Case Studies

Question 1

Vitamin B12 is essential for the synthesis of…

Answer 1

tetrahydrofolate, the biologically active form of folic acid

Question 2

Folate is required for the synthesis of…

Answer 2

deoxythymidylate monophosphate, which is required for DNA synthesis

Question 3

What kind of thrombophilia is associated with arterial thrombosis

Answer 3

HHC- hyperhomocysteinemia (elevated levels of homocysteine in the blood)

Question 4

How is hyperhomocysteinemia acquired?

Answer 4

From vitamin B12/folate deficiency or inherited MTHFR deficiency

Question 5

MTHFR

Answer 5

methylene tetrahydrofolate reductase; deficiency associated with hyperhomocysteinemia and thrombophilia

Question 6

Myeloid blast phase in CML would contain which markers

Answer 6

CD13, CD33, or myeloperoxidase

Question 7

Which blast phase of CML responds better to tx?

Answer 7

Lymphoid blast phase responds better to therapy than myeloid blast phase

Question 8

The characteristic of marrow fibrosis and failure is seen in which 2 disease types?

Answer 8

Primary myelofibrosis and spent phase of polycythemia vera

Question 9

Characterisitics typical of Primary myelofibrosis

Answer 9

Leukoerythroblastosis, DACROCYTES (teardrop cells), hypercellularity, marrow fibrosis and failure, enlarged spleen

Question 10

What resembles reactive thrombocytosis

Answer 10

essential thrombocytosis

Question 11

What condition is often associated wtih acute promyelocytic leukemia

Answer 11

DIC- low platelet count and fibrinogen (being consumed leading to bleeding, high number of clots leading to elevated D-Dimer, PT, and PTT

Question 12

How is acute promyelocytic leukemia inherited?

Answer 12

t(15;17) translocation- rearrangement of retinoic acid receptor alpha gene on chromosome 17 with the PML gene on chromosome 15

Question 13

Retinoic acid

Answer 13

important for granulocytic maturation

Question 14

Tx for acute promyelocytic leukemia

Answer 14

chemo, ATRA- all trans retinoic acid allows granulocytic neoplastic cells to mature, and helps with DIC in acute promyelocytic leukemia

Question 15

Myeloperoxidase

Answer 15

Enzyme present in primary granules of granulocyte precursors

Question 16

A positive myeloperoxidase stain diagnostic of…

Answer 16

acute myelogenous leukemia

Question 17

Blasts that express CD10, CD19, CD20, CD22, but not surface immunoglobulin diagnostic of

Answer 17

precursor B ALL

Question 18

Blasts that express CD13, CD14, CD15, and CD33 diagnostic of

Answer 18

AML

Question 19

AML classifications

Answer 19

AML caused by recurrent cytogenic abnormality, AML with multilineage dysplasia, therapy related AML (from chemo), and AML not otherwise categorized

Question 20

2 man features in classifying AML not otherwise classified

Answer 20

Degree of differentiation of the neoplastic cells and the myeloid lineages represented

Question 21

Different degrees of differentiation of AML

Answer 21

Minimally differentiated, AML without maturation, and AML with maturation

Question 22

Which form of differentiated AML lacks myeloperoxidase expression

Answer 22

Minimally differentiated AML

Question 23

Different types of AML based on myeloid lineages

Answer 23

acute promyelocytic leukemia, acute myelomonocytic leukemia, acute monocytic leukemia, acute erythroid leukemia, acute megakaryoblastic leukemia

Question 24

Acute myelomonocytic leukemia

Answer 24

AML with both granulocytic and monocytic differentiation

Question 25

What is t(15;17) translocation associated with

Answer 25

form of AML in which the cells cannot matures past the promyelocyte (promelanocyte) stage of differentiation into myelocyte stage

Question 26

What is t(8;21) translation associated with

Answer 26

AML with maturation

Question 27

inv(16)

Answer 27

acute myelomonocytic leukemia accompanied by numerous abnormal eosinophils

Question 28

Abnormality at band 11q23

Answer 28

Acute monoblastic leukemia

Question 29

What are myelodysplastic syndromes characterized by? (2 main features)

Answer 29

Ineffective hematopoiesis and morphologic dysplasia resulting from abnormal cellular maturation

Question 30

Hypogranular cytoplasm, giant hypogranular platelets, abnormal lobulated erythroid precursors, and pseudo-pelger huet neutrophils along with hypercellular bone marrow

Answer 30

Myelodysplastic syndrome

Question 31

Difference between myelodysplastic syndromes and myeloproliferatve disorders

Answer 31

Both produce hypercellular bone marrows, but MDS produces blood cytopenias (dec amounts of erythrocytes, platelets, and WBC's), while myeloproliferative disorders produce increased peripheral counts

Question 32

What can MDS progress to

Answer 32

AML, so take these very seriously

Question 33

Primary determinants of successful BMT

Answer 33

How close the HLA types are, how severe GVHD is, ability of the host to withstand the conditioning regimen

Question 34

Basis of acute vs. chronic GVHD

Answer 34

reactivity of donor lymphocytes present in the bone marrow graft against host tissues- the greater the number of target antigens, the higher the incidence of GVHD

Question 35

What is necessary for bone marrow engraftment?

Answer 35

Donor T cells

Question 36

What is most important common infection in the first few months post BMT?

Answer 36

Cytomegalovirus infection

Question 37

"owl-eye" appearance (intranuclear inclusion surrounded by clear halo), general interstitial thickening by mononuclear infiltrate, scattered cells with enlarged nuclei characteristic of

Answer 37

CMV-infection from BMT

Question 38

Looking at slide, you see small cavities degenerating and necrosis of basal cells and acanthocytes, individual keratinocytes dying with lymphocytes, satellitosis, spongiosis, bullase. What disease process are these associated with

Answer 38

Acute Graft vs. host disease

Question 39

How does acute Graft vs host disease affect GI tract

Answer 39

individual apoptotic epithelial cells in intestinal crypts- most severe changes found in distal ileum. Gland lumen with sloughed epithelial cells and debris, relative sparseness of the mononuclear cell infiltrate in colon

Question 40

Where are the major affected cells in the liver in acute GVH disease located

Answer 40

within the portal triads