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38 Cards in this Set
- Front
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General causes of leukopenia?
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Decreased production
Aplastic anemia, myelophthisic anemia, sepsis, inherited Increased destruction Immune, drugs, hypersplenism <500 neutrophils increases risk for bacterial infections |
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Neutrophilia:
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acute bacterial infections
Dohle bodies and toxic granules, cytoplasmic vacuolization |
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Lymphocytosis:
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viral infections (reactive lymphs)
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Eosinophilia:
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allergies, asthma, parasites, neoplasia (Hodgkin disease)
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Monocytosis:
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chronic infections (TB, endocarditis)
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Basophilia:
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(almost never, except CML)
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Acute leukemia
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increased blasts in bone marrow and peripheral blood
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Myelodysplastic syndromes (MDS)–
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“pre-leukemia” with dysplastic cells (ineffective hematopoiesis) and peripheral blood cytopenias (usually pancytopenia)
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Chronic myeloproliferative diseases (MPD)–
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increased mature cells in marrow and blood
Chronic myelogenous (or myelocytic) leukemia (CML) Polycythemia rubra vera Essential thrombocytosis Myelofibrosis |
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Chronic lymphoproliferative diseases
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Chronic lymphocytic leukemia (CLL)
Hairy cell leukemia (HCL) |
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Plasma cell neoplasms
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Plasma cell myeloma (multiple myeloma)
Monoclonal gammopathy of uncertain significance (MGUS) Waldenstroms macroglobulinemia |
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General age distributions
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ALL: children <15 yrs
AML: young adults 15-40 CML: middle age 40-60 CLL, MDS, HCL, myeloma: older adults >60 |
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Def’n: Acute leukemias
signs/symptoms? lab test? |
>20% blasts in the bone marrow
Signs & symptoms Neutropenia: bacterial infections, fever Anemia: fatigue, no energy Thrombocytopenia: bleeding, petechiae, epistaxis, gum bleeding Maybe hepatosplenomegaly, lymphadenopathy (in ALL), bone pain, possibly asymptomatic GET A CBC !!!!! |
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Stain for AML?
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myeloperoxidase
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Stain for ALL?
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Tdt + (terminal deoxynucleotidyl transferase)
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Tests for any acute leukemia?
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Bone marrow exam
Special stains Flow cytometry (diagnostic and prognostic) Genetics (diagnostic and prognostic) |
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AML M3 mutation?
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t(15;17) involves PML fusion with RARα. The RARα gene normally activates transcription, but fusion with PML acts as a repressor, blocking differentiation
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AML M3 fun facts:
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M3: acute progranulocytic leukemia APL, multiple Auer rods (faggot cells), *medical emergency because of severe DIC, t(15;17), Rx with ATRA all trans-retinoic acid, then chemo
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WHO AML classification
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Classification based on specific genetic abnormality or arising from MDS or therapy-related
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Genetic defect in ALL L3
aka Burkitts leukemia/lymphoma |
t(8;14)
Tdt negative! |
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ALL Most common genetic abnormalities are
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hyperdiploidy, t(12;21), t(9;22)
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ALL Good prognostic factors
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Age 2-10, hyperdiploidy or t(12;21), CALLA+ (CD10, common ALL antigen), WBC normal range
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ALL Bad prognostic factors
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Burkitt translocations, t(9;22) (produces a p190 gene product, compare later to CML p210), t(1;19), T-cell worse than B-cell ALL
older age |
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Myelodysplastic syndromes MDS
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Older adults, clonal stem cell diseases with hypercellular marrow but ineffective hematopoiesis (abnormal or dysplastic cells) resulting in pancytopenia
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Chronic myeloproliferative diseases
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Chronic = slow onset and progression, more mature cells (rather than blasts)
Hypercellular marrow, high count of one of the CBC parameters High WBC = chronic myelogenous leukemia CML High RBC = polycythemia rubra vera High platelets = essential thrombocytosis Myelofibrosis = burnout marrow with fibrosis, massive splenomegaly with leukoerythroblastic peripheral blood and teardrop RBCs |
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CML vs leukemoid reaction
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CML --> WBC >50,000
Blasts to myelocytes↑ Basophils increased nRBCs present Splenomegaly, massive LAP leukocyte alkaline phosphatase low Leukemoid rxn --> WBC <50,000 Increased bands, High LAP |
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Genetic defect seen in CML?
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t(9;22)
Produces an abnormal tyrosine kinase of size p210 (the MAJOR bcr/abl fusion protein; compare to the MINOR p190 fusion protein in ALL); can be detected in peripheral blood Rx with imatinib (Gleevec) targets the abnormal tyrosine kinase |
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Accelerated phase of CML
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Means the disease is transforming to acute leukemia
Basophils >20% in blood Blasts >15% in blood Genetic clonal evolution (additional genetic abnormalities) |
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Polycythemia vera
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JAK2 mutation in 90%
Normal O2 saturation, be sure to get carboxyhemoglobin levels to rule out CO poisoning Transforms to myelofibrosis frequently (burn-out) |
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Primary (essential) thrombocytosis
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Platelets >600,000 (usually > 1million)
Dx of exclusion Fe deficiency sometimes get platelets >1 mil CML with misleading low WBC but very high platelets JAK2 mutation in 50% Signs & symptoms Bleeding and thromboses, erythromelalgia (painful red limbs), splenomegaly |
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Chronic lymphoproliferative diseases
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CLL chronic lymphocytic leukemia
Elderly patients (same as SLL), fairly common High WBC, absolute lymphocytosis with many smudge cells Monoclonal B cells , CD5 and 23+ Dx by flow cytometry |
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Hairy cell leukemia HCL
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Uncommon, B-cell
Triad: elderly male, massive splenomegaly, pancytopenia TRAP+ cells (tartrate-resistant acid phosphatase) |
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Plasma cell myeloma
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Bone lytic lesions (axial skeleton)
Bence-Jones proteinuria |
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Waldenstrom’s macroglobulinemia
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IgM always
Plasma cells, lymphocytes and plymphocytes |
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More myeloma facts
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<10% plasma cells, no lytic lesions but a monoclonal protein means MGUS monoclonal gammopathy of uncertain significance
Dx serum/urine protein electrophoresis screen for monoclonal protein, immunofixation to type the monoclonal protein, bone marrow exam, skeletal survey for lytic lesions |
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Things you'll find in blood in people with no spleen
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Howell-Jolly bodies (loss of splenic pitting fxn)
Target cells (loss of RBC re-modeling) |
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Hypersplenism
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May lead to cytopenias by sequestration
Dx triad Splenomegaly Cytopenia (decreased WBC or RBC or plts) Cytopenia corrected by splenectomy |
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Thymic hyperplasia and thymomas are common in ??
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myasthenia gravis
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