Flashcards in Heme Deck 1 Deck (40):
Vitamin B12 is essential for the synthesis of...
tetrahydrofolate, the biologically active form of folic acid
Folate is required for the synthesis of...
deoxythymidylate monophosphate, which is required for DNA synthesis
What kind of thrombophilia is associated with arterial thrombosis
HHC- hyperhomocysteinemia (elevated levels of homocysteine in the blood)
How is hyperhomocysteinemia acquired?
From vitamin B12/folate deficiency or inherited MTHFR deficiency
methylene tetrahydrofolate reductase; deficiency associated with hyperhomocysteinemia and thrombophilia
Myeloid blast phase in CML would contain which markers
CD13, CD33, or myeloperoxidase
Which blast phase of CML responds better to tx?
Lymphoid blast phase responds better to therapy than myeloid blast phase
The characteristic of marrow fibrosis and failure is seen in which 2 disease types?
Primary myelofibrosis and spent phase of polycythemia vera
Characterisitics typical of Primary myelofibrosis
Leukoerythroblastosis, DACROCYTES (teardrop cells), hypercellularity, marrow fibrosis and failure, enlarged spleen
What resembles reactive thrombocytosis
What condition is often associated wtih acute promyelocytic leukemia
DIC- low platelet count and fibrinogen (being consumed leading to bleeding, high number of clots leading to elevated D-Dimer, PT, and PTT
How is acute promyelocytic leukemia inherited?
t(15;17) translocation- rearrangement of retinoic acid receptor alpha gene on chromosome 17 with the PML gene on chromosome 15
important for granulocytic maturation
Tx for acute promyelocytic leukemia
chemo, ATRA- all trans retinoic acid allows granulocytic neoplastic cells to mature, and helps with DIC in acute promyelocytic leukemia
Enzyme present in primary granules of granulocyte precursors
A positive myeloperoxidase stain diagnostic of...
acute myelogenous leukemia
Blasts that express CD10, CD19, CD20, CD22, but not surface immunoglobulin diagnostic of
precursor B ALL
Blasts that express CD13, CD14, CD15, and CD33 diagnostic of
AML caused by recurrent cytogenic abnormality, AML with multilineage dysplasia, therapy related AML (from chemo), and AML not otherwise categorized
2 man features in classifying AML not otherwise classified
Degree of differentiation of the neoplastic cells and the myeloid lineages represented
Different degrees of differentiation of AML
Minimally differentiated, AML without maturation, and AML with maturation
Which form of differentiated AML lacks myeloperoxidase expression
Minimally differentiated AML
Different types of AML based on myeloid lineages
acute promyelocytic leukemia, acute myelomonocytic leukemia, acute monocytic leukemia, acute erythroid leukemia, acute megakaryoblastic leukemia
Acute myelomonocytic leukemia
AML with both granulocytic and monocytic differentiation
What is t(15;17) translocation associated with
form of AML in which the cells cannot matures past the promyelocyte (promelanocyte) stage of differentiation into myelocyte stage
What is t(8;21) translation associated with
AML with maturation
acute myelomonocytic leukemia accompanied by numerous abnormal eosinophils
Abnormality at band 11q23
Acute monoblastic leukemia
What are myelodysplastic syndromes characterized by? (2 main features)
Ineffective hematopoiesis and morphologic dysplasia resulting from abnormal cellular maturation
Hypogranular cytoplasm, giant hypogranular platelets, abnormal lobulated erythroid precursors, and pseudo-pelger huet neutrophils along with hypercellular bone marrow
Difference between myelodysplastic syndromes and myeloproliferatve disorders
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
What can MDS progress to
AML, so take these very seriously
Primary determinants of successful BMT
How close the HLA types are, how severe GVHD is, ability of the host to withstand the conditioning regimen
Basis of acute vs. chronic GVHD
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
What is necessary for bone marrow engraftment?
Donor T cells
What is most important common infection in the first few months post BMT?
"owl-eye" appearance (intranuclear inclusion surrounded by clear halo), general interstitial thickening by mononuclear infiltrate, scattered cells with enlarged nuclei characteristic of
CMV-infection from BMT
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
Acute Graft vs. host disease
How does acute Graft vs host disease affect GI tract
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