Bone Marrow Disorders (Holman)

Question 1

What is the first major distinction that occurs in the life of a cell that began as a pluripotent stem cell?

Answer 1

differentiation to myeloid or lymphoid lineage

Question 2

Which of the following cannot be determined about red blood cells via peripheral blood smear?
A. cell number
B. morphology
C. polychromasia
D. left-shift
E. rouleaux

Answer 2

D. Left-shift means that there are more than are expected of the earlier myeloid precursors. This information can be obtained via bone marrow aspiration.

polychromasia is a disorder where there is an abnormally high number of red blood cells found in the bloodstream as a result of being prematurely released from the bone marrow during blood formation. These cells are often shades of grayish blue and can be seen on blood smear.

rouleaux are stacks or aggregations of red blood cells (RBCs) which form because of the unique discoid shape of the cells. This is in indicator of disease.

Question 3

Which of the following cannot be determined from a bone marrow aspirate?
A. dyspoiesis
B. number of megakaryocytes
C. myeloid:erythroid ratio
D. number and morphology of lymphocytes
E. evaluation for metastatic tumor

Answer 3

B. Megakaryocytes tend to get pushed to the edge of an aspirate and are not well represented. They are much easier to find in a biopsy.

With a bone marrow biopsy you can also determine #cells:fat and confirm the myeloid to erythroid ratio.

Because the architecture of the cells remains intact you can also see fibrosis and aggregates of lymphocytes, blasts and tumor cells.

Question 4

When does mitosis cease in a granulocytic cell?

Answer 4

At the myelocyte - metamyleocyte transition. At this point cells stop dividing and just mature.

Question 5

the _____ is composed of neutrophils that are counted in a peripheral blood smear whereas the _____ contain neutrophils that remain adhered to the sides of the vessels until they are mobilized (ie, by stress or infection)

Answer 5

circulating granulocyte pool; marginating granulocyte pool

Question 6

where does nuclear extrusion of red blood cells occur?

Answer 6

in the bone marrow, just before the cells are released into the blood

Question 7

a disease in which stem cells are unable to generate mature blood cells, causing a deficiency of all three blood cell types (pancytopenia): red blood cells (anemia), white blood cells (leukopenia), and platelets (thrombocytopenia)

Answer 7

aplastic anemia

Question 8

disorder in which there are plenty of erythroid precursors but few granulocyte precursors (ie, the body makes an antibody to its neutrophil precursors)

Answer 8

granulocytic aplasia

Question 9

ineffective hematopoiesis, in which the marrow is packed with plenty of granulocyte precursors but they are not able to mature and get out into the blood stream, may be caused by what deficiency?

Answer 9

vitamin b12

Question 10

gum inflammation is a common clinical presentation of _____.

Answer 10

neutropenia

Question 11

an increase in the number of neutrophils, resulting in demargination, mobilization of stores, and increased production is known as reactive _____.

Answer 11

neutrophilia

Question 12

extreme leukocytosis in response to stress or infection that may resemble leukemia; increased immature cells are seen

Answer 12

leukemoid reaction

Question 13

____ leukemia is mostly associated with blast formation and ____ leukemia is associated with differentiated cells

Answer 13

acute; chronic

Question 14

malignant clonal neoplasm of any myeloid lineage cell characterized by the accumulation of myeloblasts in the blood and bone marrow (at least 20%)

Answer 14

acute myeloid leukemia (AML)

Question 15

All of the following are markers of myeloid cell lineage EXCEPT:
A. CD117
B. CD13
C. CD19
D. CD33
E. Auer rods

Answer 15

C. This is a B cell marker.

Question 16

subtype of AML characterized by either microgranules or hypergranules with a butterfly shaped nucleus and multiple auer rods

Answer 16

acute promyelocytic leukemia; caused by a t(15;17) translocation (KNOW THAT), that leads to constituous activation of a tyrosine kinase protein leading to proliferative blast formation and eventually DIC (not good)

Treatment with all trans retinoic acid (ATRA) to allow cells to progress past the blast stage

Question 17

subtype of AML characterized by abundant blast cell cytoplasm and may exhibit extramedullary presentation (ie, skin, gums, CNS)

Answer 17

acute myelomonocytic leukemia

Question 18

subtype of AML characterized by blebbing cytoplasm off the side of the cell

Answer 18

acute megakaryoblastic leukemia

Question 19

malignant neoplasm of lymphoid stem cells, may show markedly increased blasts and is the most common cancer in children age 1-17

Answer 19

acute lymphoblatsic leukemia (ALL)

classified by flow cytometric markers, with most being precursor B cells; good prognosis in children (85% cure rate)

Question 20

What do the presence of auer rods and a positive MPO tell you, without the need for flow cytometry?

Answer 20

indicative of AML

Question 21

leukemia that is rare in children and is characterized by the presence of mostly differentiated cells (e.g., neutrophils and granulocytic precursors), left shift, and invariable basophilia

Answer 21

chronic myeloid leukemia

etiology: t(9;22)
presentation: fatigue and weight loss, sometimes splenomegaly that gets worse with progression of disease

Question 22

drug developed to treat CML, which fits into the binding pocket of the fused constiutively active bcr/abl protein, interrupting phosphorylation of downstream substrates

Answer 22

imatinib (Gleevac); expanded the life expectancy from 5 years to normal (!!)

Question 23

clonal B-cell neoplasm which expresses mature B-cell antigen, monotypic lambda or kappa light chains, and aberrantly express the T-cell marker CD5; “smudge” cells apparent on blood smear

Answer 23

chronic lymphocytic leukemia (CLL)

presentation: > 60 years old, most often in males, generalized lymphadenopathy in 50-60% of cases, widespread lymphocytosis, immune dysregulation

Question 24

name given to clonal myeloid stem cell disorders, which are characterized by cytopenia, ineffective hematopoiesis, and dyspoiesis with an increased risk of progression to AML (1 in 3)

Answer 24

myelodysplastic syndromes (MDS)

presenation: elderly patients >60, 50% are asymptomatic, the other 50% present with fatigue, hemorrhage, infections; variable but generally poor prognosis

Question 25

disorder whose blood smear shows dimorphic red cell population, hypercellular marrow, dyspoiesis, and ringsideroblasts

Answer 25

MDS

etiology: mostly trisomies and deletions

Question 26

Which of these is NOT a type of chronic myeloproliferative neoplasm?
A. Primary myelofibrosis
B. MDS
C. Essential thrombocythemia
D. CML
E. Polycythemia Vera

Answer 26

B.

Question 27

A mutation in this gene is the hallmark of all chronic meyloproliferative neoplasms (excluding CML, which is characterized by a mutation in the BCR/ABL gene)

Answer 27

JAK2 gene

Question 28

All of the following are characteristic of essential thrombocythemia EXCEPT:
A. Most commonly appears in children
B. Splenomegaly is commonly seen
C. May present with either thrombosis or hemorrhage
D. Indolent disorder with long survival
E. Rarely transforms to AML

Answer 28

A. This is an adult disease that most commonly affects people age 50-60

Question 29

All of the following are characteristic of essential thrombocythemia EXCEPT:
A. Usually an adult disease
B. Splenomegaly is commonly seen due to extramedullary hematopoiesis
C. May present with either thrombosis or hemorrhage
D. It is an indolent disorder with long survival that rarely transforms to AML
E. Characterized by large atypical platelets and megakaryocytes

Answer 29

B. This is a feature of primary myelofibrosis.

Question 30

All of the following are characteristic of primary myelofibrosis EXCEPT:
A. Most common in middle age and elderly
B. Associated with poor prognosis with up to 20% developing AML
C. Teardrop red blood cells and clustered megakaryocytes
D. Reactive deposition of fibrous connective tissue in the bone marrow
E. Leukoerythroblastic reaction (ie, circulating bone marrow components in the blood stream) is specific for this disease

Answer 30

E. This reaction may be seen in response to normal stress situations as well (e.g., newborns display this reaction as well)

Question 31

diseases characterized by clonal proliferation of terminally differentiated B cells capable of producing Ig and producing large amounts of M protein

Answer 31

plasma cell neoplasms

examples: MGUS, plasmacytoma, plasma cell myeloma

Question 32

most asymptomatic plasma cell neoplams marked by a small increase in monoclonal (M) protein without overt disease

Answer 32

monoclonal gammopathy of undetermined significance (MGUS)

About 25% of patients progress to plasma cell neoplams over 20 years

Question 33

disease marked by solitary bone lesion or extraosseus lesion, usually lung, pharynx or nasal sinuses; patients do not often show M spike

Answer 33

plasmacytoma

Question 34

bone marrow based disease with involvement of the skeleton at multiple sites and shows significant M spike

Answer 34

plasma cell myeloma

pneumonic for symptoms, CRAB =
Calcemia
Renal insufficiency
Anemia
Bony lytic lesions