Leukemia Flashcards
how do you calculate the absolute neutrophil count
- ANC= WBC X total neutrophils
what is a normal ANC
- > 1000
- usu 1500- 8000
what is a neutropenic ANC
- 500-1000
what is severe neutrophenia
- chronically < 500
what is the role of neutrophils
- fight bacterial infections
indications for bone marrow bx
- pt specific i.e. unexplained anemia
- pancytopenia
- abnormal cells in circulation
contraindcations for bone marrow bx
- hemophilia
- severe DIC
- other severe bleeding disorders
things that are NOT contraindications to bone marrow bx
- thrombocytopenia
- therapeutic anticoagulation
sites for bone marrow aspiration
- posterior superior iliac crest and spine*
- anterior iliac crest
- greater troch
- vertebral bodies or ribs
- sternum- usu C/I
- tibia in kids < 12-18 mo
what causes leukemia
- too many progenitor cells produced in bone marrow
- abnormal cells dont die when they should
- overproduction of bad cells interferes with prod of good cells
- cells accumulate and occupy space -> leukostasis
how do you categorize leukemias
- lymphoid vs myeloid cells
- acute vs chronic
what is the blast cell count in leukemia
- > 20%
what is the blast cell count in myelodysplastic syndrome
- < 20%
what type of leukemia is most common in kids
- ALL
what type of leukemia is most common in adults
- AML
what are myelodysplastic syndromes
- bone marrow does not make enough good cells
- make immature cells < 20%
- may present with initial low grade anemia
types of myelodysplastic syndromes
- refractory anemia
- refractory cytpopenia with multilineage dysplasia
- refractory anemia with ringed sideroblasts
- refractory cytopenia with multilineage dysplasia and ringed sideroblasts
- refractory anemia with excess blasts
risk factors for myelodysplastic sydromes
- age*- rarely in pts < 60
- smoking
- long term exposure to chemicals
- radiation
- inherited disorders
inherited disorders that cause myelodysplastic syndromes
- fanconi anemia
- shwahmann diamond syndromes
- familial platelet disorder
- severe congenital neutropenia
acute myelogenous leukemia (AML)
- rapid accum of immature meyloid cells
- accum in bone marrow -> interfere with other production lines
- increased incidence with age
risk factors for AML
- genetics- trisomy 21, fanconi
- chemicals
- high dose radiation
- drugs- alkylating agents, topoisomerase II inhibitors, chloramphenicol, phenylbutazone
si/sx of AML
- can be gradual or abrupt
- fatigue
- anorexia, weight loss
- fever +/- infx
- bleeding, easy bruising
- HA
PE findings for AML
- fever
- splenomegaly
- LAD
- sternal tenderness
- ecchymosis, petechiae
- papilledema
- CN abnormalities
- gum hypertrophy
- skin infiltrates
- bone pain
work up for AML
- auer rods on smear*
- bone marrow bx and aspirate*
- LP if suspect CNS involvement
- immunophenotyping
- cytogenetics
treatment for AML
- depends on genetics and prognosis*
- goal is destruction of all leukemia cells
- after remission pts will cont to be treated to prevent relapse
- may get HLA matched SCT
chronic myeloid leukemia (CML)
- increased proliferation of granulocytes (neutrophils, eos, basos)
- BCR-ABL rearrangement -> philadelphia chromosome
at what age is CML usu diagnosed?
- in 60s
cause of CML
- no known genetic, geographical, or ethnic assoc
- no chemical or infx assoc
- possible increased risk with ionizing radiation
stages of CML
- chronic
- accelerated: 15% blasts
- blast: 30% or more blasts, extramedullary disease and resembles AML
- some progress through stages in order, some dont
PE findings for CML
- splenomegaly- in blast crisis
- mild hepatomegaly
- LAD
dx of CML
- bone marrow aspirate and bx*
- cytogenetics + Philadelphia chromo
- fish/molecular studies + for BCR-ABL gene
what determines prognosis of CML
- % blasts
- cytogenetics
- age of pt
- platelets
- spleen size
what is assoc with a poor prognosis for CML
- older age
- severe anemia
- neg Ph chromosome
- thrombocytopenia
- hepato/ splenomegaly
treatment for CML
- imatinib
- if acute or blast phase, use tyrosine kinase inhibitor + chemo -> allogenic SCT
acute lymphoblastic leukemia (ALL)
- proliferation and accumulation of lymphoid progenitor cells
- most common childhood cancer, responds well to tx
- bimodal age distribution: kids, 40s
etiology of ALL
- largely unknown
- possible chromo translocation in utero
- high incidence in kleinfelter and down syndrome
- possible viral assoc
viral assoc with ALL
- HTLV-1
- varicella
risk factors for ALL
- prev CA tx
- exposure to radiation
- genetic disorder, esp down syndrome
- sibling with ALL
si/sx of ALL
- signs of bone marrow failure
- malaise, fatigue
- bleeding, bruising
- secondary infection
- B sx
- CNS involvement: CN palsies, meningeal infiltration
what are B sx
- fever
- weight loss
- night sweats
work up for ALL
- bone marrow aspirate and bx
- LP if CNS involvement
- anemia, thrombocytopenia, lymphoblasts
- platelets < 100,000
- immunophenotyping
- cytogenetics
- molecular studies
chronic lymphocytic leukemia (CLL)
- most common leukemia in western hemisphere
- expansion of CD5 pos B cells
- B cells undergo gene arrangement in marrow -> immuno-incompetent B cells
possible causes for CLL
- 15-20% of pts have family member with CLL or lymphoproliferative disorder
- single nucleotide polymorphisms have been identified
gene alterations assoc with CLL
- most common is 13q deletion
- worst prognosis is 17p
- 11q assoc with advanced disease
si/sx of CLL
- insidious onset
- recurring infx
- enlarged LN
- fatigue
- loss of appetite
- B sx
PE findings for CLL
- LAD- cervical, supraclavicular, axillary
- spleno/ hepatomegaly
dx of CLL
- flow cytometry to confirm B cell surface antigens
- must distinguish mantle cell and large granular leukemia
- AIHA commonly present
- smudge cells on smear
staging systems used for CLL
- Rai staging: 0-IV
- binet staging: A, B or C
treatment for CLL
- early stage- follow pt, no tx
- chemo
- immunochemo
- B cell signaling inhibitors
- stem cell transplant
- palliative care
harry cell leukemia
- rare
- B cell lineage
- mostly in adult males
- presents like CLL but NO LAD
