Heme/onc: random memorization Flashcards
Life-span of platelets?
8-10 days
What fraction of platelet pool is stored in spleen?
1/3
What do platelet dense granules contain?
ADP
Calcium
What do platelet alpha granules contain?
vWF
Fibrinogen
What’s the vWF receptor?
GpIb
What’s the fibrinogen receptor?
GpIIb/IIIa
What do pmn specific granules contain?
ALP
Collagenase
Lysozyme
Lactoferrin
What do pmn azurophilic granules (lysosomes) contain?
Proteinases
Acid phosphatase
Myeloperoxidase
Beta-glucuronidase
CD14 is a cell surface marker for what cell?
Macrophages
What kind of nucleus do eosinophils have?
Bilobed
Eosinophils make 2 major substances that help limit rxns after mast cell degranulation. What are they?
Histaminase
Arylsulfatase
What do basophils’ granules contain?
Hepatin
Histamine
Leukotrienes
Isolated basophilia can be a sign of what scary disease?
CML
What 3 substances are released upon mast cell degranulation?
Histamine
Heparin
Eosinophilic chemotactic factor
Eosin-5-maleimide binding test is used to screen for what hematologic disease?
Hereditary spherocytosis
Why can patients with G6PD deficiency have increased bac/fungal infections?
Decreased glutathione interferes w/ normal fxns of MPO system, needed for clearance of bac/fungi.
People with HbC defect have what very characteristic finding in their rbcs?
HbC crystals
What is glutamic acid mutated to in HbC?
Lys
Rx of PNH?
Eculizumab
PNH has an increased risk of what kind of cancer?
Acute leukemias
Sickle cell trait: where’s the only site of sickling (under normal conditions)?
Renal medulla
MCC of death in sickle cell dz: adults?
Acute chest syndrome
MCC of death in sickle cell dz: kids?
Infection w/ encapsulated organisms
AutoAbs in ITP?
Anti-GpIIb/IIIa
Prothrombin gene mutation causes a hypercoagulable state. Describe the actual mutation.
Mutation in 3’ UTR–> incr production of prothrombin
What inherited disease would cause a diminished increase in PTT after heparin admin?
Antithrombin 3 deficiency
Which step of protoporphyrin synth requires B6? Name reactants, products, and the enz.
Gly + succinyl CoA–> delta-ALA.
Enz: Delta-ALA synthase
What’s the rate-limiting enz of protoporphyrin synth?
Delta-ALA synthase
Deficiency of ALA-synthase causes what disease?
X-linked sideroblastic anemia
Deficiency of porphobilinogen deaminase causes what disease?
Acute intermittent porphyria
Deficiency of uroporphyrinogen deaminase causes what disease?
Porphyria cutanea tarda
Why is DDAVP helpful in vWD?
Releases stored vWF from the endothelium
What could cause acquired antithrombin deficiency?
Renal failure or nephrotic syndrome
How does leukocyte ALP differ in CML and leukemoid rxn?
Increased in leukemoid rxn; decreased in CML
1 unit of packed RBCs should increase HCT by how much?
3%
1 unit of platelets should increase platelet count by how much?
5000 platelets/ mm^3
How can transfusion affect K levels?
Can cause hyperkalemia due to lysis of RBCs in old blood
What translocation is seen in mantle cell lymphoma? What gene does this activate?
t(11;14), cyclin D1
The mixed cellularity type of Hodgkin’s lymphoma can characteristically release a certain IL, which attracts a specific set of wbcs. Name the IL and wbc.
IL-5, eosinophils
