1 hematopathology 1: Myeloid and Lymphoid Bone Marrow Disorders (mike) Flashcards
What is the normal myeloid to erythroid ration?
3:1
What is the approximate calculation of bone marrow cellularity?
100-age
Review the things a bone marrow biopsy offers that an aspirate might not…
Marrow cellularity
megakaryocytic numbers
aggregates of lymphocytes/blasts/tumor cells
fibrosis/or not
What is the order of granulocyte maturations from myeloblast to neutrophil?
myeloblast–> promyelocyte –> myelocyte –> metamyelocyte–> band –> neutrophil
What is the order of erythropoiesis from multi potential HSC to erythrocyte?
multipotential HSC–>common myeloid progenitor –> pro erythroblast–> basophilic erythroblast–> polychromatic erythroblast–> orthochromatic erythroblast–> polychromatic erythrocyte (reticulocyte)–>erythrocyte
Agranulocytosis has marked neutropenia to less than what level?
What is aplastic anemia? Is there an increase in reticulocytes?
- lack of hematopoietic cells in the bone marrow
2. No
What is granulocytic aplasia?
lack of granulocytic precursors in the marrow.
What is aplastic anemia? Is there an increase in reticulocytes? what does it look like?
- lack of hematopoietic cells in the bone marrow
- No
- just fat with little cellular type stuff
What is granulocytic aplasia? what does it look like?
- lack of granulocytic precursors in the marrow.
2. It looks like a bunch of schistocytes and target cells and few progenitors
In B12 deficiency there is defective DNA synthesis, but not defective RNA synthesis-what happens to the cells? what does it look like?
- cytoplasm matures, but nucleus remains immature (megaloblastic change)
- intramedullary death- pink jumbled mess of cells with a lot of cytoplasm and cells that look stuck in synthesis stages/metaphase stages
What is the clinical presentation of neutropenia
Infections- ulcerating necrosis of the mouth, necrotizing infections of the lung and UTI, massive colony bacteria growth
What are the 3 causes of reactive neutrophilia?
- demargination (from epinephrine release, acute stress, exercise, steroids)
- mobilization of maturation-storage compartment (steroids, infection, inflammation)
- Increased production (sustained infection, chronic)
What neoplasms (in general) lead to neutrophilia?
myeloproliferative neoplasms.
What neoplasms (in general) lead to neutrophilia? What does neutrphilia look like?
- myeloproliferative neoplasms.
2. increased absolute number of mature neutrophils in the CBC
What is a left shift?
immature granulocytic cells (band etc.) circulating in the blood. REMEMBER band cells over like 10% was SIRS
When are toxic changes in neutrophils seen?
acute infection and after administration of GCSF
In order for it to be toxic change in neutrophils in needs at least one thing from a criteria of 3. What is the criteria of three?
- toxic granulation (distinct course purple)
- cytoplasmic vacuolization
- Dohle bodies- pale blue structure (RER)
What is characterized by extreme leukocytosis, in response to stress or infection, may resemble leukemia, and has an increased immature cell population? what does it look like?
- leukemoid reaction
2. tons of neutrophils and band cells
What is a malignant neoplasm of hematopoietic cells characterized by diffuse replacement of the bone marrow by the neoplastic cells?
Leukemia
What is leukemia classified on the basis of? 3
- cell lineage (myeloid/lymphoid)
- Maturity (blasts vs. differentiated cells)
- chronicity (acute/chronic)
What are the most common types of leukemia in adults?
AML and CLL
What is the most common types of leukemia in children?
ALL
What is a malignant clonal neoplasm of multi potent myeloid stem cells characterized by the accumulation of myeloblasts in the bone marrow and blood?
AML- block in differentiation of leukemic stem cells, and replacement/suppression of normal hematopoietic precursors.
Review the sub-categories of AML?
- Based on specific cytogenetic abnormalities, dysplasia of the other cell lines, previous chemotherapy.
- may arise from any myeloid lineage cell
- monocytic, erythroid, megakaryocytic
What three antigens in flow cytometry should we be thinking in AML?
CD 13, 33, 117
In AML, what is a distinct histo characteristic we should be looking for?
Auer rods- only in myeloid blasts- red granular material containing MPO
Is the MPO stain ever seen in lymphoid blasts?
NO!- postitive in a subset of myeloid leukemias only.
T-F in acute promyelocytic leukemia blasts are often kidney shaped or bilobed?
True
What are the granules like in Acute promyelocytic leukemia? 2 variants
- microgranular variant- fine/delicate
2. hypergranular variant- intense coarse azurophilic granules
What is the translocation for acute promyelocytic leukemia with tons of ayer rods? What do we treat with?
- 15:17
2. all trans retinoic acid
What is a large cell with abundant cytoplasm, fine azurophilic granulation; round nuclei with delicate chromatin and nucleoli?
Monocytic blast- monoblast
What is a Irregular/convoluted nuclear configuration (looks like crumpled tissue paper); granulation more prominent than monoblast (may resemble that of monocytes)?
promonocyte
What are smaller cells, round/oval nuclei, scant cytoplasm which is usually agranular
myeloblast
When do we see monocytic blasts, promonocytes and myeloid blasts?
in acute myelomonocytic leukemia
What do we see in acute megakaryoblastic leukemia on blood smear?
- cytoplamic blebs which break off to become hypo granular platelets (just a little larger than RBCs)
What do we see in acute megakaryoblastic leukemia on blood smear?
1.megakaryoblasts- cytoplamic blebs which break off to become hypo granular platelets (just a little larger than RBCs)
Is AML mainly a disease of children? what percentage of childhood leukemias does it represent? What are the main acute symptoms at onset? Does i often present with leukocytosis? pancytopenia?
- mainly adults
- 20% of childhood leukemias
- fatigue, infections, bleeding
- often leukocytosis
- occasionally pancytopenia
In what AML subtype to we see DIC in?
acute promyelocytic leukemia
In what AML subtype do we see infiltration of gums, skin, CNS more?
monocytic differentiation.
What percentage of patients reach initial remission of AML? is relapse common? What is the 5 year survival for all AML? 5 year survival for children?
- 60-80%
- yes
- 25%
- 65%
What is a neoplasm of lymphoid stem cells with accumulation of lymphoblasts with loss of normal hematopoietic cells? what does the peripheral blood smear show?
- ALL
2. may show markedly increased blasts or only rare blasts, anemia, thrombocytopenia
t-f– ALL is the 2nd most common cancer in children 1-7?
Faslse- 1st
What are the presenting features of ALL?
fatigue, infections, bleeding, bone pain, hepatosplenomegaly, CNS symptoms
Is the prognosis good in ALL?
Yes- most achieve complete remission and approximately 85% of children are cured
What determine lineage in ALL?
flow cytometic markers [most are precursor B cells and less are precursor T-cells)
What are the 2 markers of immaturity in ALL?
TdT and CD34
What are b-lineage markers in ALL?
CD19, 20, 79a, 22
10 is seen in B cell stuff too, but not as prominent in ALL as the above
What are T-lineage markers in ALL?
CD3 !!!! and 2,4,5,7,8
In B-ALL- is a high hyper diploid favorable or not? t(12;21)? t(9;22)? 11q23?
- Yes
- Yes
- No
4 No