Malouf: Clinical leukemias

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ALL is a disease of what group?

children and young adults

AML is a disease of what group?

most common in adults, though it is seen from childhood through all groups

CML is a disease of what group?

middle-age and older adults (can rarely occur in children/adolescents)

CLL is a disease of what group?

older adults

How does one stage a lymphoma/leukemia?

CT or MRI: allows ID of solid tumors in the brain, chest, abdomen, pelvis, and lymph node chains

lumbar puncture is also done at this time

In children, the primary cancers are ______________ or _______________. ___% are metastatic at the time of dx.

lymphohematopoietic, embryonal
80%

In adults, the primary cancers are ________ in origin. This means that identification of the neoplasia occurs _______.

epithelial
earlier (only 20% have metastasis at the time of dx)

What kind of effects can pediatric oncology have on normal growth?

Radiation increase the risk of adult epithelial cancers
Childhood cancers are mostly more responsive to chemo than adult cancers, and children suffer fewer tx-related side effects than adults.

What is the incidence of ALL?

Accounts for 77% of childhood leukemias

Peak incidence is 2-6 years old, male > female

What is the etiology of ALL?

GENETIC ABNORMALITIES (Down's, Bloom, ataxia-telangectsia syndrome, Fanconi anemia)
IONIZING RADIATION the only causal relationship in U.S.
EBV is causal for B-cell ALL in developing nations

What are the symptoms of ALL?

Non-specific low-grade fever, irritability, anorexia, fatigue
BONE PAIN in lower extremities is less common in early stages

Late stages of BM failure: PALLOR, BRUISING, EPISTAXIS, RECURRENT INFECTION

What do you see on physical exam with ALL?

Pallor, petechiae/purpura, listlessness (BM failure)
Lymph/spleno/hepatomegaly
tender, swollen joints
testicular mass
supine respiratory distress (large anterior mediastinal mass)
papilledema, cranial nerve palsies, retinal hemorrhages (signs of increased intracranial pressure)

What is papilledema?

Swelling of the optic nerve causes blurring of the optic nerve at physical exam

What is retinal hemorrhage?

Bleeding of the blood vessels in the retina, in this case due to low platelet counts

What is cranial nerve palsy?

Increased intercranial pressure causes the cranial nerves to lose partial or complete function

First to be affected is usually CNVI (abducens)

What are the lab findings in ALL?

CBC/peripheral smear:
Anemia, thrombocytopenia
Lymphoblasts

Bone marrow aspirate: >25% cells are homogeneous population of lymphoblasts

CSF analysis to look for lymphoblasts (means additional therapies in treatment plan)

What is the treatment for ALL?

Initial treatment = INDUCTION - goal is to eradicate leukemic cells from the marrow (a.k.a. REMISSION)
~ 4 weeks

Phase 2 (CONSOLIDATION) involves lumbar puncture/chemo to prevent CNS relapse.
Patients with t(9;22) (& some others) receive bone marrow transplantation

Phase 3 (MAINTENANCE) (~2-3 years) = monitoring for relapse (15-20% of pts)

Overall survival > 80% at 5 years

What are the side effects of ALL treatment?

tumor lysis syndrome, renal failure, myelosuppression, infection (immunosuppressed, plus central line)

What can be given for tumor lysis syndrome?

allopurinol to remove uric acid
loop diuretic for calcium and phosphate
bicarb to reverse hyperkalemia
FLUIDS
if no improvement, then hemodialysis

What is the incidence of AML?

most common adult leukemia in adults (80%), mostly male
11% of childhood leukemias

More common with genetic abnormalities (Down's, Bloom, Fanconi anemia, ataxia-telectangiectsia, others)

What is the etiology of AML?

IONIZING RADIATION, chemotherapy, organic solvents
possibly ↑ risk in smokers; genetics

What are the symptoms of AML?

signs of marrow failure
complaints of "rashes"
bleeding

What is the physical presentation of AML?

Signs of marrow failure
"Blueberry muffin" lesions - papable, purpuric subcutaneous nodules (AKA Leukemia Cutis)
Gingival infiltration
Chloromas

What is the pathophysiology of gingival bleeding?

Myelogenous cells infiltrate the gingiva, causing swelling and bleeding

Form of cutaneous AML involvement

How do you dx a chloroma?

Greenish color from myeloperoxidase
Fluoresces red under UV light

What are the labs for AML?

↑ WBC
↓ platelets
normocytic, normochromic anemia

BM: hypercellular marrow with >30% blasts
karyotyping has prognostic and tx value

What is the treatment for AML?

induction chemo achieves remission in 80%; 105 die from infection/bleeding prior to remission

SCT or BMT after remission from an appropriate donor can improve survival (graft-vs-leukemia effect favors allogeneic transplantation)

In lieu of transplant, maintenance chemo can be curative for some

Here's a review for CML.

2-3% of all childhood leukemia
Features the Philadelphia chromosome with subsequent BCR-ABL gene rearrangement
Presents with splenomegaly, thrombocytosis, leukocytosis
Chronic phase in children lasts 3-4 years, followed by blast crisis, chronic phase may last 10-15 years in adults
Treatment with imatinib (gleevec) results in 70% major response
Stem cell transplantation or bone marrow transplant results in 80% cure rate

Here's a review for CLL.

same thing as small cell leukemia
hypo-IgG, autoantibodies present & made by normal B cells, indicating immune disregulation
over time, tumor cells displace the healthy marrow elements, resulting in anemia, thrombocytopenia, and neutropenia

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