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Childhood Leukemias
APHON: Childhood Leukemias
| Question | Answer |
|---|---|
| What is Leukemia? | Disease of white blood cell proliferation. Maligant cell = blast. Blasts do not mature & crowd out normal cells |
| How is leukemia classified? | 1) onset/ course of illness: acute/chronic 2) cell lineage myloid/lymphoid EG ALL, AML, CML |
| Def: acute leukemia | Sudden onset. Death in weeks to months if untreated. |
| Def: chronic leukemia | insidious onset, death in years if untreated |
| name the two lyphoid cell lines | B & T lymphocytes |
| name the 4 cells in the myeloid cell line | platelets, RBC, neurtophils, macrophages |
| children at increased risk for leukemia (4) | Down syndrome, neurofibromatosis type I, fanconi anemia, bloom syndrome |
| Peak age of occurance of ALL? Incidence? | 2-4 years (75% of all leukemia, 25% of all childhood cancer) |
| Peak age of occurance of AML? Incidence? | Neonate & teen (% of all leukemia, 25% of all childhood cancer) |
| Peak age of occurance of CML? Incidence? | None. <5% of all childhood leukemia, <1% of all childhood cancer |
| Pathophysiology of Leukemia? | Genetic damage to single bone marrow cell Uncontrolled proliferation of blasts Decreased production of normal cells Accumulation of blasts in body organs/tissues |
| Clinical presentation of leukemia? | Fatigue, pallor, anorexia (anemia, Bruise/ bleeding (thrombocytopenia &/or DIC) Fever, infection (neutropenia), Bone/joint pain, Abdominal pain (hepatosplenomegaly or mesenteric adenopathy) H/A, V, vision change(CNS involvement) |
| (7) Clinical presentation signs of Leukemia | Lymphadenopathy, Hepatosplenomegaly, cranial nerve palsies, testicular involvement, chloromas, leukemiacutis, gingival infiltration |
| What is a "Chloromas"? | Leukemic cell infiltration into other tissues. Named because the tissue turns greenish when exposed to air. Massive range of apperence: papule, nodule, papura/ patecia, open wound/lesion. |
| In which type of leukemia are is leukemic inflitration into other tissues most common? | AML> ALL Eg gingival infultration, leukemia cutis, chloromas. |
| What is Juvenile Myelomoncytic Leukemia (JMML)? | Rare form of eukemia seen exclusively in very young children(<4yrs. Poor prognosis, SCT only |
| Clinical features of Juvenile Myelomocytic Leukemia? | HSM, lymphadenopathy, fever/ pallor/ rash. |
| Lab findings of Juvenile Myelomocytic Leukemia? | Leukocytosis (WBC >10K), monocytosis (>1000/mm3), increased fetal hemoglobin (>10%) |
| What is Myelodysplastic Syndromes (MDS)? | "preleukemia" Ineffective hematopoiesis with dyplasia,starts in one cell line, progresses to pancytopenia, & BM failure. "transforms" into AML |
| 3 types of ALL | Pre-B (84%)Most common type Commonly seen in preschoolers, T-cell (15%)Assoc. with high WBC,assoc. with mediastinal mass Commonly seen in adolescent males, B-cell/burkitt's(1%)Responds poorly to standard ALL therapy Tx same as for Burkitt’s lymphoma |
| Diagnostic W/U | CBC, Chem 20, pt/ptt, D-dimer, Virus titers, quantitative immunoglobulins, HLA typing, CXR (r/o mediastinal mass, Bone marrow analysis: morphology (inc blast %, cellularity & cell lines), cytochemistrym immunophenotyping, cytogenetics, |
| Cell classification of Leukemia | Cells classified according to the FAB (French-American-British) system ALL: FAB L1 – L3 AML: FAB M0 – M7 |
| Bone Marrow Analysis: Cytochemistry | Helps to differentiate cell lineage (AML versus ALL) Helps to differentiate AML subtypes |
| Bone Marrow Analysis: Immunophenotyping | Identifies markers (antigens) on blast cells Helps to differentiate: ALL versus AML T versus B lineage ALL Certain subtypes of AML |
| Bone Marrow Analysis: Cytogenetics | Analysis of leukemic cell chromosomes Chromosome number (ploidy) Chromosome structure - Deletions - Translocations |
| Diagnostic Workup: Lumbar Puncture | Standard component of workup for acute leukemia Cell count # WBC & RBCs per mm3 of CSF Cytology Presence/absence of blasts in CSF Initial intrathecal chemotherapy often administered during diagnostic tap |
| Types of ALL | Pre-B cell (84%) Most common type; Pop= preschoolers. T-cell (15%) Assoc with high WBC.Often assoc with mediastinal mass. Pop= adolescent males. Mature B-cell/ Burkitt's (1%)Responds poorly to ALL therapy Treatment same Burkitt’s lymphoma |
| Common Immunophenotypes ALL with B-lineage | CD 19, CD 22, CD 24, CD 79a, HLA-DR |
| Common Immunophenotypes ALL with T-lineage | CD 2, CD 3, CD 5, CD 7 |
| Common Immunophenotypes in all ALL subtypes | CD 10 (cALLa) – positive in 80-90% TdT – positive in most subtypes |
| Cytogenetics in ALL: Ploidy | Ploidy = number of chromosomes Normal – diploid (46) Hyperdiploid – extra copies of chromosomes (usually favorable) Hypodiploid – missing copies of chromosomes (always unfavorable |
| Cytogenetics in ALL: Two favourable structural genetic changes | 1)Type: t(12;21), Associated Gene Product: TEL-AML1. 2)Type: Trisomy 4,10,17. Associated Gene Product: none. |
| Cytogenetics in ALL: Two unfavourable structural genetic changes | 1) Type: t(4;11) Associated gene product: MLL 2)Type: t(9;22). Associated Gene Product: BCR/ABL (philadephia chromosome). |
| Childhood ALL: Unfavourable prognostic factor | WBC at dx >50,000. |
| Childhood ALL: Favourable prognostic factors | Age 1-9urs. Rapid early responce to tx. Correct tx for dx (properly identified cells) |
| Childhood ALL: Therapy | Risk-directed therapy Based on risk of relapse “Standard” versus “High” risk Therapy intensified for “slow” responders Tailored therapy minimizes late effects |
| Childhood ALL:Bone Marrow Rating | M1 < 5% blasts M2 5-25% blasts M3 > 25% blasts |
| Childhood ALL: Remission | Absence of clinical signs of disease < 5% blasts in marrow with normal cellularity (M1 marrow) Near normal peripheral blood counts: ANC > 500 Plts > 100K |
| ALL Therapy: Phases | Induction Consolidation/Intensification Maintenance/Continuation CNS-Directed Therapy Length of treatment usually 2 to 3 years |
| ALL: Induction | First phase of treatment (usually 1 month) Goal = remission Agents: Glucocorticoid, vincristine, asparaginase, + anthracycline + intrathecal chemotherapy > 97% of patients achieve remission by end of Induction |
| ALL: Consolidation/Intensification | Intensified treatment (to solidify remission) Agents with varying mechanisms of action, such as: Cytarabine, methotrexate, doxorubicin, etoposide, cyclophosphamide Goal = eradicate most of remaining leukemia |
| ALL: Continuation/Maintenance | Goal = to “maintain” remission Usually antimetabolite-based - Daily mercaptopurine - Weekly methotrexate Intermittent pulses - Vincristine + glucocorticoid |
| ALL: CNS Prophylaxis | Intrathecal chemotherapy All treatment phases + High dose IV methotrexate and/or cytarabine Cranial irradiation High risk patients only |
| ALL: CNS Therapy | Treatment for CNS leukemia - Blasts in CSF at diagnosis - Clinical signs of CNS disease Goal: Eradicate CNS leukemia Cranial +spinal radiation Intrathecal + high dose IV cytarabine and/or methotrexate |
| PCP Prophylaxis | To prevent Pneumocystis carinii pneumonia (PCP) TMP/SMX (Septra®/Bactrim™) 2-3 days/week Dapsone daily/weekly Pentamidine via aerosol or IV monthly |
| ALL: Management of Relapse | Treatment depends on timing of relapse: Early (on therapy) Late (>6 months off therapy) Management challenging Prognosis often poor |
| Childhood ALL: Nursing Care | Tumor Lysis Syndrome Leukostasis Bleeding Fever and Infection Providing information Caring for child/family Preserving child’s uniqueness and normalcy Conquering fears |
| Childhood ALL: Maintaining Normalcy | School Play Growth Development Discipline Belief in future Living fullest life possible Coping with survival |
| Acute Myeloid Leukemia | Rapidly progressive disease characterized by replacement of bone marrow with: Malignant cells Of myeloid origin |
| Myeloid cell line | Plt, RBC, Neutrophils, Macrophages |
| Morphology in AML: FAB Classification | M0:Undifferentiated myelocytic M1:Poorly differentiated myelocytic M2:Myelocytic M3:Promyelocytic M4:Myelomonocytic M5:Monocytic M6:Erythroleukemia M7: Megakaryocytic |
| Cytochemistry in AML | Cytochemical stains useful in confirming AML diagnosis Myeloperoxidase (MPO) Usually strongly positive Sudan Black B (SBB) Usually positive Esterase stains Help distinguish granulocytic versus monocytic lineage |
| Immunophenotyping in AML | Common myeloid-associated antibodies include: CD 13, CD 14, CD 15 CD 33, CD 34, CD 65 HLA-DR One of these antibodies is expressed in the majority of AML cases |
| Three favourable Cytogenetic Abnormalities in AML | 1) t(8;21)associated with M1, M2 2) t(15;17) associated with M3 3) inv 16, associated M4 |
| two unfavourable cytogenetic abnormalities in AML | 1) t(9;11), 11q23 associated with M4,M5 & therapy -realated AML 2) FLT3, ITDs. No assoicated substype. |
| Childhood AML: Favourable Prognostic Factors | Down Syndrome, M3 (promyelocytic) FAB subtype. |
| Childhood AML: Unfavourable Prognostic Factors | WBC >100,000, Treatment-related AML |
| Childhood AML:Bone Marrow Rating | M1 < 5% blasts M2 5 - 29% blasts M3 > 30% blasts |
| Childhood AML: Remission | Absence of clinical signs of disease M1 marrow < 5% blasts Evidence of trilineage recovery Recovery of peripheral blood counts ANC > 1000 Plts > 75K |
| AML Therapy: Phases | Induction Post-Remission Therapy Intensive chemotherapy Hematopoietic cell transplant Usually no “Maintenance” phase Length of treatment usually < 1 year |
| AML: Treatment | Very intensive therapy required To achieve remission To attain cure Intensive timing of induction therapy improves survival Therapy usually includes: Anthracyclines + cytarabine CNS-directed therapy,Lengthy hospitalizations expected |
| Role of Stem Cell Transplant: AML in First Remission | Allogeneic matched related HCT If donor available Autologous or unrelated HCT Sometimes used if related donor not available |
| Role of Stem Cell Transplant:Relapsed AML | Alternative donor source often used Unrelated Mismatched related If relapse occurs after transplant, options may include: Donor lymphocyte infusion Second transplant |
| Acute Promyelocytic Leukemia (APL) | Blasts release procoagulants -> DIC that worsens with chemo. Remission induction with ATRA (Differentiating agent,PO, reduces DIC incidence/severity)Improves survival. ATRA not curative. Consol with chemo needed. Mainten tx= 1yr. Arsenic may be used |
| Down Syndrome & leukemia | Favorable prognosis in AML Better response to therapy Less intensive treatment indicated FAB-M7 subtype more common |
| AML: Potential Complications | Hemorrhage/DIC Leukostasis (WBC>200,000) Tumor lysis syndrome Prolonged pancytopenia Infection/sepsis Bacterial (alpha strep) Viral (HSV) Fungal |
| AML: Supportive Care | Placement of Hickman/Broviac Vancomycin, antifungals, Avoid steroid, Acyclovir prophylaxis For HSV+ patients Prophylactic IVIG Hepafiltered/positive air flow room Aggressive antiemetic regimen Nutritional support Enteral (NG/NJ)(TPN) Oral care |
| Chronic Myeloid Leukemia (CML) | High WBC (often >100K) Often asymptomatic Splenomegaly Ph chromosome + Phases: Chronic Accelerated Blast Crisis |
| CML: Treatment | Hydroxurea, Busulfan, Alpha Interferon, Cytarabine in chronic phase (not curative) HCT curative for ~80% with matched sibling donor if transplanted within 1 year of dx. Gleevec: daily oral therapy AML/ALL therapy for blast crisis (palliative |
| Myelodysplastic Syndromes (MDS) | Ineffective hematopoiesis with dysplasia May start in one cell line Usually progresses to pancytopenia and bone marrow failure Transformation to AML common Sometimes called “preleukemia” |
| Juvenile Myelomonocytic Leukemia (JMML) | Rare leukemia of early childhood. Presentation: HSM, Lymphadenopathy Fever, pallor, skin rash Lab features:Leukocytosis (WBC > 10K) Monocytosis (>1000/mm3) fetal hemoglobin (> 10%) Prognosis generally poor Allogeneic HCT is only known cure |
Created by:
JennRN
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