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PNCE > Heme JB > Flashcards

Heme JB Flashcards

1
Q

alpha thalassemia labs

A

CBC: hypo chromic microcytic anemia. normal/incr RBC, normal/inc serum iron and iron stores. hgb low
BPS: target cells, Heinz bodies
Hb electrophoresis

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2
Q

alpha thalassemia tx

A

mild (alpha): no tx
mod: folate if reticent high, avoid oxidative stress, avoid iron
severe: blood transfusion weekly, Vit C, folate. iron chelating agents. splenectomy.
bone marrow transplant definitive

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3
Q

anemia of chronic disease labs

A

decreased serum iron
increased ferritin
decreased TIBC

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4
Q

intrinsic (inherited disorders) in hematology

A

sickle cell
thalassemia
G6PD
hereditary spherocytosis

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5
Q

extrinsic (acquired disorders) in hematology

A

autoimmune hemolytic anemia
DIC
TTP
HUS
paroxysmal nocturnal hemoglobinuria
hypersplenism

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6
Q

autoimmune hemolytic anemia tx

A

warm (IgG ab): CS 1st line –> splenectomy or rituximab –> immunosuppressants, IVIG
cold (IgM ab): avoid cold, rituximab. plasmapheresis if refractory
Coombs + distinguishes it from hereditary spherocytosis

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7
Q

beta thalassemia sx

A

minor: asx
intermedia: assoc w/ anemia, hepatosplenomegaly, and bony disease
major (Cooleys anemia)L asx @ birth but sx @ 6mo. frontal bossing, maxillary overgrowth, hepatosplenomegaly, severe hemolytic anemia, osteopenia, iron overload, pigmented gallstones

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8
Q

beta thalassemia dx

A

CBC: hypo chromic microcytic anemia, normal/inc RBC and serum iron.
PBS: target cells
electrophoresis: low HbA, high HbA2, high HbF
xray: skull bossing w/ hair on end appearance

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9
Q

beta thalassemia tx

A

minor: no tx, genetic counseling
major/severe: periodic transfusions, VitC and folate supplementation
avoid excess iron intake. iron chelating agents. splenectomy if refractory.
allogenic bone marrow transplant definitive

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10
Q

CML sx

A

plastic crisis: acute leukemia (splenomegaly, + Philadelphia t9:22, increased WBC)

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11
Q

Protein C deficiency sx

A

recurrent DVT and PE

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12
Q

Protein C deficiency tx

A

heparin –> oral coagulation for life

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13
Q

DIC sx

A

bleeding out and clotting
widespread hemorrhage at venipuncture sites, mouth, nose, extensive bruising
thrombosis

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14
Q

DIC tx

A

tx underlying
severe bleeding –> FFP + cryoprecipitate +/- plt transfusion
severe thrombosis –> heparin

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15
Q

anticoagulants drug mechanism

A

heparin: intrinsic pathway; PTT/ factors 8, 9, 11, 12
warfarin: extrinsic pathway; PT/INR, factors 2, 7, 9. 10

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16
Q

factor V leiden sx

A

hx of pregnancy loss, DVTs or unprovoked DVTs

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17
Q

factor V leiden dx

A

activated protein C resistance assay
if positive, confirm w/ DNA testing
normal PT/PTT

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18
Q

factor V leiden tx

A

high risk: indefinite anticoagulants. may need thromboprophylaxis during pregnancy to prevent miscarriage
moderate risk: (thrombotic event w/ prothrombotic stimulus or asx) prophylaxis during high risk procedures

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19
Q

G6PD sx

A

African American
acute hemolysis from infection, sulfa drugs, or fava beans
jaundice, pallor, dark urine

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20
Q

G6PD dx

A

PBS: schistocytes (bite cells); Heinz bodies

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21
Q

G6PD tx

A

self limiting

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22
Q

good pasture syndrome sx

A

IgG abs against type IV reaction
glomerulonephritis (rapidly progressing) +
pulmonary hemorrhage (hemoptysis)

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23
Q

good pasture syndrome dx

A

bx: linear IgG deposits in glomeruli or alveoli
+ anti-GBM ab

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24
Q

good pasture syndrome tx

A

CS + cyclophosphamide
plasmapheresis

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25
Q

hemochromatosis sx

A

arthritis
bronzing of skin
cirrhosis

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26
Q

hemochromatosis dx

A

increased serum iron, increased serum transferrin saturation. normal/dec TIBC, increased ferritin
+/- increased LFT
liver bx gold standard: increased liver parenchymal hemosiderin

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27
Q

hemochromatosis tx

A

phlebotomy weekly until ferritin dec, transferrin dec, or mild anemia
maintenance phlebotomy 3-4x/yr for life
chelating agents if unable to do phlebotomy

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28
Q

hemolytic anemia tx

A

glucocorticoid and cytotoxic drugs

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29
Q

hemolytic anemia secondary to renal failure tx

A

hemodialysis

30
Q

hemolytic anemia secondary to renal failure sx

A

red/brown urine and plasma
low haptoglobin, high LDH

31
Q

HUS sx

A

kid with GI sx (e. coli, shigella, salmonella)
anemia, kidney damaged (inc BUN/Cr)
thrombocytopenia (petechiae, bruising)
no neuro sx or fever like in TTP

32
Q

.

A
33
Q

HUS tx

A

observation, usually self limited, IV fluids
plasmapheresis if severe, neuro complications, non-renal complications
abx may worsen sx

34
Q

hemophilia A sx

A

factor 8 deficiency
intrinsic pathway
hemarthrosis, excessive hemorrhage in response to trauma and surgery/incision

35
Q

hemophilia A dx

A

low factor 8
prolonged PTT
mixing study w/ normal plasma will correct PTT

36
Q

hemophilia A tx

A

factor 8 infusion
DDAVP before procedures

37
Q

hemophilia B sx

A

Christmas disease
deficiency in factor 9
deep tissue bleeding

38
Q

hemophilia B dx

A

decreased serum factor 9
prolonged PTT

39
Q

hemophilia B tx

A

factor 9 infusion
DDAVP not useful

40
Q

HSP sx

A

MC children after URI
HA, F, anorexia –> rash (legs, symmetrical, palpable purpura)
abd pain, subcutaneous edema, hematuria, proteinuria

41
Q

HSP dx

A

mainly clinical
kidney bx: mesangial IgA deposits
normal coags (PT/PTT)
normal plt

42
Q

HSP tx

A

self limiting
NSAID for joint pain

43
Q

hereditary spherocytosis sx

A

anemia, jaundice, hepatosplenomegaly
pigmented black gallstones (calcium bilirubinate)

44
Q

hereditary spherocytosis dx

A

PBS: hyper chromic microcytosis (RBC lacking central pallor)
+ osmotic fragility test
- Coombs test
increased MCHC

45
Q

hereditary spherocytosis tx

A

folic acid
splenectomy TOC in severe dz

46
Q

hodgkin dz sx

A

20yo or 50yo
painless lymphadenopathy in upper body, hepatosplenomegaly
pel ebstein fever: cyclical fever that increases and decreases over 1-2wks
night sweats, wt loss, anorexia
reed Sternberg cells
associated with EBV (mono)

47
Q

ITP sx

A

usually asx
increased mucocutaneous bleeding: purpura, bruises, petechiae, epistaxis
no splenomegaly
acute: MC in children after viral infx (self limiting)
chronic: MC in adults (often recurrent)

48
Q

ITP dx

A

isolated thrombocytopenia w/ normal coags
PBS: may show megakaryocytes

49
Q

ITP tx

A

child: observation +/- IVIG
adults: CS +/- IVIG, splenectomy if refractory
plt transfusion if <20,000

50
Q

iron deficiency anemia labs

A

decreased serum iron, decreased ferritin
increased TIBC, decreased RDW, decreased RBC/Hgb/Hct
decreased MCV, decreased transferrin saturation
decreased reticulocytes

51
Q

iron deficiency anemia tx

A

ferrous sulfate PO daily

52
Q

type of bilirubin that is always pathalogic

A

increased direct bilirubin

53
Q

CLL sx

A

adults, asx
fatigue, DOE, increased infx, painful lymphadenopathy
hepatosplenomegaly
smudge cells

54
Q

multiple myeloma sx

A

bone pain
recurrent infx
hypercalcemia, anemia, kidney failure

55
Q

multiple myeloma dx

A

M protein spine on electrophoresis
Bence jones proteinuria
Rouleaux formation –> increased ESR
punched out lytic lesions on skull xray
bone marrow bx: plasmacytosis

56
Q

multiple myeloma tx

A

stem cell transplant definitive +/- chemo or alkylating agents (melphan)
bony destruction: bisphosphonates (alendronate)

57
Q

paroxysmal nocturnal hemoglobinuria tx

A

eculizumab (anti complement CD5 ab)
prednisone decreases hemolysis
marrow transplant

58
Q

pernicious anemia dx

A

increased MCV
decreased B12
+ intrinsic factor Ab, parietal cell ab
increased gastrin levels
+ schilling test

59
Q

pernicious anemia watch for which lab during tx

A

signs of hypokalemia

60
Q

polycythemia vera dx

A

screening: Hgb >16, Hct >48%
diagnostic test: subnormal EPO and JAK2 V617F or exon 12 mutation

61
Q

polycythemia vera (primary erythrocytosis) caused by

A

JAK2 mutation

62
Q

polycythemia vera (primary erythrocytosis) sx

A

increased Hct without hypoxia
HA, dizzy, tinnitus, blurry vision
pruritus after hot bath
episodic burning/throbbing of hands and feet w/ edema
splenomegaly, facial plethora

63
Q

polycythemia vera (primary erythrocytosis) tx

A

therapeutic: phlebotomy (management of choice) until Hct <45%
low dose aspirin to prevent thrombosis
myelosupression: hydroxyurea, IF alpha
allopurinol if hyperuricemic
ruxolitinib

64
Q

secondary polycythemia sx

A

increased hct d/t another process
cyanosis, clubbing, HTN, hepatosplenomegaly +/- heart murmur
hx of COPD, renal cell carcinoma, dehydration

65
Q

secondary polycythemia dx

A

increased RBC/hct w/ normal WBC and plt
normal WBC/plt is difference between primary and secondary

66
Q

secondary polycythemia tx

A

tx underlying, quit smoking

67
Q

sickle cell dz aplastic crisis caused by

A

parvovirus B19

68
Q

Sickle cell dz dx

A

CBC w/ PBS: dec Hgb, dec hct, increased reticulocytes
Howell jolly bodies

69
Q

sickle cell dz tx

A

pain management- IV fluids, O2, folic acid, +/- RBC transfusion

70
Q

vaccinations for sickle cell dz

A

children: S. pneumococcus, Hib, N. meningococcus
prophylaxis penicillin 4mo-6yo

71
Q

TTP sx

A

pentad:
thrombocytopenia
microangiopathic hemolytic anemia
kidney failure
neuro sx
fever
increased incidence w/ HIV

PNCE (33 decks)

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