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137 Cards in this Set
- Front
- Back
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More than 20% myeloblasts in the bone marrow
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Acute Myeloid Leukemia
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Auer rods
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Acute Myeloid Leukemia
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Peroxidase positive granules
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Acute Myeloid Leukemia
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Acute Myeloid leukemia flow cytometry CDs
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CD 13, 14, 15, 33, 34, 64, 117
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Remember, Monoblasts have NO auer rods in AML
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Notes
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There is BLOCKAGE of differentiation and maturation in acute myeloid leukemia
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Notes
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M1
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AML without maturation
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M2 and genetics
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AML with maturation
t(8;21) |
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M3 and Genetics
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Acute Promyelocytic Leukemia
t(15;17) |
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M4 and Genetics
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Acute myeloMONOcytic leukemia
inv 16 |
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M5
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Acute Monocytic Leukemia
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Differentiate myeloblasts vs monoblasts
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Myeloblasts- auer rods, peroxidase positive
Monoblasts- No auer rods, peroxidase negative, Non-specific esterase positive granules |
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Clinical features of AML
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Anemia, Neutropenia, Thrombocytopenia, DIC!
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What is a chloroma?
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Soft tissue mass of myeloblasts, usually in breasts
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Promyelocytic Leukemia
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1- DIC
2- t(15;17) translocation- prevents myeloid differentiation |
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DIC
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Promyelocytic Leukemia
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Philadelphia is in CHRONIC, not ACUTE Myeloid Leukemia
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Notes
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Childhood leukemia
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B-cell ALL
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B cell ALL can spread to other organs
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Notes
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T cell ALL presents as a thymic mediastinal mass in young adults
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Notes
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PAS positive (periodic acid schiff)
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ALL
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TRAP (tartrate resistant acid phosphatase) positive
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Hairy Cell Leukemia
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Cytoplasmic extensions
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Hairy Cell Leukemia
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What is hairy cell leukemia?
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Neoplastic clonal proliferation of B cells
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B cell ALL can spread to other organs
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Notes
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T cell ALL presents as a thymic mediastinal mass in young adults
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Notes
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PAS positive (periodic acid schiff)
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ALL
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Differentiate myeloblasts vs monoblasts
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Myeloblasts- auer rods, peroxidase positive
Monoblasts- No auer rods, peroxidase negative, Non-specific esterase positive granules |
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Clinical features of AML
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Anemia, Neutropenia, Thrombocytopenia, Leukemia Cutis
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What is a chloroma?
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Soft tissue mass of myeloblasts, usually in breasts
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Promyelocytic Leukemia
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1- DIC
2- t(15;17) translocation- prevents myeloid differentiation |
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DIC
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Promyelocytic Leukemia
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Philadelphia is in CHRONIC, not ACUTE Myeloid Leukemia
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Notes
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Childhood leukemia
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B-cell ALL
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TRAP (tartrate resistant acid phosphatase) positive
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Hairy Cell Leukemia
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Cytoplasmic extensions
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Hairy Cell Leukemia
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What is hairy cell leukemia?
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Neoplastic clonal proliferation of B cells
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Rouleaux Formation- coinstack RBCs
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Multiple myeloma
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Lytics punched out bone lesions
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Multiple myeloma
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Most people with multiple myelomas die because of 2 things
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1- Infections
2- Renal disease, Myeloma nephrosis, Myeloma kidney- light chain damage, light chains are very toxic to the tubules |
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Anything that is multinucleate is a no no
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Notes
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What is the most common cause of monoclonal gammopathy?
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MGUS- Monoclonal Gammopathy of Unknown Significance
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Hyperviscosity Syndrome seen in
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Waldenstrom's Macroglobulinemia
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What is M5?
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Acute monocytic leukemia
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Acute Myeloid Leukemia is an accumulation of what cells?
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Myeloblasts
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What is M6?
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Acute erythroleukemia
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AML Immunophenotype
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(+) CD13, CD14, CD15, CD33, CD34, CD64, CD117
CD41, CD61 positive for megakaryocytes |
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Leukemia cutis (skin) seen in
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AML
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t(15;17) and DIC
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Acute Promyelocytic Leukemia (PML)
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Acute Promyelocytic Leukemia (PML)
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t(15;17) and DIC and AUER RODS!
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Immunophenotype of Acute Lymphoblastic Leukemia (ALL)
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(+) CD10,19,TdT
t(12;21) -offers favorable prognosis |
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What age group does T-cell ALL affect?
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15-20 yrs presenting as a lymphoblastic lymphoma
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Notch mutation seen in
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T-cell ALL
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What is the presentation of T-cell ALL?
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Mediastinal (thymic) mass in young adults
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Hairy cell leukemia
Description Bone Marrow Immunophenotype Clinical features |
Neoplastic clonal proliferation of MEMORY B cells.
BM- Diffuse interstitial infiltrate of cells with reniform nucleus and blue cytoplasm (fried-egg appearance) Immunophenotype + CD11c,19,20,25,103 (-)CD5,10,23 Clinical- middle aged caucasians, hepatosplenomegaly, TRAP positive- hairy cells on blood smear |
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Multiple Myeloma
Description Immunophenotype Serum/Urine Electrophoresis |
Neoplastic clonal proliferation of plasma cells
(+) CD56,79a, IgG and IgA mainly- Bence Jones light chains Monoclonal Spike |
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Clinical features of Multiple Myeloma (8)
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Bone Lesions
Hypercalcemia Hyperuricemia Anemia Infections Hyperviscosity Renal Amyloidosis |
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Peripheral smear of Rouleaux formation and Pancytopenia indicates
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Multiple Myeloma
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What is hoff body?
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Golgi- looks pale in a plasma cell
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Bence Jones light chain seen in
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Multiple Myeloma
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Monoclonal spike in urine/serum
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Multiple Myeloma
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Waldenstrom's Macroglobulinemia also called
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LymphoPLASMAcytic Lymphoma
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Waldenstrom's Macroglobulinemia
Description Genetics Immunophenotype Bone Marrow Bone Clinical Features |
Abnormal neoplastic clonal proliferation of small lymphocytes and plasma cells. Production of heavy and light chains is balanced. IgM produced
Genetics- 6q deletion, t(9;14), PAX 5 gene (+)CD20 (-)CD5,10,23 BM- hypercellular, MAST cells Bone- NO LESIONS Hyperviscosity Syndrome |
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Multiple Myeloma vs Waldenstrom's Macroglobulinemia
Which affects bone and kidneys? |
Multiple myeloma
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Multiple Myeloma vs Waldenstrom's Macroglobulinemia
Which causes organomegaly and hyperviscosity syndrome? |
Waldenstrom's Macroglobulinemia
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Ig type of Waldenstrom's Macroglobulinemia
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IgM
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Ig type of Multiple Myeloma
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IgG, IgA
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What are the tumor cells of multiple myeloma?
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Plasma cells
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What are the tumor cells of Waldenstrom's Macroglobulinemia?
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Lymphocyte
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CRAB for multiple myeloma
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HyperCALCEMIA
RENAL insufficiency Anemia Bone/Back pain |
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9-22 Chromosone Reciprocal Translocation associated with what
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Chronic Myeloid Leukemia
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Main diagnostic feature of Chronic Myeloid Leukemia
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BCR-ABL fusion gene
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BCR-ABL fusion gene produces what?
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Protein p210 with tyrosine kinase activity
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You cannot diagnose Chronic Myeloid Leukemia WITHOUT...
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Philadelphia Chromosome
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Liver, spleen enlarged, what leukemia?
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CML
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LAP = 0
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CML
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High basophilia
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CML
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Sea blue histiocytes
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CML
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Polycythemia Vera
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Increased RBC mass, decreased EPO
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What mutation causes Polycythemia Vera?
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JAK-2
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Secondary Polycythemia
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Increased EPO levels due to tumors, drugs, etc
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Primary Polycythemia
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Low EPO due to genetic mutations- polycythemia vera
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Relative polycythemia
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Reduced plasma volume
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Philadelphia Chromosome is ABSENT in Polycythemia Vera
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Notes
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2 common causes of splenomegaly
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1) Malignant Lymphoma
2) Cirrhosis of liver |
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Essential Thrombocythemia
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Thrombocytosis- high platelet count
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How do you diagnose Primary Myelofibrosis?
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Dry Tap of Bone Marrow Aspiration
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Tear Drop RBCs
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Primary Myelofibrosis
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What is thrombocytosis?
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High platelet count
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What is primary myelofibrosis? (4)
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Marrow fibrosis
Tear drop RBC's Leukoerythroblastosis- premature RBCs and WBCs, pushed out of bone marrow Pancytopenia |
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Myelodysplastic Syndrome (4)
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1. Ineffective hematopoiesis
2. Dysplastic morphology of cells 3. ↑ Apoptosis 4. Pancytopenia |
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Chromosomal Abnormalities of Myelodysplasia
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Monosomy 5,7, Trisomy 8
Deletion of 5q 7q 20q |
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Ringed Sideroblasts
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MDS
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Pawn-ball megakaryocytes
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MDS
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Pseudo-pelger-huet neutrophils
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MDS
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Chronic Myeloid Leukemia
Description Diagnosis Genetics Peripheral Blood Bone marrow Clinical |
Pluripotent stem cells that give rise to myeloid (sometimes lymphoid) cells with TERMINAL differentiation
Diag- Philadelphia chromosome, BCR-ABL fusion gene t(9;22) (q34;11) Blood- LAP absent BM- Hypercellular, sea blue histiocytes, BASOPHILIA Clinical- organomegaly, |
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LAP absent in blood
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CML
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JAK-2 mutation
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Polycythemia Vera
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Polycythemia Vera
Descript |
Neoplastic clonal proliferation of stem cells with increased production of everything blood
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EPO levels in polycythemia vera
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Low
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EPO levels in secondary polycythemia
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High
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Essential Thrombocythemia
Description Diagnosis |
Increased megakaryocytes/platelets
Diagnosis by exclusion |
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Primary myelofibrosis
Descript Diagnosis Blood |
Marrow fibrosis- megakaryocytes produce PDGF and TGF-Beta
Diagnosis- Bone marrow BX Blood- pancytopenia, tear drop cells, leukoerythroblastosis |
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Myelodysplastic Syndrome
Descript Genetics Key cells seen |
1- Ineffective hematopoiesis
2- Dysplasia 3- Increased apoptosis 4- Peripheral blood cytopenia Monosomy 5,7, Trisomy 8, Deletion 5q,7q,20q Ringed sideroblasts,pseudo-pelger huet cells, Pawn-Ball megakaryocytes |
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Ringed Sideroblasts
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Myelodysplastic Syndrome
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What are pseudo-pelger huet cells and what disease?
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Neutrophils with only two nuclear lobes
-Myelodysplastic syndrome |
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What are pawn-ball megakaryocytes and what disease?
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Cells with multiple, separate nuclei
-Myelodysplastic syndrome |
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What are ringed sideroblasts and in what disease?
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Erythroblasts with iron laden mitochondria
Myelodysplastic Syndrome |
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RBCs are megaloblastic and multinucleated
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Myelodysplastic Syndrome
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Myeloid Proliferative Disorders (6)
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1- Chronic Myeloid Leukemia
2- Acute Myeloid Leukemia 3- Polycythemia Vera 4- Primary myelofibrosis 5- Essential Thrombocythemia (ET) 6- Myelodysplastic Syndrome (MDS) |
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Myeloid metaplasia also known as
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Primary myelofibrosis
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JAK-2 (involved in hematopoietic growth factor signaling) is in all chronic myeloproliferative except
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CML
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Only leukemia with thrombocytosis
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CML
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What is a leukomoid reaction?
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Benign, exaggerated leukocyte response
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How do corticosteroids affect eosinophils?
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Corticosteroids sequester eosinophils in lymph nodes
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Infectious mononucleosis is caused by
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EBV
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How do corticosteroids affect:
-Neutrophils -Eosinophils -Lymphocytes |
Increase neutrophils
Decrease lymphocytes and eosinophils |
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What is the general definition of leukemia?
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Malignant transformation of marrow stem cells
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Most common overall type of leukemia
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CLL
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>60 years old, most likely leukemia
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CLL
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Appropriate absolute polycythemia
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increased EPO due to hypoxic stimulus
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Inappropriate absolute polycythemia
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Increased EPO due to ectopic production like in renal cell carcinoma
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Pruritis after bathing seen in
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Polycythemia vera- mast cells degranulate with change in skin temp
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Decreased EPO indicatess
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Polycythemia Vera
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BCR-ABL fusion gene
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CML
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RX for CML
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Imatiniib
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What does Leukoerythroblastic reaction mean?
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Immature bone marrow cells in the peripheral blood
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Myelofibrosis
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Marrow fibrosis and teardrop cells
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JAK2 mutations seen in 3 conditions
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1- Polycythemia vera
2- Essential thrombocythemia 3- Myelofibrosis |
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Cytopenias and hypercellular marrow
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MDS
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CALLA, CD10
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ALL (Acute lymphoblastic leukemia)
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thymic mediastinal mass in young adults
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T-cell ALL
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Leukemia that spreads to CNS and testicles
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B-cell ALL
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Most common leukemia
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CLL
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Only leukemia without adenopathy
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Hairy Cell Leukemia
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t(12,21)
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ALL- offers good prognosis
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HTLV-1 associated with
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Adult T cell leukemia
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