismus/nystagmus, sunsetting eyes, head tilt

How do you manage brain tumors?

• Monitor for ICP, admin dexamethasone to decrease intracranial inflammation, prevent BM straining

• Regulate fluid admin as excess can worsen cerebral edema

• Assess neuro VS

• Position child on unaffected side of with bed flat and side position is preferred

Describe neuroblastoma

• Occurs in abdomen and mainly adrenal gland

• Staging determines course of treatment

• S/s: swollen/asymmetric abdomen, neck and facial swelling, edema around eyes, bruising

Describe osteosarcoma

• Common sites are in long bones

• S/S: limping, ROM limited, dull bone pain, gait changes

• Tx: removal neccessary = chemo before surgery to dec size

Describe rhabdomyosarcoma

• Soft tissue tumor from cells that form striated muscle

• Common locations = head, neck, GU tract, extremities

• Highly malignant

• S/S = lump or swelling in affected area

Describe wilms tumor

• Most common renal tumor usually only affecting 1 kidney

• Rapid growth & large @ diagnosis

• Metastasis via bloodstream

• Tx: surgical removal, radiation/chemo

Describe retinoblastoma

• Highly malignant tumor that arises from embryonic retinal cells

• Diagnosed by 5; 5 yr survival rate = 90% when confined to retina

• Can grow into vitreous cavity = retinal detachment

What are the s/s and treatment for retinoblastoma?

• S/s: “cat’s eye reflex” or “ whitewash glow” (leukocoria) to affected pupils

• Tx:

  • Remove tumor, preserve vision

  • Radiation, chemo, laser, cryotherapy

  • In cases of massive tumor w retinal detachment = removal of eye is necessary