Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
38 Cards in this Set
- Front
- Back
|
General causes of leukopenia?
|
Decreased production
Aplastic anemia, myelophthisic anemia, sepsis, inherited Increased destruction Immune, drugs, hypersplenism <500 neutrophils increases risk for bacterial infections |
|
|
Neutrophilia:
|
acute bacterial infections
Dohle bodies and toxic granules, cytoplasmic vacuolization |
|
|
Lymphocytosis:
|
viral infections (reactive lymphs)
|
|
|
Eosinophilia:
|
allergies, asthma, parasites, neoplasia (Hodgkin disease)
|
|
|
Monocytosis:
|
chronic infections (TB, endocarditis)
|
|
|
Basophilia:
|
(almost never, except CML)
|
|
|
Acute leukemia
|
increased blasts in bone marrow and peripheral blood
|
|
|
Myelodysplastic syndromes (MDS)–
|
“pre-leukemia” with dysplastic cells (ineffective hematopoiesis) and peripheral blood cytopenias (usually pancytopenia)
|
|
|
Chronic myeloproliferative diseases (MPD)–
|
increased mature cells in marrow and blood
Chronic myelogenous (or myelocytic) leukemia (CML) Polycythemia rubra vera Essential thrombocytosis Myelofibrosis |
|
|
Chronic lymphoproliferative diseases
|
Chronic lymphocytic leukemia (CLL)
Hairy cell leukemia (HCL) |
|
|
Plasma cell neoplasms
|
Plasma cell myeloma (multiple myeloma)
Monoclonal gammopathy of uncertain significance (MGUS) Waldenstroms macroglobulinemia |
|
|
General age distributions
|
ALL: children <15 yrs
AML: young adults 15-40 CML: middle age 40-60 CLL, MDS, HCL, myeloma: older adults >60 |
|
|
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 !!!!! |
|
|
Stain for AML?
|
myeloperoxidase
|
|
|
Stain for ALL?
|
Tdt + (terminal deoxynucleotidyl transferase)
|
|
|
Tests for any acute leukemia?
|
Bone marrow exam
Special stains Flow cytometry (diagnostic and prognostic) Genetics (diagnostic and prognostic) |
|
|
AML M3 mutation?
|
t(15;17) involves PML fusion with RARα. The RARα gene normally activates transcription, but fusion with PML acts as a repressor, blocking differentiation
|
|
|
AML M3 fun facts:
|
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
|
|
|
WHO AML classification
|
Classification based on specific genetic abnormality or arising from MDS or therapy-related
|
|
|
Genetic defect in ALL L3
aka Burkitts leukemia/lymphoma |
t(8;14)
Tdt negative! |
|
|
ALL Most common genetic abnormalities are
|
hyperdiploidy, t(12;21), t(9;22)
|
|
|
ALL Good prognostic factors
|
Age 2-10, hyperdiploidy or t(12;21), CALLA+ (CD10, common ALL antigen), WBC normal range
|
|
|
ALL Bad prognostic factors
|
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 |
|
|
Myelodysplastic syndromes MDS
|
Older adults, clonal stem cell diseases with hypercellular marrow but ineffective hematopoiesis (abnormal or dysplastic cells) resulting in pancytopenia
|
|
|
Chronic myeloproliferative diseases
|
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 |
|
|
CML vs leukemoid reaction
|
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 |
|
|
Genetic defect seen in CML?
|
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 |
|
|
Accelerated phase of CML
|
Means the disease is transforming to acute leukemia
Basophils >20% in blood Blasts >15% in blood Genetic clonal evolution (additional genetic abnormalities) |
|
|
Polycythemia vera
|
JAK2 mutation in 90%
Normal O2 saturation, be sure to get carboxyhemoglobin levels to rule out CO poisoning Transforms to myelofibrosis frequently (burn-out) |
|
|
Primary (essential) thrombocytosis
|
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 |
|
|
Chronic lymphoproliferative diseases
|
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 |
|
|
Hairy cell leukemia HCL
|
Uncommon, B-cell
Triad: elderly male, massive splenomegaly, pancytopenia TRAP+ cells (tartrate-resistant acid phosphatase) |
|
|
Plasma cell myeloma
|
Bone lytic lesions (axial skeleton)
Bence-Jones proteinuria |
|
|
Waldenstrom’s macroglobulinemia
|
IgM always
Plasma cells, lymphocytes and plymphocytes |
|
|
More myeloma facts
|
<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 |
|
|
Things you'll find in blood in people with no spleen
|
Howell-Jolly bodies (loss of splenic pitting fxn)
Target cells (loss of RBC re-modeling) |
|
|
Hypersplenism
|
May lead to cytopenias by sequestration
Dx triad Splenomegaly Cytopenia (decreased WBC or RBC or plts) Cytopenia corrected by splenectomy |
|
|
Thymic hyperplasia and thymomas are common in ??
|
myasthenia gravis
|
