Case Studies Flashcards

1
Q

Vitamin B12 is essential for the synthesis of…

A

tetrahydrofolate, the biologically active form of folic acid

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2
Q

Folate is required for the synthesis of…

A

deoxythymidylate monophosphate, which is required for DNA synthesis

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3
Q

What kind of thrombophilia is associated with arterial thrombosis

A

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

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4
Q

How is hyperhomocysteinemia acquired?

A

From vitamin B12/folate deficiency or inherited MTHFR deficiency

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5
Q

MTHFR

A

methylene tetrahydrofolate reductase; deficiency associated with hyperhomocysteinemia and thrombophilia

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6
Q

Myeloid blast phase in CML would contain which markers

A

CD13, CD33, or myeloperoxidase

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7
Q

Which blast phase of CML responds better to tx?

A

Lymphoid blast phase responds better to therapy than myeloid blast phase

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8
Q

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

A

Primary myelofibrosis and spent phase of polycythemia vera

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9
Q

Characterisitics typical of Primary myelofibrosis

A

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

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10
Q

What resembles reactive thrombocytosis

A

essential thrombocytosis

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11
Q

What condition is often associated wtih acute promyelocytic leukemia

A

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

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12
Q

How is acute promyelocytic leukemia inherited?

A

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

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13
Q

Retinoic acid

A

important for granulocytic maturation

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14
Q

Tx for acute promyelocytic leukemia

A

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

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15
Q

Myeloperoxidase

A

Enzyme present in primary granules of granulocyte precursors

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16
Q

A positive myeloperoxidase stain diagnostic of…

A

acute myelogenous leukemia

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17
Q

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

A

precursor B ALL

18
Q

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

A

AML

19
Q

AML classifications

A

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

20
Q

2 man features in classifying AML not otherwise classified

A

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

21
Q

Different degrees of differentiation of AML

A

Minimally differentiated, AML without maturation, and AML with maturation

22
Q

Which form of differentiated AML lacks myeloperoxidase expression

A

Minimally differentiated AML

23
Q

Different types of AML based on myeloid lineages

A

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

24
Q

Acute myelomonocytic leukemia

A

AML with both granulocytic and monocytic differentiation

25
Q

What is t(15;17) translocation associated with

A

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

26
Q

What is t(8;21) translation associated with

A

AML with maturation

27
Q

inv(16)

A

acute myelomonocytic leukemia accompanied by numerous abnormal eosinophils

28
Q

Abnormality at band 11q23

A

Acute monoblastic leukemia

29
Q

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

A

Ineffective hematopoiesis and morphologic dysplasia resulting from abnormal cellular maturation

30
Q

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

A

Myelodysplastic syndrome

31
Q

Difference between myelodysplastic syndromes and myeloproliferatve disorders

A

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

32
Q

What can MDS progress to

A

AML, so take these very seriously

33
Q

Primary determinants of successful BMT

A

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

34
Q

Basis of acute vs. chronic GVHD

A

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

35
Q

What is necessary for bone marrow engraftment?

A

Donor T cells

36
Q

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

A

Cytomegalovirus infection

37
Q

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

A

CMV-infection from BMT

38
Q

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

A

Acute Graft vs. host disease

39
Q

How does acute Graft vs host disease affect GI tract

A

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

40
Q

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

A

within the portal triads