Hematologic and Neoplastic Disorders

Cellular regulation

process by which cells replicate, proliferate, and grow.

Hematologic system

• consists of blood and blood forming tissues of the body

• Integrally involved in cellular regulation

Red Blood Cells (RBC)

transport nutrients and O₂ to body tissues and waste products from the tissues

White Blood Cells (WBC)

fight infection

Platelets

clotting

Alterations of cell regulation

• Problems are often related to

  • Production of blood cells (too much or too little)

  • Loss and destruction of cells

• Neoplastic cells- cells that abnormally proliferate

• Cancer results from alteration in cellular regulation resulting in out-of-control cell growth

Pediatric variations

• Hemoglobin (Hgb): Hgb A, Hgb A₂, Hgb F

•  After 6 mos, Hgb A is predominant

•  In neonates, Hgb F has higher quantities, which puts infants at risk for anemia and leads to problems with the O₂ carrying capacity

•  In fetus, blood cells formed in liver. Production of cells then transfers from liver to the bone marrow of long and flat bones. Balance between oxygenation and production may be affected during this time.

•  Physiologic anemia in infancy

Health Hx

inherited d/o’s, immune d/o’s, genetic d/o’s, lead exposure risk

HPI

 fatigue, pallor, unusual bleeding, petechiae, excessive bleeding, Pain (PQRST), frequent infections, morning HA’s with n/v, gait changes, visual disturbances, hx of non-traumatic bone fractures

Objective findings

thin, frail, altered LOC, pale mucous membranes, asymmetry or masses of face, thorax, extremities. Pallor, clubbing, bruising, petechiae, decreased output, rectal bleeding. Murmurs, abnormal breath sounds, pulse strength, lymphadenopathy, cap refill, hepatosplenomegaly, joint tenderness, limited ROM

Diagnostic testing

• Complete Blood Count (CBC)

• RBC count

• Hemoglobin (Hgb)

• Hematocrit (Hct)

• MCV, MCH, MCHC, RDW

• WBC count

• Platelet count

• MPV

Anemia

• Anemia- levels of RBC’s and Hgb are lower than normal

• Nutritional deficiencies ** most common type in children

  • Iron deficiency

  • Folic acid deficiency

  • Pernicious anemia

• Toxin exposure

• Adverse medication reaction

• Hemolytic anemia

  • Sickle cell

  • Thalassemia

Iron Deficiency Anemia

 Body does not have enough iron to produce Hgb

Iron Deficiency Anemia S/sx

weakness, fatigue, SOB, pallor, systolic murmur, brittle, spoon-shaped nails, cognitive delays, behavioral changes

Iron Deficiency Anemia risk factors

Maternal anemia during pregnancy, prematurity, cow’s milk before 12 mos or excessive cow’s milk consumption, lack of iron supplement after 6 mos in breast-fed infants, acute blood loss, restricted diets, antacid use, low SES, heavy menses

Iron Deficiency Anemia lab values

◦Decreased RBC count, Hgb, Hct

◦Decreased MCV, MCH, MCHC (microcytic, hypochromic)

◦Decreased serum ferritin

Iron Deficiency Anemia management

• Parent education

• Iron supplementation

  • Liquid must be placed behind teeth or with straw

  • Vit C increases absorption

  • Constipation, GI upset common

  • Give 1 hr before or 2 hrs after milk or antacid

Lead Poisoning

• Usually ages 1 to 5 years old

• Caused by ingestion of lead

• Lead in the blood interferes with biosynthesis of heme

• Causes hypochromic, microcytic anemia

Lead Poisoning child appearance

• Child may exhibit behavioral problems, learning difficulties, fatigue, abdominal pain

• High levels may have encephalopathy, seizures, brain damage

Lead Poisoning > 3.5mcg/dL

• requires follow-up

• Lead exposure must be decreased; elevated lead requires close follow-up and health dept referral

Lead Poisoning > 45

• will require chelation

• Chelation removes lead from soft tissue/bone and excreted through renal system

• Monitor I’s and O’s and ensure hydration

Sickle Cell Anemia (Autosomal recessive)

• Most common type of sickle cell disease

• Newborn screening mandatory in all 50 states

• Hgb electrophoresis is definitive diagnostic test

• 1 in 400 black people has SCA, Black people primarily affected

• Hgb SS instead of Hgb AA

• Sickle cell trait has Hgb AS

• Infants asymptomatic until 3-4 mos because of protection of Hgb F

What happens to the body when sickle cells occurs?

• RBC’s sickle, usually because of stress—> fever, acidosis, dehydration, physical exertion, cold exposure, hypoxia

• Cells sickle

• Blood more viscous because sickled cells clump together

• Prevents normal blood flow to tissues of that area

• Vaso-occlusion leads to local tissue hypoxia, followed by ischemia, and possibly infarction

• Pain crisis results due to decreased circulation

• Clumping cells in lungs (Acute Chest Syndrome) aka ACS

• Splenomegaly, abdominal pain, anemia

Acute Sickle Cell Anemia

• acute chest syndrome

• aplastic crisis

• bacterial sepsis or meningitis

• bone infacrtion

• dactylitis

• hematuria

• recurrent pain episodes

• pain crisis

• splenic sequestration

• stroke

• priapism

Chronic Sickle Cell Anemia

• Anemia

• Avascular necrosis of the hip

• Cardiomegaly, functional murmur

• Cholelithiasis

• Delayed growth and development

• Delayed puberty

• Functional asplenia

• Hyposthenuria (low urine specific gravity) and enuresis

• Jaundice

• Leg ulcers

• Proteinuria

• Pulmonary hypertension

• Restrictive lung disease

• Retinopathy

Sickle Cell Anemia prevention

Educating family and child: health maintenance and immunizations, prophylactic PCN, febrile illness, adequate fluid intake, signs of pain crisis developing

Sickle Cell Anemia management

• Pain assessment: always BELIEVE child

• Moderate to severe pain usually need opioids/PCA pump

• NSAID’s/acetaminophen for less severe

• Relaxation, play, massage, music may help

• Treat underlying condition (infection, injury)

• Increase fluid intake

• Maintain appropriate electrolytes and pH

• Evaluate respiratory and circulatory status

• Monitor LOC

• May need blood transfusions (Usually packed RBC’s)

• Prevent infection

  • 2 mos-5 yrs usually receive oral penicillin V potassium as prophylaxis against pneumococcal infection (erythromycin if allergic)

  • Immunizations: in addition to schedule: PCV-23 annually after 2 yr old

β-thalassemia major (Cooley anemia) (Inherited, autosomal recessive)

• Primarily affects those of African descent and Middle Easterners

• The β-globulin chain in hemoglobin synthesis is reduced or absent

• Large number of unstable globulin chains accumulate, causing the RBC’s to be rigid and hemolyzed easily resulting in severe anemia and chronic hypoxia

• To compensate for the hemolysis, an overabundance of erythrocytes are formed causing massive bone marrow expansion and thinning of bony cortex

• Hemosiderosis causes bronze skin and altered organ function

β-thalassemia major (Cooley anemia) tx

• Monitor Hgb and Hct, blood iron

• Transfusing PRBC’s at regular intervals

  • Maintains adequate level of Hgb for O2 delivery to tissues

  • Suppresses erythrocytosis in the bone marrow

  • Monitor for reactions to transfusions

• Chelation therapy

  • Removes excess iron (hemosiderosis)

  • Administer the chelating agent deferoxamine with the transfusion (or orally)

• Educating family: must understand importance of transfusions and chelation therapy, genetic counseling, family support group

Idiopathic Thrombocytopenia Purpura (ITP)

• Immune response following viral infection that produces antiplatelet antibodies which destroy platelets

• Causes petechiae, purpura, and excessive bruising

• Most children spontaneously recover, but complications include severe hemorrhage

Disseminated Intravascular Coagulation (DIC)

• Usually occurs in critically ill children

• Triggers: septic shock, tissue necrosis/injury, cancer tx

• Secondary condition so must address triggering event

• Nurses must be vigilant in observing for changes

Hemophilia (X-linked recessive)

•  Hemophilia A is most common, factor VIII deficiency

•  Factor VIII is essential in activating factor X, which is required for conversion of prothrombin into thrombin. If deficient, results in inability of platelets to be used in clot formation

•  Hemophilia classified by severity

•  Primary goal is to prevent bleeding

Hemophilia Prevent

• Need regular exercise to strengthen joints and muscles (swimming, bicycling)

• Avoid activities with high injury potential (football, skateboarding, gymnastics)

• Toddlers may need soft helmets and padding in home

• Hemlibra (emicizumab) subcutaneous injections

  • Causes lab errors in PTT and certain Factor VIII assays

Hemophilia Managing

• Administer factor VIII replacement IV push per order

• Desmopressin for mild hemophilia A

• Apply pressure with external bleeding

• If inside joint, apply cold compresses and elevate unless contraindicated

Hemophilia Education

• Child sure wear medic alert, school to be aware

• Parents and caregiver instruction on administering intravenous factor VIII

  • Involve children as developmentally appropriate

Hemophilia Labs

• Prolonged partial thromboplastin time (PTT)

• Platelets and PT normal

• Factor-specific assays determine deficiency

Hemophilia Objective data

• Hemarthrosis

• Hematomas

• Hematuria

• Epistaxis

• Bleeding gums

• HA’s, decreased LOC

Von Willebrand Disease

• Genetically transmitted bleeding disorder affecting both genders, all races

• Deficiency in von Willebrand factor (vWF), causing mild bleeding disorder

• Children bruise easily, epistaxis, bleeding gums, menorrhagia in females

• Nursing management is the same as hemophilia

  • Major difference is administration of intranasal desmopressin with bleeding episode

  • vWD intravenous infusion in some cases

Cancer

• Cancer results in an alteration in cellular regulation resulting in out-of-control cell growth

• Accounts for most deaths in children over 1 year of age

Most common childhood cancers

◦Leukemia

◦CNS tumors

◦Lymphoma

◦Neuroblastoma

◦Rhabdomyosarcoma

◦Wilms tumor

◦Bone tumors

◦Retinoblastoma 

Childhood cancer usually affects tissues (blood, lymph, bone), has very little environmental influence, very responsive to treatment, and metastasis often present at diagnosis

Cancer chemotherapy

◦Drugs divided into classes based on slightly different actions and effect of different portions of the cell cycle

◦Combination of drugs common

◦Disrupt not only cell cycle of cancer cells but also normal rapidly dividing cells (digestive system, reproductive system, hair follicles)

Cancer Chemotherapy Nursing

◦Provide antiemetics prior to chemo

◦Observe for mouth ulcerations

◦Offer cool fluids

◦Educate family/child about side effects

◦Encourage immunizations, infection control

Cancer Radiation Therapy

◦Uses high-energy radiation to damage or kill cancer cells

◦May affect rapidly growing normal cells

◦Dose-calculated and delivered divided over weeks

◦Adverse effects: fatigue, n/v, oral mucositis, myelosuppression, alterations in skin integrity at radiation site. Long-term complications also

Cancer Radiation Therapy nursing

◦Encourage loose clothing

◦Do not wash off marks for targeted areas

◦No deodorants or perfumed lotions

◦Protect skin from sun

Cancer Hematopoietic Stem Cell Transplantation (HSCT)

◦Transplant after abnormal cells purged by chemo and radiation to replace marrow and reestablish hematopoiesis in child

◦Used most often in refractory or advanced disease

◦Adverse effects: infection, electrolyte imbalance, bleeding, long-term complications

Cancer Hematopoietic Stem Cell Transplantation (HSCT) Nursing

◦Prior to transplant: protective isolation in positive-pressure room, limited visitors

◦After transplant: monitor for diarrhea or rash (signs of GVHD)

◦For several months after transplant: monitor and prevent infection, blood products as needed, family support

Common labs and diagnostics in oncology

•  α-Fetoprotein

•  Bone marrow aspiration and biopsy

•  Bone scan

•  CXR

•  CBC with differential

•  CT scan

•  Lumbar puncture (LP)

•  MRI

•  Urine catecholamines (VMA, HVA)

•  Ultrasound

Lumbar Puncture (LP)

• Needle is placed in subarachnoid space  of spinal column, below base of cord, and CSF withdrawn for analysis

  • Use EMLA before procedure

  • Position child appropriately

  • Use distraction techniques

  • Encourage child to recline up to 12 hrs after LP

Leukemia

Leukemia is a primary disorder of the bone marrow in which normal elements are replaced with abnormal WBC’s

Acute Leukemia

• rapidly progressive affecting undifferentiated or immature cells

Chronic Leukemia

progress more slowly, permitting maturation and differentiation of cells

Leukemia Early Manifestations

low-grade fever, pallor, increased bruising and petechiae, listlessness, HA, enlarged liver and lymph nodes, constipation, vomiting

Leukemia Late Manifestions

pain, hematuria, mouth ulcerations, increased ICP

Leukemia Labs

 low platelets, anemia, low neutrophils, immature WBCs

Leukemia Tx

chemotherapy, hematopoietic stem cell transplant (HSCT), allogeneic transplant, radiation to brain

Acute Leukemias: Acute Lymphoblastic Leukemia (ALL)

• Most common form of CA in children

• Usually in children 2-10 yrs

• Abnormal lymphoblasts grow excessively and replace normal cells in bone marrow

• Bone marrow expands or leukemic cells can infiltrate the bone and joints, lymph nodes, liver and spleen, CNS

• Overall cure rate is > 70%

Acute Leukemias: Acute Myelogenous Leukemia (AML)

•  Incidence peaks during adolescence

•  Affects myeloid cell progenitors or precursors in bone marrow, resulting in malignant cells

•  Long-term survival is 50%

•  AML is less responsive to tx than ALL

Acute Leukemias: 4 Phase of Chemotherapy

• Induction therapy- rapid induction of complete remission

  • Lasts 3-4 weeks

• Consolidation (intensification)- strengthen remission, prevent leukemic resistant cells from emerging

  • Variable length of time

• Maintenance- eliminate all residual leukemic cells

  • Lasts 2-3 years

• CNS prophylaxis- reduce risk of development of CNS disease

  • Given periodically at each phase

Chemotherapy side effects

• Mucosal ulceration

  • Provide oral care w/ local anesthetics

    • NO viscous lidocaine, hydrogen peroxide, milk of magnesia, lemon glycerin swabs

• Skin breakdown

• Neuropathy

  • Constipation, foot drop, jaw pain

    • High fiber diet, fluids, stool softeners

• Loss of appetite

  • Small, frequent meals, monitor electrolytes

• Hemorrhage cystitis

• Alopecia

Acute leukemia management

◦Infection

◦Pain

◦Anemia

◦Bleeding

◦Hyperuricemia

◦Adverse effects of treatment

neutropenic precautions

Acute leukemia reduce pain

◦From lumbar puncture, bone marrow aspiration

◦May have HA, peripheral neuropathy

◦Use distraction techniques, mild analgesics, EMLA cream, heat or cold to painful area, narcotic analgesics

Lymphomas

•  Lymphoma is a tumor of the lymph tissue (lymph nodes, thymus, spleen)

•  Hodgkin Disease or non-Hodgkin lymphoma

Hodgkin Disease

◦Malignant B lymphocytes grow in lymph tissue, usually starting in one area of lymph nodes

◦Most common in adolescents and young adults

◦S/Sx: wt loss, fever, fatigue, lymphadenopathy, hepatosplenomegaly

◦Prognosis depends on stage of disease

◦Tx: chemotherapy (combo of drugs), radiation possibly, HSCT if no remission or experiences relapse

Non-Hodgkin

◦Mutations of B and T lymphocytes that lead to uncontrolled growth

◦Affect lymph nodes located more deeply in body

◦Spreads rapidly with aggressive malignancy, very responsive to treatment

◦Spreads easily to CNS

◦Usually only symptomatic for days or weeks prior to diagnosis

◦S/sx: abdominal pain, diarrhea, constipation, labored respirations, facial edema, lymphadenopathy, abdominal mass

Brain tumors

• Brain tumors are most common form of solid tumor in childhood

•  Prognosis depends on location and extent of tumor

•  Causes increased ICP and compression of vital structures

Brain tumors tx

 surgical resection if possible, radiation if over 2 yrs old, chemotherapy

Brain tumor s/sx

 n/v, HA, unsteady gait, blurred or double vision, seizures, weakness, behavior changes, motor abnormalities, nystagmus, abnormal pupil reactions, bulging fontanel in infant

Brain tumors nursing

 provide pre-op and post-op care

◦Avoid increase in ICP à steroids, stool softeners

◦Shave head as appropriate

◦Regulate IVF’s, meds to decrease cerebral edema

◦Neuro checks

◦Check head dressings, cool compresses to eyes if incomplete closure

Neuroblastoma

• Malignancy of the adrenal gland, but may occur along the paravertebral sympathetic chain (retroperitoneum, head, neck, pelvis, chest)

• By time of diagnosis, the neuroblastoma has usually metastasized

• 90% of cases diagnosed before age of 5 yrs

• Metastasis to bone is worst prognostic factor

Neuroblastoma tx

 surgical removal of neuroblastoma, chemotherapy, radiation

Neuroblastoma s/sx

depends on location of tumor. Swollen abdomen, bowel or bladder dysfunction, neuro sx, bone pain, weight loss

Neuroblastoma nursing

◦ post-op surgical care

◦Emotional support especially with poor prognosis

Osteosarcoma

•  Most common malignant bone CA in children, most frequently occurring in adolescents at peak of growth spurt

•  Most common sites: long bones

Osteosarcoma s/sx

pain, gait changes, limited motion, erythema, swelling, warmth and tenderness at site of mass

Osteosarcoma tx

 surgical removal

◦Amputation, limb salvage procedure

◦Chemo before and/or after surgery

Osteosarcoma nursing

• Routine orthopedic post-op care

• Emotional support

  • Significant body image changes

  • Peer support groups

Ewing sarcoma

•  Highly malignant bone tumor, more rare than osteosarcoma

•  Occurs most frequently in pelvis, chest wall, vertebrae, and mid-shaft of long bone

•  25% metastasize to lungs, bone and bone marrow

•  Prognosis depends on extent of metastasis

Ewing sarcoma s/sx

 intermittent pain that becomes progressively worse, swelling and edema at tumor site

Ewing sarcoma tx

• radiation, chemotherapy, surgical excision used in combination

  • May need myeloablative chemotherapy followed by stem cell transplant with metastatic disease

Ewing sarcoma nursing

◦Before treatment, discourage active play or weight bearing on affected extremity

◦Therapeutic play while hospitalized

◦Manage adverse affects of treatment

Wilm’s tumor

•  Renal tumor with peak incidence between 2 and 5 years old

•  May grow rapidly and usually large at diagnosis; may metastasize but rare

•  Overall survival rate 90%

•  Associated anomalies: hemihypertrophy of spine, GU abnormalities, absence of iris, family hx of CA

Wilm’s tumor s/sx

abdominal pain or swelling, constipation, vomiting, anorexia, difficulty breathing, HTN (25%)

Wilm’s tumor tx

surgical removal of tumor and affected kidney; radiation or chemotherapy before or after surgery

Wilm’s tumor nursing

◦AVOID palpating abdomen after initial assessment

◦ abdominal post-op care

Retinoblastoma

• Congenital, highly malignant tumor that arises from embryonic retinal cells

• Usually diagnosed by age 5 yrs

• May be hereditary on non-hereditary

Retinoblastoma complications

spread to brain and opposite eye, metastasis to lymph nodes, bone, bone marrow and liver. May develop secondary tumors

Retinoblastoma s/sx

leukocoria, erythema, orbital inflammation, hyphema

Retinoblastoma tx

• radiation, chemotherapy, laser surgery, cryotherapy

• May be able to preserve moderate vision with milder disease

• Enucleation if massive tumor or retinal detachment

Retinoblastoma nursing

large pressure dressing if eye enucleated, eye exams every 3-6 mos, prosthetic eye fitting several weeks after removal, genetic counseling, eye protection