hematopoetic cancers. hematopoesis leukemia new diagnoses each year in the us: 40, 800 adults...
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LeukemiaTRANSCRIPT
Hematopoetic Cancers
Hematopoesis
Leukemia
Leukemia
New diagnoses each year in the US:
40, 800 Adults
3,500 Children
21,840 died of leukemia in 2010
Leukemia
Lifetime Risk: 1/77
Median age of diagnosis = 66 YOA
Median age of death = 73 YOA
5 year survival = 54.1%
Symptoms
HeadachesVomitingConfusion
Loss of Muscle ControlSeizures
Swollen Lymph NodesFeversPallor
Weakness or FatigueFrequent Infections
Bleeding and BruisingSwollen or Uncomfortable Abdomen
Joint Pain
Risk Factors
Radiation
Smoking
Chemotherapy
Down Syndrome
HTLV-1 Infection
Family History
Lymphoblastic / Lymphocytic Cancers
Acute Lymphoblastic Leukemia
Lifetime Risk: 1/799
Median age of diagnosis = 13 YOA
Median age of death = 49 YOA
5 year survival = 65.2%
ALL Detection
No evidence that screening improves outcomes
Generally detected with presented illness
ALL Factoids
~5000 new cases each year
Primarily affects children- > 50% or cases occur before 20 years of age.
Most common childhood cancer (23% of childhood cancers)
Hispanic > White > Black
Males > Females
ALL Treatment
Chemotherapy 2-3 years (1% die initially and another 1-3% die during initial remission). [>95% experience complete remission w/i 4 weeks]
Some Pts have CNS involvement (WBCs in CNS-3%); Systemic + Intrathecal Chemotherapy + Cranial radiation.
Some Pts have testicular involvement (2% of males) Systemic Chemotherapy + Testicular radiation
ALL Survival
5 year survival = 89% for children <15 (Best outcomes 1-9 YOA)5 year survival = 50% for children 15-19
Worse outcomes for > 20 YOA and < 6 MOA
Chronic Lymphoblastic Leukemia
Lifetime Risk: 1/210
Median age of diagnosis = 72 YOA
Median age of death = 79 YOA
5 year survival = 78.4.2%
CLL Detection
No evidence that screening improves outcomes
Generally detected with presented illness or routine CBC
CLL Factoids
~14,990 new cases each year
Primarily in middle-aged and elderly adults- increasing frequency in successive decades of life.
Often slow progression
Hyperproliferative cells have both B- and T- cell surface antigens (CDs 19/20 and CD 5)
White > Black > Hispanic > Asian
Males (~2x) > Females
CLL Treatment
Dependent upon staging, no treatment (stage 0) to combination chemotherapy 2-3 years + / - radiation (stages I-IV) [Little evidence suggesting improved survival with these treatments]
Symptomatic treatment
Bone marrow transplant [Under clinical investigation, particularly for males < 60 YOA at diagnosis]
Complications of non-treatment include ‘hyperviscosity syndrome’, autoimmune hemolytic anemia, and opportunistic infections resulting from hypogammaglobinemia
Hairy Cell LeukemiaAbnormal B Cells
80% Affected = Adult MaleAverage age of diagnosis = 55 YOA
Myelogenous Cancers(Myelocytic / Myeloblastic)
Acute Myeloid Leukemia
Lifetime Risk: 1/266
Median age of diagnosis = 67 YOA
Median age of death = 72 YOA
5 year survival = 23.6%
AML Detection
No evidence that screening improves outcomes
Generally detected with presented illness or routine CBC
AML Factoids
~12,330 new cases each year
primarily in middle-aged and elderly adults.
All races approximate equal prevalence with White slightly higher
Males > Females
Some Pts have CNS involvement (5%)
AML Treatment
Systemically administered combination chemotherapy treatment divided into two phases: remission induction (to attain remission) and post-remission (to maintain remission).
Blood composition complications must be treated by replacement therapy and/or myeloid growth factor (e.g., GM-CSF) administration.
Bone marrow transplant—30% cure rate in Pts who have experienced an early relapse following remission
AML Treatment Factoids
Approximately 60% to 70% of adults with AML can be expected to attain complete remission following therapy.
More than 25% of adults with AML (about 45% of those who attain complete remission) can be expected to survive 3 or more years and may be cured.
Remission rates in adult AML are inversely related to age, and remissions are generally shorter in older patients.
Other adverse prognostic factors include central nervous system involvement . systemic infection at diagnosis, elevated white blood cell count (>100,000/mm3), treatment-induced AML, and others.
Chronic Myeloid Leukemia
Lifetime Risk: 1/635
Median age of diagnosis = 65 YOA
Median age of death = 74 YOA
5 year survival = 56.8% (median survival 4-6 years)
CML Detection
Splenomegaly (although 10% of Pts have no splenic enlargement.
Chronic (<10%) Accelerated (10-19%) Blastic phases (>20%) [increasing immature myeloid cells]
Anemia
CML Factoids
~4870 new cases each year
>95% of patients have a distinctive cytogenetic abnormality, the “Philadelphia chromosome”.
All races approximate equal prevalence with Hispanic and Asian slightly lower
Males (~2x) > Females
CML Treatment
Survival following ‘accelerated phase’ is generally < 1 year
Pts with Ph1-negative CML generally have a poorer response to treatment and shorter survival than Ph1-positive pts.
Imatinib (Y-kinase inhibitor) for Ph1-positive Pts. (2 years-indefinitely)
Interferon-
Other chemotherapy
Bone marrow transplant
Lymphoma
Lymphoma
New diagnoses each year in the US:
71,810 Adults
2,220 Children
21,530 died of lymphoma in 2010
Lymphoma
Median age of diagnosis = 64 YOA
Median age of death = 75 YOA
5 year survival = 70.1%
Hodgkin Lymphoma
Lifetime Risk: 1/428
Median age of diagnosis = 38 YOA
Median age of death = 63 YOA
5 year survival = 84.7%
Detection
Unexplained loss of more than 10% of body weight in the 6 months before diagnosis.
Unexplained fever with temperatures above 38°C.
Drenching night sweats.
Confirmed by biopsy and/or CT scan
5% of Pts have bone marrow involvement
Hodgkin Lymphoma Factoids
~8490 new cases each year
All races approximate equal prevalence with Asian 2x lower
Males > Females
Stage Distribution and 5-year Relative Survival by Stage at Diagnosis
Stage Distribution and 5-year Relative Survival by Stage at Diagnosis for 1999-2006, All Races, Both Sexes
Stage at Diagnosis Stage Distribution (%)
5-year Relative Survival (%)
Localized (confined to primary site) 19 90.3
Regional (spread to regional lymphnodes) 40 91.1
Distant (cancer has metastasized) 35 74.2
Unknown (unstaged) 5 82.3
Hodgkin Treatment
Chemotherapy + Radiation Therapy to affected lymph nodes
Pts who do not respond to chemotherapy (about 10%–20% of all presenting pts) have <10% survival rate at 8 years
Non-Hodgkin Lymphoma
Lifetime Risk: 1/48
Median age of diagnosis = 67 YOA
Median age of death = 75 YOA
5 year survival = 67.4%
NHL Detection
Unexplained loss of more than 10% of body weight in the 6 months before diagnosis.
Unexplained fever with temperatures above 38°C.
Drenching night sweats.
The majority of pts present with advanced (stage III or stage IV) disease that is often identified with CT scanning and biopsies of the bone marrow
NHL Factoids
65,540 new cases each year
B Cell and T Cell Varieties:B Cell: 14-20/100,000; median age at diagnosis = 67T Cell: 14-23/1,000,000; median age at diagnosis = 59
White > Black, Hispanic and Asian
Males > Females
Less predictable than Hodgkin lymphoma with a far greater predilection to disseminate to extranodal sites.
Non-Hodgkin Lymphoma Treatments
Varies with staging, ranging from ‘watchful waiting, to chemotherapy + / - radiation Therapy to affected lymph nodes
Rituximab (anti CD20) for B Cell variety