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FA organs HY > Heme > Flashcards

Heme Flashcards

1
Q

Increased Hct means?

A

Polycythemia/ Erythrocytosis

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2
Q

What do Dense nd alpha granules contain in platelets?

A 
dense= Ca + ADP
alpha= Fibrinogen + vWF
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3
Q

Blood cell differential?

A 
PMNs= 54-62%
Lymphs= 25-33%
Monocytes= 3-7%
Eos= 1-3%
Basophils= 0-1%
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4
Q

What cell contains Azurophilic granules?

A

PMNs= Lysosomes

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5
Q

Large cell with Kidney shaped nucleus and frosted glass cytoplasm?

A

Monocyte

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6
Q

What cell contains Histaminase and arylsulfatase to help limit rxn following mast cell degranulation?

A

Eosinophils

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7
Q

What do basophils contain?

A

Heparin
Histamine
LTD4

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8
Q

What is CD28?

A

Co-stimulatory signal required for Tcell activation

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9
Q

What coagulation factors are in the COMMON pathway?

A 
X
V
II (Thrombin)
I (Fibrinogen)
XIII (cross links fibrin)
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10
Q

What is the function of Protein C/S?

A

Inactivate Factors V + VIII

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11
Q

What is the function of Ca & ADP released by platelets?

A

Ca=> required for coagulation pathways

ADP=> induces GPIIb/IIIa (fibrinogen) receptor expression

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12
Q

Def in GpIb receptor?

A

Bernard-Soulier==> NO vWF adhesion

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13
Q

Def in GpIIb/IIIa receptor?

A

Glanzmanns thombasthenia ==> binds Fibrinogen

Receptor blocked by Abciximab*

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14
Q

Abciximab function like what disease?

A

Glanzmann’s thrombosthenia

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15
Q

What blocks ADP receptor?

A

Clopidogrel

ticlopidine

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16
Q

What is inside Weibel Palade bodies of endothelial cells?

A

vWF
PGI2
tPA
thromboplastin

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17
Q

Ringed sideroblasts?

A

Excess IRON inside Mitochondria

18
Q

Heinz bodies vs howell jolly bodies?

A

Heinze=> Oxidation of iron leads to denatured Hb

Howell=> Nuclear remnant (asplenics)

19
Q

Mothball (naphthalene) ingestion or asplenic blood smear shows?

A

Howell jolly bodies

20
Q

Triad of Fe def + esophageal webs + Atrophic glossitis?

A

Plummer vinson syndrome

21
Q

HbH vs Hb Barts?

A

Alpha thalasemia
Barts ==> NO alpha chains = 4 Gamma chain= Hydrops
H ==> 3alpha deletion = 4 Beta chains

22
Q

What causes Beta thalasemia?

A

Point mutation @ splice site or Promoter sequence

23
Q

Pt with Increased HbA2 vs HbF (“chipmunk facies”)?

A

HbA2==> beta thal Minor

HbF/chipmunk ==> beta thal Major

24
Q

Lead inhibits?

A

Ferrochelatase + ALA dehydratase

rRNA degradation

25
Q

Hereditary Siderblastic anemia vs INH similarities?

A 
Hereditary = ALA synthase def
INH = B6 (pyridoxine) = cofactor for ALA synthase
26
Q

Child with megaloblastic anemia not corrected with B12 + Folate?

A

Orotic aciduria==> Rx with Uridine monophosphate

27
Q

Newborn with hemolytic anemia, thorny RBCs, and enzyme defect?

A

Pyruvate kinase defect==> dec ATP = rigid RBCs

28
Q

What is Eculizumab?

A

inhibits Complement cascade== Rx for PNH (CD55/59 def)

29
Q

SS mutation?

A

Glutamic acid–> Valine @ p6

>60%= sickling

30
Q

Cryoprecipitate vs FFP?

A

Cryo==> contains Fibrinogen + VIII (given for selective def)

FFP ==> All coag factors (Warfarin OD, DIC)

31
Q

Risks of repeated Transfusions?

A

Iron Overload
Hypocalcemia (citrate chelates Ca)
Hyperkalemia (lysed RBC release IC K+)

32
Q

Why is leukocyte Alkaline phosphatase important?

A

PMNs that are active have ALP
Left shift in Inflammation= Increased ALP
CML left shift = Dec ALP

33
Q

Binucleate cell that is CD30+ & CD15+?

A

Reed Stenrberg cell= Hodgkins

34
Q

Post transfusion pt gets fever, back pain, and hypotension?

A

Hemolytic transfusion rxn = T2HS

35
Q

t(12;21), TdT+, CALLA+?

A

ALL

36
Q

smudge cells on smear + autoimmune warm hemolytic anemia?

A

CLL

37
Q

pt with Basophilia, neutrophilia, increased metamyelocytes. Low ALP?

A

CML

**Can have blast crisis .vs. [CLL which CANNOT]

38
Q

What are auer rods?

A

Azurophilic granules in APML t(15;17)

39
Q

Child with lytic bone lesions, Rash, and biopsy shows S100+ CD1 cells with Birbeck granules?

A

Langerhans histiocytosis

40
Q

What mutation causes Myelofibroproliferative disorders?

A

JAK2 mutations (receptor-associated TK)

FA organs HY (11 decks)

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