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