Leukemias Flashcards
What do abnormal CBC patterns indicate
Leukocytosis: reactive vs cancer or BM d/o
Leukopenia: meds, splenic sequestration, BM d/o
Polycythemia: dehydration, BM d/o
Thrombocytosis: reactive vs BM do
Thrombocytopenia: consumptive vs BM d/o
What are the WBC
Neutrophils Eosinophils Basophils Monocytes Lymphoctes
What do different leukocytosis findings indicate
Blasts: AML/ALL
Bands: infection
High neuts, low lymphs, no abn cells: Corticosteroids
If you find polycythemia vera (high H&H), inquire about these
Dehydration Tobacco use OSA Pickwickian (very obese= high risk OSA) JAK2 mutation High EPO levels
If you find thrombocytosis, inquire about these
Co-existing anemia
infection
removed spleen
non-infx inflammatory condition
What are Myeloproliferative Neoplasm
Acquired clonal abnormalities, can lead to MDS
Basically, the thermostat that control blood cell production is turned UP!
MPN and MDS are on a continuum as part of chronic myeloid neoplasms and can progress to acute leukemia*
Compare MPN vs MDS
MPN: terminal myeloid expansion= too much blood!
MDS: cell dysplasia= not enough blood!
What are the chronic myeloproliferative neoplasms
CML polycythemia vera essential thrombocytopenia primary myelofibrosis non-classic MPN
What are the types of polycythemia
Primary: 2/2 acquired or inherited mutation; polycythemia vera, chuvash polycythemia, idiopathic erythrocytosis
Secondary: 2/2 elevated Epo; Epo secreting tumor, Hypoxia
Combined: decreased plasma volume + increased RBC mass; tobacco use
What is polycythemia vera
JAK 2 mutation (exon 14&12) causes increased RBC mass, with a low Epo level
Dx w/ BM Bx: hypercellular with absent Fe stores (overproducing RBC but not iron)
MC in 60+ y/o
How does polycythemia vera present
Hgb: 18 Pruritis after warm shower HA, dizziness blurred vision tinnitus fatigue early satiety PUD Sx Erythromelalgia of hands and feet Facial plethora plenomegaly HTN Excoriated skin Thrombosis or hemorrhage
How do you treat PV
Phlebotomy! Want Hct <45%
Low dose aspirin
Cytoreductive agents: Hydroxyurea* (brings all cell counts down!)
-Prevent it from progressing to primary myelofibrosis
What is essential thrombocythemia (primary thrombocytosis)
Plt: 1 million! (can dx at >450)
BM Bx: normocellularity w/ prominent megakaryocytes
JAK2 mutation
-R/o iron deficiency
-Increased risk for hemorrhage and thrombosis
How do you treat Essential Thrombocythemia
Low dose ASA
Hydroxyurea
What is CML
Overproduction of myeloid cells (WBC 100-200)
MC in middle aged
*Philadelphia chromosome associated
What are the phases of CML
Chronic stable: MC. if not Tx, can progress to blast phase
Accelerated: increased blasts. will progress to blast phase in 4-6 months
Blast phase (crisis): survive 1-2 years. May see ALL or AML
How does CML present
Fatigue
night sweats
low grade fever
abd pain (2/2 splenomegaly)
CML labs will show
High WBC (100-200) low LAP score Presence of BCR or ABL gene <5% blasts Dont need BM Bx, but it is strongly required
How do you treat CML
Allogenic stem cell transplant may cure if pt is really sick!
Tyrosine Kinase Inhibitors for disease control: Gleevec* (imatinib) may decrease need for transplant in CML pts
Palliative care w/ cytotoxics: Hydroxyurea (IF + cytarabine have more ADE)
What are the types of transplant you can get
Allogenic: from others
Autologous: from yourself after it has been cleaned
How does Gleevec work in CML
Inhibits tyrosine kinase which may be an abnormal defect in philadelphia chromosome
What is non-classic MPN
Chronic neutrophilic leukemia
hypereosinophilic syndrome (HES)
chronic eosinophilic leukemia (CEL)
-Basically MPN W/O: BCR-ABL gene
What do you need to rule out in HES/CEL
Parasitic infection!
You suspect it because both have elevated eosinophils, which indicate parasite infx
What happens to cells in acute leukemia
Progenitor cells lose the ability to differentiate
The worse acute leukemia prognosis is if it arises from MDS or MPN
Can also be caused by benzene and cytotoxic agents (melphalan, etoposide- chemo drugs)
Differentiate AML vs ALL
AML: MC in elderly, + Auer Rods*
ALL: MC in kids, peak 3-7 y/o
Both AML and ALL have
*Pancytopenia w/ circulating blasts
Leukocytosis >200 (paradoxical infections)
*Leukostasis (med emergency, decreased tissue perfusion in pulmonary and cerebrovascular)
-Spleen enlargement, purple petechial rash, bone pain, SOB, LAD
What is specific to ALL that is really bad
Crosses the BBB so you can have CNS Symptoms
What are some characteristics of AML
Incidence increases w/ age! Mean 65 y/o
Caused by: Radiation, chemicals, drugs, Down Syndrome*, Klinefelter syndrome
Auer Rods are present
How do you treat AML
Young: treat to cure and try to obtain complete remission- give Chemo! Anthracycline and Cytabine
+/- Consolidation chemo or SCT if candidate
Allogenic SCT- cures 60% of younger
What are some characteristics of ALL
MC in kids 3-7
Cytogenics determine prognosis
How do you treat ALL
Chemotherapy: danurubicin, vincristine, prednisone, and asparginase
+/- followed by SCT if needed
IC chemo only for CNS prophylaxis
What are some characteristics of CLL
B lymphocyte malignancy
CLL Mc than ALL
Found incidentally on CBC (may have fatigue and LAD, and splenomegaly)
Associated with Richter’s transformation (high grade lymphoma) and Smudge cells
What are some causes of CLL
Abn response to antigenic stimulation
Cytogenic abn
Genetic changes
Microenvironmental changes
How do you treat CLL
Dont need to know
What is aplastic anemia
Life threatening pancytopenia caused by drugs, radiation, infection, or inherited
Bone marrow just does not work
Present with bleeding and infection
MC at 25 y/o
Drugs that can cause aplastic anemia are
Chloramphenicol
Benzene
Toluene
Chemotherapy
On PE for aplastic anemia you may find
pallor
purpura
petechiae
NO LAD or splenomegaly
What is the Mc form of inherited aplastic anemia
Fanconi anemia
How do you treat aplastic anemia
Allogenic SCT or immunosuppression
What is hairy cell leukemia
Uncommon B cell lymphoproliferation MC in men Patients have palpable splenomegaly CBC shows pancytopenia w/low monocytes BM Bx shows "hairy cells" Most pts live >10 years!
In a patient with CLL what would you expect to see on CBC
Lymphocytosis
Leukocytosis
What test would you use to rule in suspected CML
BM Bx
