Heme Flashcards
Increased Hct means?
Polycythemia/ Erythrocytosis
What do Dense nd alpha granules contain in platelets?
dense= Ca + ADP alpha= Fibrinogen + vWF
Blood cell differential?
PMNs= 54-62% Lymphs= 25-33% Monocytes= 3-7% Eos= 1-3% Basophils= 0-1%
What cell contains Azurophilic granules?
PMNs= Lysosomes
Large cell with Kidney shaped nucleus and frosted glass cytoplasm?
Monocyte
What cell contains Histaminase and arylsulfatase to help limit rxn following mast cell degranulation?
Eosinophils
What do basophils contain?
Heparin
Histamine
LTD4
What is CD28?
Co-stimulatory signal required for Tcell activation
What coagulation factors are in the COMMON pathway?
X V II (Thrombin) I (Fibrinogen) XIII (cross links fibrin)
What is the function of Protein C/S?
Inactivate Factors V + VIII
What is the function of Ca & ADP released by platelets?
Ca=> required for coagulation pathways
ADP=> induces GPIIb/IIIa (fibrinogen) receptor expression
Def in GpIb receptor?
Bernard-Soulier==> NO vWF adhesion
Def in GpIIb/IIIa receptor?
Glanzmanns thombasthenia ==> binds Fibrinogen
Receptor blocked by Abciximab*
Abciximab function like what disease?
Glanzmann’s thrombosthenia
What blocks ADP receptor?
Clopidogrel
ticlopidine
What is inside Weibel Palade bodies of endothelial cells?
vWF
PGI2
tPA
thromboplastin
Ringed sideroblasts?
Excess IRON inside Mitochondria
Heinz bodies vs howell jolly bodies?
Heinze=> Oxidation of iron leads to denatured Hb
Howell=> Nuclear remnant (asplenics)
Mothball (naphthalene) ingestion or asplenic blood smear shows?
Howell jolly bodies
Triad of Fe def + esophageal webs + Atrophic glossitis?
Plummer vinson syndrome
HbH vs Hb Barts?
Alpha thalasemia
Barts ==> NO alpha chains = 4 Gamma chain= Hydrops
H ==> 3alpha deletion = 4 Beta chains
What causes Beta thalasemia?
Point mutation @ splice site or Promoter sequence
Pt with Increased HbA2 vs HbF (“chipmunk facies”)?
HbA2==> beta thal Minor
HbF/chipmunk ==> beta thal Major
Lead inhibits?
Ferrochelatase + ALA dehydratase
rRNA degradation
Hereditary Siderblastic anemia vs INH similarities?
Hereditary = ALA synthase def INH = B6 (pyridoxine) = cofactor for ALA synthase
Child with megaloblastic anemia not corrected with B12 + Folate?
Orotic aciduria==> Rx with Uridine monophosphate
Newborn with hemolytic anemia, thorny RBCs, and enzyme defect?
Pyruvate kinase defect==> dec ATP = rigid RBCs
What is Eculizumab?
inhibits Complement cascade== Rx for PNH (CD55/59 def)
SS mutation?
Glutamic acid–> Valine @ p6
>60%= sickling
Cryoprecipitate vs FFP?
Cryo==> contains Fibrinogen + VIII (given for selective def)
FFP ==> All coag factors (Warfarin OD, DIC)
Risks of repeated Transfusions?
Iron Overload
Hypocalcemia (citrate chelates Ca)
Hyperkalemia (lysed RBC release IC K+)
Why is leukocyte Alkaline phosphatase important?
PMNs that are active have ALP
Left shift in Inflammation= Increased ALP
CML left shift = Dec ALP
Binucleate cell that is CD30+ & CD15+?
Reed Stenrberg cell= Hodgkins
Post transfusion pt gets fever, back pain, and hypotension?
Hemolytic transfusion rxn = T2HS
t(12;21), TdT+, CALLA+?
ALL
smudge cells on smear + autoimmune warm hemolytic anemia?
CLL
pt with Basophilia, neutrophilia, increased metamyelocytes. Low ALP?
CML
**Can have blast crisis .vs. [CLL which CANNOT]
What are auer rods?
Azurophilic granules in APML t(15;17)
Child with lytic bone lesions, Rash, and biopsy shows S100+ CD1 cells with Birbeck granules?
Langerhans histiocytosis
What mutation causes Myelofibroproliferative disorders?
JAK2 mutations (receptor-associated TK)
