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Heme JB Flashcards

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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
hemochromatosis sx
arthritis bronzing of skin cirrhosis
26
hemochromatosis dx
increased serum iron, increased serum transferrin saturation. normal/dec TIBC, increased ferritin +/- increased LFT liver bx gold standard: increased liver parenchymal hemosiderin
27
hemochromatosis tx
phlebotomy weekly until ferritin dec, transferrin dec, or mild anemia maintenance phlebotomy 3-4x/yr for life chelating agents if unable to do phlebotomy
28
hemolytic anemia tx
glucocorticoid and cytotoxic drugs
29
hemolytic anemia secondary to renal failure tx
hemodialysis
30
hemolytic anemia secondary to renal failure sx
red/brown urine and plasma low haptoglobin, high LDH
31
HUS sx
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
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33
HUS tx
observation, usually self limited, IV fluids plasmapheresis if severe, neuro complications, non-renal complications abx may worsen sx
34
hemophilia A sx
factor 8 deficiency intrinsic pathway hemarthrosis, excessive hemorrhage in response to trauma and surgery/incision
35
hemophilia A dx
low factor 8 prolonged PTT mixing study w/ normal plasma will correct PTT
36
hemophilia A tx
factor 8 infusion DDAVP before procedures
37
hemophilia B sx
Christmas disease deficiency in factor 9 deep tissue bleeding
38
hemophilia B dx
decreased serum factor 9 prolonged PTT
39
hemophilia B tx
factor 9 infusion DDAVP not useful
40
HSP sx
MC children after URI HA, F, anorexia --> rash (legs, symmetrical, palpable purpura) abd pain, subcutaneous edema, hematuria, proteinuria
41
HSP dx
mainly clinical kidney bx: mesangial IgA deposits normal coags (PT/PTT) normal plt
42
HSP tx
self limiting NSAID for joint pain
43
hereditary spherocytosis sx
anemia, jaundice, hepatosplenomegaly pigmented black gallstones (calcium bilirubinate)
44
hereditary spherocytosis dx
PBS: hyper chromic microcytosis (RBC lacking central pallor) + osmotic fragility test - Coombs test increased MCHC
45
hereditary spherocytosis tx
folic acid splenectomy TOC in severe dz
46
hodgkin dz sx
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
ITP sx
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
ITP dx
isolated thrombocytopenia w/ normal coags PBS: may show megakaryocytes
49
ITP tx
child: observation +/- IVIG adults: CS +/- IVIG, splenectomy if refractory plt transfusion if <20,000
50
iron deficiency anemia labs
decreased serum iron, decreased ferritin increased TIBC, decreased RDW, decreased RBC/Hgb/Hct decreased MCV, decreased transferrin saturation decreased reticulocytes
51
iron deficiency anemia tx
ferrous sulfate PO daily
52
type of bilirubin that is always pathalogic
increased direct bilirubin
53
CLL sx
adults, asx fatigue, DOE, increased infx, painful lymphadenopathy hepatosplenomegaly smudge cells
54
multiple myeloma sx
bone pain recurrent infx hypercalcemia, anemia, kidney failure
55
multiple myeloma dx
M protein spine on electrophoresis Bence jones proteinuria Rouleaux formation --> increased ESR punched out lytic lesions on skull xray bone marrow bx: plasmacytosis
56
multiple myeloma tx
stem cell transplant definitive +/- chemo or alkylating agents (melphan) bony destruction: bisphosphonates (alendronate)
57
paroxysmal nocturnal hemoglobinuria tx
eculizumab (anti complement CD5 ab) prednisone decreases hemolysis marrow transplant
58
pernicious anemia dx
increased MCV decreased B12 + intrinsic factor Ab, parietal cell ab increased gastrin levels + schilling test
59
pernicious anemia watch for which lab during tx
signs of hypokalemia
60
polycythemia vera dx
screening: Hgb >16, Hct >48% diagnostic test: subnormal EPO and JAK2 V617F or exon 12 mutation
61
polycythemia vera (primary erythrocytosis) caused by
JAK2 mutation
62
polycythemia vera (primary erythrocytosis) sx
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
polycythemia vera (primary erythrocytosis) tx
therapeutic: phlebotomy (management of choice) until Hct <45% low dose aspirin to prevent thrombosis myelosupression: hydroxyurea, IF alpha allopurinol if hyperuricemic ruxolitinib
64
secondary polycythemia sx
increased hct d/t another process cyanosis, clubbing, HTN, hepatosplenomegaly +/- heart murmur hx of COPD, renal cell carcinoma, dehydration
65
secondary polycythemia dx
increased RBC/hct w/ normal WBC and plt normal WBC/plt is difference between primary and secondary
66
secondary polycythemia tx
tx underlying, quit smoking
67
sickle cell dz aplastic crisis caused by
parvovirus B19
68
Sickle cell dz dx
CBC w/ PBS: dec Hgb, dec hct, increased reticulocytes Howell jolly bodies
69
sickle cell dz tx
pain management- IV fluids, O2, folic acid, +/- RBC transfusion
70
vaccinations for sickle cell dz
children: S. pneumococcus, Hib, N. meningococcus prophylaxis penicillin 4mo-6yo
71
TTP sx
pentad: thrombocytopenia microangiopathic hemolytic anemia kidney failure neuro sx fever increased incidence w/ HIV

PNCE (33 decks)

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