cancer

Peripheral blood stem cell transplant (PBSCT) is also used in children with cancer  CSF given prior to transplant

Apheresis involves blood components removed from a patient, separated & then either returned to the bloodstream or replaced with donor blood products

chemo— interfering with the function or production of nucleic acids, DNA, or RNA.

Alkylating; Antimetabolites; Plant alkaloids; Antitumor antibiotics

Biologic Therapy- uses substances made from living organisms, derived from living organisms

  • immunotherapy or biologic response modifier therapy (enhance the immune system)

  • those that use antibodies or segments of genetic material to target cancer cells directly (use monoclonal antibodies to bind to cancer cells)

  • targeted therapies (blockers)

Leukemia (Most common): Unrestricted proliferation of immature WBC in blood forming tissues; inc in immature WBC rather than mature blood cells

  • They do not destroy normal cells but rather compete for metabolic elements → Infiltration into spleen and liver

Acute Lymphoblastic Leukemia

Leukemic cell characteristics, Initial WBC count, CNS or testicular involvement, Down syndrome

Dx. bone marrow aspiration and biopsy, smear (LYMPHOBLASTS)

RFS: Prenatal X-ray exposure, previous chemo, genetic issues like DS

Bone Marrow Infiltration (Leukemia)

  • ↓ RBCs → anemia

  • ↓ Neutrophils → infection risk (reverse isolation) Fatigue/illness that doesn’t resolve

  • ↓ Platelets → bleeding risk (bleeding precautions)

  • ↑ WBC (>10,000/mm³) in ~50% IMMATURE??

Organ infiltration

s/s: FEVER, Hepatosplenomegaly / splenomegaly, Lymphadenopathy

Insidious onset. (slow, vague symptoms that persist or worsen)

Tx: medication adherence because anything less than 95% adherence increases the risk of relapse

  • Induction (4–5 weeks): steroids + chemo → achieve remission

  • CNS prophylaxis / consolidation: intrathecal chemo ± systemic chemo; high risk may get cranial radiation

  • Remission: <5% blasts in bone marrow, no detectable disease 

  • Critical period: very immunosuppressed → infection + bleeding risk

  • Post-remission therapy: interim maintenance → delayed intensification → maintenance

  • Common protocol: BFM (“Berlin-Frankfurt-Münster”) backbone- drug regimen for maintenance, less intensification/continuation post tx? - STRICT MEDICATION ADHERENCE <95% ↑↑↑↑ RISK FOR RELAPSE

Acute Myeloid Leukemia 

s/s: Petechiae for ALL, leukemia cutis for AML (blue purple), chloromas around eyes(blue green)- all painless lumps, bleeding gums in AML, losing weight and appetite

Dx: Hx, physical, blood smear (AUER RODS), bone marrow aspiration or biopsy

Tx: chemo: P1- induction x2 P2- post-remission consolidation/intensification x2 CNS tx is the same? intrathecal chemotherapy is given every 1 to 2 months during intensification (P2)- not needed

For both  AML,ALL: anemia, thrombocytopenia, neutropenia

AML

ALL

Most common in

Older adults

Children

CBC

↓ RBC, ↓ WBC, ↓ platelets

↓ RBC, ↓ WBC, ↓ platelets

Blood smear

Auer rods

Lymphoblasts

Bone marrow

>20% myeloblasts

>20% lymphoblasts

Key tests

DIC labs

Flow cytometry (B vs T cells)

Major risks

DIC, leukostasis

CNS spread, SVC syndrome (T-cell)

Lymphomas- Hodgkin- lymphoid and hematopoietic systems neoplastic disease; originates in lymphoid system & involves the lymph nodes -> spleen, liver, bone marrow, lungs, & mediastinum

15-19 , RARE BEFORE 5

Dx. CT of neck and chest, and CT or MRI of abdomen and pelvis, CBC, ESR, CRP

Stage 1- regional , 1 lymph node only

Stage 2- cancer in 2 or more lymph nodes same side of diaphragm

Stage 3- both sides of the diaphragm so above and below

Stage 4- spread outside the lymph nodes, so organs, bone marrow and lungs

Hodgkin Lymphoma

Reed-Sternberg cells: large abnormal multinucleated B cells with an “owl-eye” appearance; hallmark of Hodgkin Lymphoma.

s/s. Painless lymph node enlargement (often supraclavicular) 

  • Mediastinal nodes → cough

  • Retroperitoneal nodes → abdominal pain

  • B symptoms: fever, night sweats, weight loss, fatigue, pruritus

Tx. cure; chemo, 

Radiation (involved/extended (involved areas plus adjacent nodes),total nodal (entire axial lymph node system))

  • N/V, weight loss, skin/mucosal irritation

  • Fatigue

  • Infertility / delayed puberty

Non-Hodgkin Lymphoma (more common in kids)

Risk factors: EBV, HIV, immunodeficiency, prior cancer tx -> Diffusion spread rather than nodular, early dissemination -> involve CNS or bone marrow → leukemia-like symptoms+ painless swollen lymph nodes in neck, armpit, and groin 

  • Aggressive B-cell

  • Lymphoblastic

  • Anaplastic large cell

Dx. imaging, lumbar puncture, bone marrow biopsy, 

  • I: single site

  • II: same side of diaphragm

  • III: both sides / extensive disease

  • IV: CNS or bone marrow involvement

Tx. lymphoblastic lymphoma [corticosteroids], diffuse mature B-cell lymphoma [Surgery Stage I–II], anaplastic large cell lymphoma [surgery (stage I) and chemo]

Tx. comp.: 

Acute Tumor Lysis Syndrome (TLS): Rapid breakdown of cancer cells → release of cell contents into blood

  • ↑ uric acid, K, PO4, urea; ↓ calcium → can lead to acute kidney failure + death

  • s/s. N/V, fatigue Flank pain, low urine output, Itching, tetany, confusion

Hyperleukocytosis: WBC >100,000/mm³

  • Thick blood → vessel blockage

  • s/s. Respiratory distress, cyanosis, Neuro changes

  • Tx. Hydration, Urine alkalinization, Allopurinol, Leukapheresis (if severe)

Superior Vena Cava (SVC) Syndrome: Chest mass or central line blocks blood flow -> airway + breathing problems

DIC: Widespread clotting → uses up clotting factors -> bleeding + clotting at same time -> Life-threatening emergency

NSG Considerations: 

  1. Infection: ANC <500 = severe infection risk; Monitor for fever, dehydration, malaise, seizures; left shift in WBC (↑ bands)

  2. Pain: pharmacologic + nonpharmacologic pain control

  3. Bleeding: Major bleeding risk when platelets ≤20,000 -> Prevent/control with platelet transfusions

  4. N/V: Treat with 5-HT3 antagonists (serotonin blockers)

  5. Nutrition: Individualized nutrition plan, Monitor intake/weight,

  6. Mucositis/Stomatitis: Painful mouth/GI ulcers from chemo: Oral ulcers (stomatitis) are red, eroded, painful areas in the mouth or pharynx.

  7. Anemia: From marrow replacement or chemo myelosuppression

  8. Neuro/Steroid Effects: Vincristine/vinblastine → constipation, foot drop, numbness, jaw pain; Steroids → Cushingoid appearance, mood changes, fluid retention

Retinoblastoma—Congenital inherited malignant tumor arising from the retina

s/s. Classic sign - whitish glow in the pupil - cat’s eye reflex or

leukokoria - light illuminates the

tumor for a moment

 Strabismus of impaired eye

 Blindness - late sign

 Red painful eye accompanied by

glaucoma