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2018 ABIM Awesome Review > Hematology 6% > Flashcards

Hematology 6% Flashcards

1
Q

Erythrocyte lineage

A

Stem cell-> proerythroblast->normoblast (nucleus)->reticulocyte (bluish cells)->RBC

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2
Q

Inc’d retic count (BM working properly)

A 
etio: 
blood loss - GI, vaginal, hemorrhage
inc'd destruction
Extrinsic - DIC, hemolysis
Intrinsic - membrane protein - hereditary spherocytosis
Metabolic enzyme - G6PD
Globinopathies - SS, Hb
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3
Q

Dec’d Retic count

A 
Dec'd prodxn
dec EPO - chronic renal dz
Dec Fe - iron def anemia
dec globin - thalassemia
dec DNA - megaloblastic anemia
BM failure - aplastic anemia - DO WELL with bm tx
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4
Q

Microcytic (MCV<80)

A

iron def anemia
anemia of inflamm (MCV 78,79,80)
Thalassemia
Sideroblatic anemia

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5
Q

Macrocytic MCV >100

A 
Vit B12/folate (MCV 110)
myelodysplasia
etoh liver dz
liver dz
blood loss
hypothyroid
inc retic hemolysis, blood loss
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6
Q

Normocytic MCV 80-100

A

renal failure
hypothyroid (98-103)
anemia of inflamm (81,82,83)
aplastic anemia

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7
Q

Iron study - normal

A

Iron 50-160, transferrin sat 20-45%, TIC 22-420 Ferritin >20 (station wagon 1/3 full)

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8
Q

Iron def anemia -

A

now school bus very empty

iron <15%, high TIBC (more seats), low ferritin (low iron=low ferritin)

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9
Q

Anemia of inflamm

A

liver secretes hepicidn, inc’d ferroportin in macrophages - internalize feroprtin so that iron can’t get out of macrophages
low iron, low to normal transferrin sat, low TIBC cuz high ferrtin just inaccessible

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10
Q

Hemochormatosis

A

screening test - transferrin saturation is most specific - high iron high ferritin low TIBC (occupied)

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11
Q

Most sensitive assay for IDA -

A

ratio soluble transferrin/log ferritin
>2=IDA
<1 anemia of inflammation

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12
Q

Chronic alcoholism

A

inc’d carbohydrate free transferrin level

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13
Q

Celiac sprue

A

oily stools, itch skin lesions (dermatitis herpetiformis - tx with dapsone) - TTG+, IDA

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14
Q

R/O IDA

A

ferritin >100

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15
Q

Paroxysmal nocturnal hematuria PNH

A

C3 dep on RBC, C4 deposits, C6-9 party->hemolysis - pt wakes up with coca cola urine -> also with abd pain -> thrombus in ODD places (splenic thrombosis) - dx with Flow cytometry CD55/59
Tx: BM tx
slso give meningiococcal vaccine

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16
Q

Anemia of inflammation (chronic dz)

A

RBC can’t use iron from reticuloendotheial stores (Macrophages etc) due to hepcidin release (traps iron in macrophages) in chronic inflamm states like Rheum arthrtisi, LE, TB etc (iron filled macros in BM)

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17
Q

Pagophagia

A

eating ice in IDA

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18
Q

Hg 10, MCV 85, TIBC low, TF sat 22% ferritin low 120 - BM shows?

A

dec sideroblasts and inc iron filled macrophages

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19
Q

70 M or post meno F with Hg9.5, MCV 70, low ferritin - wtd?

A

GI w/u
Young - EGD
Old - colonoscopy

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20
Q

25yo F Hg 9, MCV 63 Ferritin low RDW 17 - hypochromic microcytosis, target cells, pencil/cigar cells dx?

A

Menstrual loss or preg with inc need

Tx - PO iron (IV only with renal failure)

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21
Q

When to check retic count during IDA tx

A

5 days

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22
Q

60yo F DM,RI p.w fatigue, Hg 9, occult neg, EPO 10 (n) started on epo, 4 wks alater Hg only 9.2 wtd?

A

start iron supp - too low to make cells

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23
Q

Hg 10.5, MCV 80, peripheral smear normal - uniform size cells RDW 14

A

inc plasma volume

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24
Q

Hg 10.5m MCV 75, hypochormic microcytic cells RDW 17%

A

IDA

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25
Q

Elderly man p/w n/abd pain diarrhea - slight confusion and gaste distrubance dec biv sense - Hg 9 , MCV78,

A

check urine for heavy metals (lead)

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26
Q

Sideroblastic anemia

A

INH witout B6, etoh abuse, batt workers, porcellin workers

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27
Q

Elderly man works at factor making batteries or lives in old buiding or alcoholic p/w anemai Hg 5, MCV 80

A

Lead tox

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28
Q

Hg A (normal)

A

2 alpha, 2 beta

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29
Q

Hg A2 (small amts ok)

A

2 alpha, 2 delta

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30
Q

HgF

A

2 alpha, 2 gamma - good prognostic factor - hyroxyurea makes Hg F

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31
Q

dec alpha

A

alpha thal

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32
Q

dec Beta

A

Beta thal

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33
Q

Point mut of B chains - SCDz

A

African americans

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34
Q

B thal Trait

A

very low MCV (<75) mild anemia, inc HgA2, inc RBC, RDW normal

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35
Q

Hemogl electrophoresis

A

alpha thal NORMAL

+ in beta thal

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36
Q

B thal Intermedia

A

low MCV, mod anemia lots of Hg A1, non-transf dependent

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37
Q

B thal Major (cooley’s anemai)

A

Hemolysis, transfusion dep - iron overload, hepatoslenomegaly, target cells, tear drop cells, alpha cahins - ppt - heinz bodies

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38
Q

Young woman or man for reg checkup - Hg 12.2, MCV 70 RDW 13.5, spear with hyochromic cells, target cells +

A

Hemoglobin electrophoresis -> b thal

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39
Q

Pt from asia p/w Hg 12, MCV 75 Hg electrophoresis NORMAL - blood refused while trying to donate blood - most likely dx

A

Alpha thal

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40
Q

Sickle cell dz

A

B glob mutation - Hb AS - B1 normal, B2 glut->valine
sickle cell trait (Hg S <50% rest Hg A
Crisis with severe hypoxiemia
Splenic sequestration - PRBC+vol support hold off splenectomy, hematuria

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41
Q

Hg SS -> B1 G->V, B2 G->V

A

Sickle cell anemia - HgS 75-95% - Hg F 2-20%, Hg A2<4%
Functional asplenia - inc capsule bug infxn (s pneumo, h flu, kleb
Vasoocculsive crisis - Acute chest syndorme

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42
Q

Hg SC - B1 G-V B2 G-> Lysine

A

Hg SC dz - aspetic necrosis femur/hum, retinal infarct, vaso occlusion CAN occur

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43
Q

AA male p.w SC anemia, joint pain, fever, Gh 10 LDH ele, retic elev, normla ferritin dx?

A

Vasoocculisive crisis - hydration, alanlesics, oxygen - PRBC if Hg still low
Prev - hydroxuria - more HgF

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44
Q

Pt with severe sicle cell anemia recurrent crisis - what can reduce circulating sickle cells

A

reduce intensity conditioning regiment followed by peripher stem cell allogenic tx from HLA matched donor
don’t use meperidine for analesia - causes seizures

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45
Q

AA SC dz right hip pain - no truama - hip xray diffuse articular sclerosis, decalcif - dx?

A

Avascular necrosis of femur - MRI confirmation

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46
Q

Sickels cell anemia with stroke

A

exchange tx

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47
Q

Sicke cell anemia with CP, fever, no PMNs, CXR infiltrates, morphine given

A

Acute chest - leading cause morality in SC pts - give abx, O2, PRBC - exhange tx if still hypoxic
Etio - microvasc infarction, fat embolism

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48
Q

Pt sickle cell trait LUQ pain - enlarging spleen, dropping hg orthostasis - dx?

A

Splenic sequestation crissi - IVF, PRBC

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49
Q

Pt with Sickle cell dz, joint pains, rash on trunk Hg 6, plt and WBC normal - retic 0%, ANA +, anticardiopan +, parvovirus +

A

Parvovirus B12 aplastic crisis - Dx IgM Ab

Tx - IVIG

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50
Q

Daycare worker with arthalgias - Hg 8 no h/o bleeds, no rash, retic count 0.2

A

Parvovirus B19 (don’t see rash in adults)

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51
Q

Preg teacher pw worriy of Hep A outbreat or parvoivurs B12 in school -

A

IgM-, IgG_ ok protected

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52
Q

Cause of osteomyelitis in SCdz

A

Salmonella>staph 2:1 - need gram neg coverage

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53
Q

Bthal

A

HgA, little HgF and HgA2

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54
Q

B thal major

A

Hg A (little) HgF (LOTS), HgA2

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55
Q

SS trait

A

Lot HgA - little HgF, some Hg S

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56
Q

SS dz

A

no HgA, little HgF, lots HgS, little HgA2

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57
Q

Hg S/C (thal)

A

little HgA, some HgF, lots HgS, some HgA

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58
Q

Pt Hg 13 MCV 70 - Electrophoresis HgA 75%, HgS 25% Hg F0.3%

A

SS trait and alpha thal

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59
Q

Macrocytic anemia

A

> 110 Vit B12 def - hypersegmentation PMN
Folate def
smooth tongue, post col, ineff ertyrhopoess, ele LDH, bili, dec retic
BM Hypercell BM, megalobalsts
MCV < 110
Myelodysplasia, liver dz, etoh, hypothyroid, blood loss (inc retics)

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60
Q

Folic acid def causes

A

etoh, methotrexate, trop sprue, pyrimethamine, pynytoin, herdi sphercytosis, chronic hemolysis

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61
Q

Pt with sickle cell dz and sz d/o on dilatin p/w severe anemia, Hg drop from 11 to 9 - MCV 110, retic 0.7, LDH 750

A

dilantin induced folate def

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62
Q

55yo chronic etoh user with anemia, elev MCV, dec folic acid - dx with folic acid def - started on daily folate - plits inc’d from 150 to 750

A

continue folic aid - known to inc plts

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63
Q

PCP starts 60yo with MCV 120, Gh 10 on folic acid - years later Hg 11.2 - has mild dementia and balancing problems - wtd

A

check B12

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64
Q

folate normal, B12 300

A

check MMA if inc’d still has B12 def

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65
Q

74yo F sx of forgetfulness - romberg sign +, B12 310 - gh 13 MCV 103 wtd?

A

Check MMA

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66
Q

Which medication dec’s absorption of Vit B12

A

metformin (lactic acidosis)

tx - oral B12

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67
Q

B12 vs B6 def

A

high MCV = B12 def

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68
Q

With any chronic hemolytic anemia (SCDz, Hered spherocytosis) pt needs to be on?

A

Folate

HgA1c falsyy low (high cell turnover)

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69
Q

Long time dok worker 80yo pw improper gait and forgetfullness - romberg pos, dec vibration, HCV 118 - B12 275 - MMA elevated dx?

A

Vit B12 defieicnecy (ele MMA)

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70
Q

Folate deficeincy

A

only homocysteine high

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71
Q

Vit B12 def

A

MMA and Homocystein high

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72
Q

Vit B6 def

A

only homocystiene high

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73
Q

Myelodyplastic syndromes

A

ringed sideroblasts
Elderly pt with aemia and or thrombocytopenia or leukopenia - HYPO seg PMN
Macrocytic picture with dysfxn plts, hypo seg PMN
Stem cell defect
refractory anemia with normo/hypercell marrow
BM: marrow precursor dyplasia with ringed sideroblasts
MDS with isolated 5q
>10% blasts - worse prognosis - may tx to AML

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74
Q

MDS Tx

A

55 Azathacine+supportive blood tx, GCSF in WBC
EPO500 - antithyoctye globulin (ATG) - if can’t tol ATG then lenaidomide
5q gene del - lenadolmide

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75
Q

Hemochromatosis

A

Iron o/s with transferrin sat >45%
best screening tool serum transferrin sat
Dx HFE gene testing

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76
Q

Sx hemochrom

A 
pancrease - DM
skin - hyperpiz - porphyria cutanea tarda
heart - CM
LFT cirrhosis, hepatoma, elel lfts
pituitary - hypogonadism
Joints - arthropathy (MCP/wrist joint)
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77
Q

Ferritin >1000 - want to check iron o/l extent -

A

liver bx

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78
Q

Tx for hemochromatosis

A

Lphlebotomy>deferoxamine

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79
Q

45yo M pain in fingers - dec libido, choroinc etoh high stress at work - joint swelling, bronzed skin - Hg 16, MCV 90, Transferrin sat 75%, ferritin 2400, elev ast/alt, elev FBS dx?

A

Dx: Hemochromatosis

Screenign - transferrin saturation

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80
Q

Aplastic Anemia

A 
Pancytopenia with HYPOcellular BM, 
Etio - idopathic or
drugs (sulf, chloramphenicol, radiation
Viruses - Parvovirus, CMV, EBC Hep B,C, HIV
B12 def
Tx < 50 allog BM tx
>50yr ATG, alemtuzumab
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81
Q

Pancytopenia

A 
MDS
B12 def
Fanconi syndrome RTA II
Viral hep B,C
HIV, hyperslpensim, hairy cell leuk, bactrim copper def
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82
Q

40 yo ecchymosis pancytopenia - c/w

A

viral hepatitis

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83
Q

Uremia, low Hg, peripheral smear with burr cells

A

anemia of renal failure - low epo

Tx - epo

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84
Q

Pt with etoh cirrhosis, worsening anemia - elev bilirubin, elev etic - no evid of bleeding, RBC with irr spiculations

A

Spur cell anemia of liver dz -

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85
Q

Pt unstable angina started on nitrates - or post EGD turns cyanotic, SOB - EKG no schanges - pulse ox 85% PO2 96

A

Dx Methhemogobinemia

Tx: methylene blue, vit C

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86
Q

Hemolytic anemias

A

Extracellular def
Acquired intracell
Inherited intracell

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87
Q

Extracell defects

A

Immune hemolytic anemias
Autoimmune - Warm, cold aggultins, paroxysmal cold hemoglobinuria
trauamtic microangiopathyic hemolytic anemia

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88
Q

Acquired intracellular defets

A

Paroxysmal noctural hemoglobinuria

CD55/59

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89
Q

Inherited intracellular defets

A

membrane abnormalities - hereditary spherocytosis

Enzyme abnormalities - G6PD def, hemoglobinopathies

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90
Q

Coombs test

A 
Ab on RBC
Warm Ab - Rh Ag - RBC torn - spherocyte
Diret coombs Ab - IgG+- C3
Etio SLE, CLL, Lymphoma
Drugs Methyldopa, PCN, Procainamide
Tx: Steroids, danazol, splenectomy Immunosupp drugs

Cold Ab - iAg
Direct coombs ab - IgM, C3
Etio - Quinidine, lymphoma, viral IM, flu, mycoplasma
Tx - cyclophosphamides, chlorambucil, NO STEROIDS

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91
Q

PCN/cephalosporin/methydopa

A

Warm Ab (antiIgG)

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92
Q

SLE - type I RTA

A

Warm Ab

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93
Q

CLL/lyphoma

A

Warm and cold

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94
Q

Quinidine

A

COld anti-IgM

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95
Q

Infxn - mycoplams, amono)

A

Cold ab

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96
Q

Role of steroids in tx

A

Warm only

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97
Q

Influenza

A

Paroxysmal cold hemoglobinuria

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98
Q

43yo on bactrim for UTI - develops anemia - LDH and retic inc’d - myoplasma titer inc - cold agglutin + - cause of anemia

A

myoplasma induced hemolysis

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99
Q

48yo M colicky abdomninal pain, u/a no rbc or wbc, hemosiderin +, hg 8, MCV 83, retic 7%, LDH 210, amyl/lip ok, coags ok, direct and indirect coombs neg, abd u/s no stones, MESENTERIC VEIN THROMBOSIS - dx?

A

paroxysmal nocturnal hemolobinuria
Dx: DAF assay/Flow cytometry CD55/59
Tx Allogenic BM tx/eculizumab
Give meningiococcal vaccine 2 weeks prior

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100
Q

Hereditary spherocytosis

A

spherocytes with chronic hemolysis, family hx and gall stones
autosomal dominant - fhx anemia
MC membrane defect (spectrind ef) -> RBC rigid, sphereocyte inc MCHC
Phagocytosed by spleen -> spelnomegaly
Chronic hemolsysi - anemia, gall stones, elev LDH, bili/retic
Smear - Spherocytes with polychromatophilia (retics)

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101
Q

Hereditary spherocytosis dx -

A

osmotic fragility testing

Tx - folic acid - splenectomy

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102
Q

Young woman on routine exam found to have Hg 9g/dL - fhx anemia - MCV 86, MCHC 38, retic 5% - direct coombs test neg, osm fragility inc’d dx?

A

Hereditary spherocytosis - cytoskeletal spectrin membrane defect

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103
Q

35yo F rec URI tx’d with amox or PCN p/w fatigue/pallor, CBC normal 1 ya now Hg 9, MCV 92, MCHC 39, reti 12 +polychromasia, +spherocytes - dx?

A

Autoimmune hemolytic anemia (warm)

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104
Q

G6PD def -

A

anemia after oxidative steress with bite/blister cell - x linked, males, mainly AA
oxid stress ‘Quinine, sufa, dapson, primaquine, fava beans
inc retic, bite cels
G6PD normal

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105
Q

Hemolysis - TTP

A 
fragmented RBCs, thombocytopenia, anemia -> microthrombi, diffuse TTP
Neuro sx - h/a, forgetfullness, tinnitius, wkness
Renal: hematuria
renal failure
fever
Tx - plasmapheresis
DO NOT GIVE PLTS (like gas on fire)
CLotting factos not deficient
PT/PTT normal - (except in DIC)
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106
Q

Renal - HUS

A 
HUS
Bloody diarrhea
Renal failure after uncooked meat
Verotoxin from enteropathogenic E coli 0157:H7
No enuro changes
No fever
Tx - supportive - DO NOT USE ABx - lyse bacteria and cause more sx
HD prn
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107
Q

Liver HELLP

A

Hemolytic anemia
Elevated liver enzymes
Low Plts
3rd trim or post partum

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108
Q

Diffuse - DIC

A

Involves clotting factors elev PT/PTT

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109
Q

Schisto only

A

Prosthetic valves - normal

110
Q

Pregnancy

A

HELLP, pre-eclampsia, acute fatty liver of preg, TTP

111
Q

18yo p/w abd pain and bloody diarrhea, no fever/MS change - low plit, high retic high LDH PT/PTT normal - peripheral smear schistocytes, retics, elev Cr - dx?

A

HUS

112
Q

Premature ejaculation

A

SSRI

113
Q

25yo normal labor p/w fatigue post partum, petetchiae over arms - gh 8, plt 20, retic 7, LDH 850, PT/PTT normal, BUN 30/Cr 1.6, AST 120 ALT 250, anisocytosis shistocytes + dx?

A

HELLP

32 wk - early delivery

114
Q

Young woman p/w mild confusion h/a x 2 days - PE palor, temp 100.4, RBC frag, Hg 9.5, plot 25, polychromasia, BUN/Cr 40/2.5, Pt/PTT normal - dx?

A

TTP
Hemolysis, anemia, thrombocytopenia, neuro sx, renal failure
Tx: plasmaphoresis (if no machine then plasma exch / infusion)

115
Q

Pt with plt 50K wit PNA - how to get TTP dx

A

Peripheral smear - schistocytes

116
Q

Which condition will you see schistocyte?

A

DIC, HUS, HELLP, TTP

117
Q

Pt getting blood tx - w/in one hour restless, dyspnic, lower back pain, fever chills, temp 101.5, HR 110, urine dark red, Hg 9 to 7 - direct coombs + IgG dx?

A

Major hemolytic rxn - ABO incompatilbiity

usually clerical error

118
Q

Post transplant blood tx

A

irradiated, CMV neg blood

119
Q

Rh incompatibility

A

delayed rxn - day 6 palor, icterus

120
Q

IgA def

A

PRBC with IgA - anaphylaxis

Need WASHED PRBC

121
Q

Pt gets 2U PRBC during surgery - returns 1 wk later with dark urine and mild icterus - Hg 11 to 9

A

Rh incompatibility - Rh incompatibility - alloimmuniz from prior Tx or preg - coombs usually neg after 1 week when hemolysis done - tell pt they need Rh NEG blood in future
No role for steroids

122
Q

After massive tx - which electrolyte decreased

A

Calcium

123
Q

After massive tx - pt has seizure - cause?

A

Citrate toxicity

124
Q

50yo etoh p/w GIB - 1 month ago hospitlized with mallory weiss tear and got 2U PRBC - on exam bp 80/60, HR 120, Hg 6.5 - got IVF and 2 units PRBC - 2 days later d/c planned but has light headedness - temp 101.5 - and repeat Gg 6.1 - what is best test -

A

Coombs test

125
Q

Pt with h/o urticaria and allergies but needs blood tx - wtd?

A

Washed PRBCs

126
Q

Pt w/ fever chills every time tx’d, no drop in h/h dx?

A

febrile rxn from leukocytes -

get leukocyte poor PRBCs - prevents NON-hemolytic rxns

127
Q

Pt gets PRBC - few minutes later HR inc, BP drops, SOB, RR high swelling lips - temp 99

A

IgA deficiency - anaphalaxis from IgA in PRBC (never seen IgA b4)
tx: Tx with WASHED PRBC

128
Q

Pt get plt tx - 2 hours later fever, hypotension, h/a, CP olgiuria bu tno red urine - dx?

A

bacterial contamination (plt’s stored…)

129
Q

Pt recieves PRBC - 1-3 hrs later cough, fever SOB, BP 90/60, 110 rr 24 pulse ox 85% - direct coombs neg - JVP 5 CXR infiltrates - urine normal dx?

A

TRALI - transf assoc acute lung injury

Anti-leukocyte ab from donor

130
Q

18yo to get solid organ tx - father insists on donating blood -

A

needs HLA matched WBC in donor blood - prev GVHD

irradiate fathers blood to prevent GVHD

131
Q

Pt with aplastic anemia gets allogenic BM tx and needs blood

A

tx irradiated PRBC

132
Q

Pt s/p BM tx, CMV neg needs blood - wtd?

A

irradiated, CMV neg blood

133
Q

25yo F 3 kids with severe menorrhagia low hg given blood tx - 1 week later with diffuse purpura - PT/PTT normal low plts

A

Post tx purpura
Etio - PLA-1 ab - alloimmunized with prior tx or preg
Tx - IVIG

134
Q

Pt unergoes complicated CABG gets 12 units PRBC, 12 units FFP - oozing blood at at drain sites and petechiae on legs - ptt/pt normal plts 30 - dx?

A

dilutional thrombocyotpenia

etio - massive prbc tx without plts - give plts next time

135
Q

45yo acute GIB with orthostatic hypotension - 8 unts PRBC and vigorous hydration then bleeds again several hours later with prolonged PT/PTT etio?

A

Plasma washout

tx - FFP

136
Q

55yo scheduled fo relective CABG - consult for AC intraop and post op PPX - had DVT in past was tx’d - HIT neg - best management

A

IV hep durign surgery + SQ fondaparinex post op

137
Q

Pt with severe MVA needs massive tx -

A

any blood group - give O neg
AB+ can get any blood (univ receipient
O- can only get O neg blood but can give to anyone (universal donor)

138
Q

Primary hemostasis

A

Platelets
immediate,
Superficial - petechiae, ecchymossi, purpura,
inc’d bleeding time, NORMAL PT/PTT

139
Q

Secondary hemostasis

A 
Clotting factors
Delayed
Joint bleeds, hematomas
Normal bleeding time
Inc'd PT - extrinsic pathway
In'd PTT - intrinsic pathway
140
Q

Primary Hemostasis - Dysfxn plts

A 
Dysfxn plts
Von Willedbrand
Bernard Soulier (giant plts)
COX inhib
Glanzmann's dz
MM
RF
141
Q

Primary Hemostasis Dec’d plts

A 
Dec'd plts
Artifact - due to EDTA - use blue to tube
ITP
TTP/HUS/HELLP - dec plts, dec Hg
DIC - dec plts, dec clotting factors
Heparin dec plts, inc thrombus - plt 4 hep complex
dilutional (s/p cabg
Tx purpura
gestation thrombocytopenia
142
Q

Microangiopathic anemia

A 
HIT dec'd by 50% no matter what level it starts at
plot 4 hep complex
antigenic
plts cry for help
thormbosis
143
Q

Plt dysfxn d/o vWF dz

A

Leading autodominant bleeding d/o, inc’d BT, inc PTT, PT normal, dec ristocetin cofactor assay
Tx - mild - DDAVP - stim’s vWF, fact VIII
Severe - Fac VIII conc (also contains vWF) -> cryoprecipitate

144
Q

Bernard Soulier dz

A

Giant plts, inc’d BT

145
Q

COX inhib

A

from ASA/NSAIDs

146
Q

Glanzmann’s dz

A

GP Ib defect (rct for vWF)- abn plt aggregation

inc BT, plt count normal

147
Q

GP IIb/IIIa inhib

A

abcimimab/eptifibatide/tirofiban
U/A
post PTCA

148
Q

Pt with inc BT, inc PTT dec ristocetin facto rassay

A

vWF dz

149
Q

Pt withinc BT normal PTT, giant plts

A

Bernard Soulier

150
Q

Pt with inc BT, nl PTT, nl Plts, abn plt aggregation

A 
Glanzman's dz
Ib defect (rct for vWF)
151
Q

40yo F recently started on ASA develops heavy menses dx?

A

vWF dz

152
Q

Pt with mucous membrane bleeds, protein 9.5, Ca 12.5, BUN/Cr 40/4.5 plt 95 cause of MM bleeds is likely…

A

MM/renal failure

153
Q

Pt withi ESRD with MM bleeds - etiology?

A

plt dyfxn from ESRD

Tx: DDAVP

154
Q

24yo 3rd trim plt 85

A

Gestational thrombocytopenia

benign - monito rplts q102wks no steroids no IVIG

155
Q

ITP

A 
Antibody vs plts
iolated  thrombocytopenia +- superficial bleeds - splenic sequestration
almost normal Hg, normal PT/PTT
Thrombopoeietin N or inc, plot prodxn dec, inc'd plt destruction
Etio - idopathic
quinidin, heparin
lymphoprolif d/o, collagen vas dz
HIV, HCV
Tx
>30K no tx
spelnectomy (cyclophasamide)
<100 with IC bleed - IVIG + plts
No need for HLA matched plts (only for pts alloimmunized to HLA antigens
156
Q

Pt with large ecchymotic area 8x12 on thigh, plts 12, PT/PTT normal - smear with low plts and megakarycyte

A

Bernard soulier -> prednisone

157
Q

Pt with multiple bruises, plt 15, Pt/PTT nromal wtd?

A

Prednisone

158
Q

Preg pt with ITP on steroids - plt 9 - wtd?

A

IVIG + steroids

159
Q

Young female dx with ITP this year with menorrhagia Hg 9 multiple bruises on leg - plt 9 - wtd?

A

IVIG

160
Q

Pt’s with throbocytopenia need what checked?

A

HIV/ HCV ab

161
Q

1 unit plts will inc plts 1 hr later by…?

A

5K

162
Q

Pt scheduled for splenectomy - wtd prior

A

pneumococcal / flu shot - (also before TNF alpha, rituximab

163
Q

Pt s/p hip replacement on SQ hep for DVT ppx - 4 days later pain in r calf - +DVT dx?

A

HIT -

Tx - stop heaprin start argatroban or lepirudin (DTI)

164
Q

Pt with angina on heparin - plts 250, on 4th day plt 100 - no bleeding or thrombus - wtd?

A

d/c hep and start DTI (argatroban)

165
Q

Low GFR which DTI ok?

A

Argatroban - cleared by liver ok in RF

Lepiriduin cleared in kidney

166
Q

Pt with DVT - on heparin and warfarin - 4th day HIT + wtd?

A

d/c hep, d/c warfarin start DTI (Lepirudin) start coumadin when Cr normalizes

167
Q

Pt with UA on hep - 200 plts - 3 days late r145 - wtd?

A

still ok c/w heparin

168
Q

Can LMWH be used in HIT

A

NO - cross reacts with heparin

169
Q

Hep-coumadin bridge

A

protein C decreases faster than thrombin so clots - need heparin to bridge a/c until coumadin catches up (knock off thrombin)

170
Q

Pt with plt 250, next day 5K no sx, no bleed, no drop in Hg, PT/PTT nl, LDH nl what happened?

A

repeat manula plt count

EDTA in blodo tube clumping - artifact

171
Q

Pt with DVT start on A/C with hep 2 days later hypotensive - Na124, K5.6 dx?

A

b/l adrenal hemorrhage 2/2 heparin - hydrocortisone, has

172
Q

Pt on heparin for DVT ppx - 10 days later serum Cr 1.4 but K 4.5 to 6 wtd?

A

change to fondaparinux (arixtra)

173
Q

Secondary hemostasis Intrinsic pathway (PTT)

A

Intrinsic pathway XII (no bleed) -> XI (rare bleed) ->IX (mod bleed) ->VIII(severe bleed - if MVA F VIII concentrate) -> X ->
prothrombin -> (V, X)-> thrombin -> fibrinogen->mono->poly

174
Q

Xa inhibitors - inhibit thrombin formation and thrombi formation

A

fondoparinux (arixtra) LMWH apixiban, rivaroxiban

175
Q

Extrinsic pathway (PT - vit K dep)

A

Tissue factor->VIII->

176
Q

Direct thrombin inhibitors

A

Lepiruden, argatroban, bivilirudin (angiomax), Dagitroban (pradaxa)

177
Q

Russel vipor venum test

A

mixing study - if clotting times continues to be prolonged then not just clotting factor problem - there is lupus anticoagulant (antiphospholipid ab)

178
Q

Inc PTT, nl Pt

A 
on hep - yes normal
no bleed - factor XII def
mild or rare bleed -> XI def
mod bleed IX def
severe bleed - VII def
179
Q

inc PTT bleed corrected b 1:1 mix normal plasma

A

factor deficiency

180
Q

in PTT bleed NOT corrected with mixing study

A

inhibitor (aquired)

181
Q

inc’d PTT no bleed NOT corrected by mixing study and inc’d r/o thrombosis/abortions

A

lupus A/C syndorme - check dRVVT

182
Q

25yo F wk left arm and aphasia h/o 3 spontaneous abortions - PE with L hemiparesis CBC incl plts normla PTT 62 s on mixing study PTT doesn’t correct

A 
antiphospolipid Ab (lupus AC)
PT INR 2-3
183
Q

Pt on exam PTT 75s surgery 2 ya no complciations - FXI levels low but mixing study did not correct, dRVVT prolonged dx?

A

Lupus anti-coagulant syndrome

184
Q

Inc’d PT, nl PTT

A

vit K def
F VII def
on coumadin

185
Q

Inc PT/PTT

A 
common pathway def
multiple factor def
DIC
Liver dz
Coumadin
brodifacoum (super warfarin posining)
186
Q

Pt with laceration delayed bleeding - INH for 8 montsh fo rPPD, cut not healing 10 days later PT/PTT/BT/plts nromal - plt aggreg study normal - clot retraction test ABNORMAL

A

factor XIII def

187
Q

Pt for pre-op - PTT 90 s, PT nromal - PTT corrected with normal plasma

A

dx F XII, XI, IX, or VIII def

188
Q

Pt post op in ICU with inc PT INR 2.4 not on coumadin admissioni INR nromal -

A

F VII def

189
Q

Which factor def NOT have excessive bleeding

A

F XII

190
Q

20yo M epistaxis for 2 days - denies any meds no fhx bleeding
Admission INR>9, PTT >30, mixding studies INR 1.2, PTT 34, post Vit K/FFP INR 3.3, PTT 62 - 24 hrs later INR>9, PTT>90

A

Brodifacoum super warfarin poisoning

tx: vit K for months, monitor PTT

191
Q

Hemophilia A (fact VIII def

A

Deep bleeds, hematomas, hemarthroses - inc PTT, nl PT, nl plts, nl BT
X linked, female carry, males suffer
Deficiency <5% mod
Tx - Factor VIII concentrate (do not use DDAVP)

192
Q

Pt with mild hemophilia A 10% factor VIII - dental extraction wtd?

A

desmopressin spray +- aminocaproic acid swich (antifibrinolytic agent protects clot

193
Q

Pt with hemophilia A with head truama

A

Factor VIII concentrate keep level >50% x 2weeks

194
Q

Pt with hemophilia A for major surgery wtd

A

Factor VIII concentrate prior to surgery and up to 72hrs later

195
Q

Pt with hemophiia dev ab to FVIII has trauma

A

Factor VIIa concentrates - indirectly inc’s thrombin

196
Q

Pt with factor IX def - sustains trauma with fracture of femur wtd?

A

F IX conc or FFP

197
Q

Prescribe warfarin for pt with afib and high chad score - finds out its rat poson wtd?

A

continue warfarin, check PT/INR

198
Q

Pt with afib on coumadin INR 5 no active bleed - wtd

A

lower dose and hold one dose

199
Q

INR 5 to 9 no active bleed

A

hold coumadin - low dose vit K - recheck INR when drops to 3 start coumadin at lower dose

200
Q

INR>9 wtd (on coumadin)

A

hold coumadin high dose vit K +-FFP recheck INR when drops to 3 then restart coumadin at lower dose

201
Q

INR 3 with acute bleed like GIB - wtd?

A

high dose vit K and FFP

202
Q

Pt shceduled for surgery and has afib - warfarin stopped to 3 days ago - morning of surger INR 1.6 wtd

A

clear for surgery

203
Q

45yo chornic alcoholic with cirrohosis and protal HTN with ascites with elev PT/PTT plts 90K Hg 9g scheduled for prota caval shunt - surgeon consults youa bout bleeding complications wtd?

A

FFP 4-6 hrs prior to procedure

204
Q

DIC

A

fibrin split prod +, d dimer +, inc PT/PTT, dec plts, dec fibrinogen, dec shistocytes
“consumption coagulopathy inc PT/PTT, dec plts
Etio - truama, ob complication - tuptured placenta, amniotic fluid embolism retained dead fetus, preeclampsia, sepsis, TNF from tumor, AML
Tx:
Treat underlying d/o, FFP, plts, antithrombin III conc

205
Q

Pt with metastatic lung CA dev sudden fluish toes and finger plits dec, PT/PTT normal/slightly elev, LFT nl, Fibringoen nl

A

Chronic DIC 2/2 cancer

Tx - if thrombi - heparin

206
Q

Pt with abruptio placentae with retained dead fetus, dec hg, dec plt, inc PT/PTT wtd?

A

extraction of fetus

207
Q

Woman with ecessive bleed after D&C - plt ct 45 d dimer elv - follow pt with ?

A

D-dimer - good NPV, dec prob PE

208
Q

Throbotic d/o

A 
Congenital
FV leiden (activ ptoein C resistance (>50yo)
FaII 20210 defect
Protein C/S - 20 to 50yo
ATIII
Dysfibrinogenemia, inc homocysteine
209
Q

Thrombotic d/o aquired

A 
Aquired
Antiphopholipid syndrome
PNH CD55/59
HIT
Cancers
Nephortic
Oral contraceptives
Tamoxifen, raloxifeneAsyx pt with abv conditions don't need a/c unless in increased risk time (surgery, immobiliz etc)
210
Q

35yo M plans travel from tokyo to NY h/o FV leiden defect

A

recommend - hydration and leg excercises during flight, no A/C

211
Q

55yo M NY to Hawaii - h/o DVT in past - best management along with hydration and leg excercise

A

Graduated knee high compression stockings (if >40yo)

212
Q

Pt h/o FV leiden or protein C def with DVT management?

A

Coumadin - 6mo (first time)

213
Q

Pt develops 2nd DVT - wtd?

A

AC for life (coumadin)

214
Q

55yo causcasian p/w leg pain -> DVT after flgiht from hong kong to LA most likely cause?

A

FV leiden or activated protein C resistance

215
Q

Pt dx with DVT started on coumadin INR 3 - 3 days later wk left side

A

Protein C def (pro-coagulant period - needed heparin or lovenox bridge)

216
Q

When to check protein C after DVT/tx

A

once 3 to 6 mo AC done and coumadin stopped x 2 weeks

217
Q

Pt with prolongued PTT, does not correct mixing or adding regular plasma - corrects when adding phopholipids - no DVT

A

monito rif pt has DVT then start heparin and coumadin

218
Q

Pt history of Breast CA->DVT wtd

A

long term LMWH (better than coumadin for onc)

219
Q

25yo preg F with DVT SOB - wtd

A

LMWH throughout preg and up to 6 weeks after delivery

220
Q

26yo F preg had DVT after knee surgery 5 ya - tx’ed with ac for 6 months

A

Only 6 weeks LMWH during post partum period (most thrombotic period) - heparin or warfarin

221
Q

Woman with prothetic valve on warfarin becomes preg and on 5th week

A

stop warfarin start heparin on 6th week - restart coumadin 13th week - continue up to mid third trim and start hep until delivery - post delivery restart coumadin

222
Q

25yo F h/o 3 abortions 1st trim - 8 wks preg - no h/o bleeding or hematomas - PTT elev, plts normal on mixing PTT doesn’t correct - does crrect with mixing phospho lipids and PTT correst -> antiphospholipid syndrome - tx?

A

Heparin or LMWH plus ASA antepartum and postpartum

223
Q

Rev cause of DVT (OCP)

A

warfarin x 3 months

224
Q

F V leiden or protein C def with DVT

A

coumadin x 6 months

225
Q

Cancer with DVT

A

extended LMWH

226
Q

Lupus anticoagular with elev PTT/ +DVT

A

coumadin indefinetly

227
Q

Lupus A/C elev PTT NO DVT

A

observe

228
Q

4 days after d/c for large DVT in leg/thigh PT/INR 1.1 on 5mg coumadin - wtd?

A

begin LMWH, inc warfarin to 7.5

229
Q

Transitioning of LMWH to warfarin

A

5 days LMWH and warfarin with target INR of 2 for 24 hrs

complication - warfarin skin necrosis

230
Q

Pt with skin necrosis what does pt have?

A

Protein C deficiency

tx? FFP - repletes protein C

231
Q

Myeloproliferative d/o’s

A

Autononous and unchecked prolif of one or more cell lines
risk of bleeding or thrombosis
can burn out myelofibrosis
splenomegaly
risk of acute leukemia
Can clog blood vessels->acute vasoocclusive crisis

232
Q

Polycythemia Vera

A 
Inc'd RBC mass, dec EPO, PO2 normal, pruritis after bathing
JAK2+, inc'd plts
inc'd risk of thrombosis (bud chiari)
Splenomegaly
Inc retinal vein involvement
erythromelagia
?iron def
Dx: Hct>60 + splenomegaly or JAK2 mut
Tx Phlebotomy Hct<45 , hydroxyurea
233
Q

65yo pt with PCV undergoes regular phelbotmies for 3 yrs with good results hasn’t required ini 6 months now with fatuge no arthralgias, splenomegaly, fatigue - blood spear shows TEAR DROP, TARGET cells, nucleated RBCs - etiology of anemia?

A

Myelofibrosis
see bizarre cells (tear drop cells)
see erythroid precursors (nucleated RBCs)

234
Q

How to manage pt with PCV then myelofibrosis with sever anemia

A

Blood transfusion

235
Q

65yo M dyspnea/headaches - chornic smoker - 35pk years - Hg 23, MCV 78, plt 550, alk phos normal sao2 normal, B12 high - tx?

A

Phlebotomy + low dose ASA

236
Q

MC seen with PCV

A

splenomegaly

237
Q

Secondary erythrocytosis

A

Hypoxia - COPD, R->L shunts, high altitute
Neoplasm - retinal tumors, cerebellar tumors
Other - polycystic dz, steroids, androgens

238
Q

Essendtial Thrombocytosis

A

inc plts, vasoocculsiv sx, h/a, stroke, plt>600k
ischemic sx >1.5 million
Vasomotor sx - erythromelagia, livedo reticularis, migrains, parathesias hand /feet
Smear - inc plt, clumps, JAK2
BM - megakaryocytes, fibrosis
Tx Stroke - plateltepheresis
Non-urgent - hydroxyurea, anagrelide keep plt <600
Low dose ASA for vasomotor sx

239
Q

Reactive thrombocytosis

A 
plt <800, secondary factor usually pressent
etio iron def
chronic infxn/inflamm
neoplasm
chornic blood loss
splenectomy
post megaloblastic anemia tx
240
Q

20yo F fatigue, palor - menses heavy - Hg 9, plt 800 MCV64 - most likely cause of thrombocytosis?

A

Reactive thrombocytosis from IDA

241
Q

25yo F routine physical - no complaints and no meds plts 800 wtd?

A

Observation….

242
Q

Pt h/o PCV p/w burning sensation in foot - erythema/warmth, tenderness, gangren of toe

A

erythromelagia - cuase plts (essential thormbocytosis) or RBCs (PCV)
Tx: ASA

243
Q

CML - chronic <2% blasts - better formed cells

A

inc WBC, dec LAP (leukocyte alk phosphate score, Phil chrom +, Splenomegaly, WBC>50, myleloid series (PMN, eos, baso, monocytes)
Dx: FISH for t(9,22) - Ph or PCR for abl/bcr fusion gene
25%/yr tx blast->AML - fever, night sweats, bone pain, blast cells -> poor prognosis
Tx: allogenic BM tx
>50yr - imantimab, mesylate (gleevec) tyrosine kinase inhib, alpha interon, hydroxyurea, busulfan
Splenomegaly - early satiety, LUQ fullness

244
Q

Leukemias

A

ALL55

245
Q

Dec LAP in…

A

CML, PNH, wilson’s dz (neuropsych, tremor)

246
Q

Myelofibrosis with myeloid metaplasia

A

Splenomegaly, tear drop cells, drytap, anemia, variable cytopenia
Other myeloprolif dz BURN out to myelofibrosis
Extramedullary hematopoeiesis->spelnomegaly
Smear - tear drop cells, nucleated RBC, giant plts
BM asp - dry tap
BM bx - inc collagen reticular stain, dysmorphic megakarycytes
Tx 55 supportive with blood tx, +- thalidomide

247
Q

AML acute myelogenous leukemia

A

Young adult with superficial bleeds - infections, leukocytosis,
AUER rods
blast cells - prev alkylateing agents, topoisomerase inhib, prev myeloprolf d/o’s (CML, myelodysplasia or fibrosis)
Infiltration of BM with WBC
inc immature WBC -> blast cells in smear and BM, infxn
Dec plts - superficial bleeds
Dec Hg - anemia, fatigue
Myeloperoxidase stain + in M2 to M5

248
Q

M3 AML - acute pro myelocytic leukemia

A

t15,17 Aur rods - good prognosis -

tx ATRA all trans retinoic acid

249
Q

AML bad prognosis

A

T(9.22) abr/bcl, prev CA, multiple cytogenetic abn, dx with bm bx/cytogenic studies, immuniophenotype
Tx < 50yo Allogenic BMTx
>50yo araC (cytarabine) and DNR (daunorubicin)
M3 - ATRA

250
Q

42yo M with AML M3 began on ATRA - next week with low grade fever - SOB, lE edema no CP, no cough, wt inc’d by 10 lbs , Cr up dx?

A

ATRA differential syndrome

Tx Dexamethasone, d/c ATRA (cytokine leak from differentiating cells)

251
Q

You would fine myeloperoxidase stain + in which of following

A

Acute PML (M3 AML), microsopic polyanteritis angiitis

252
Q

50yo F with sudden onset fever, leukocytosis and erythematous plaques and nodules on face and extrem - lesions are tender, bx shows dense neutrophilic infiltrate dx?

A 
Sweet syndrome (acute febrile neutropenic dermatosis) 
skin dz with fever, leukocytosis
Bm bx - cytogenics study
a/w leukemias - mainly AML
Tx: Steroids
253
Q

CLL

A

> 60yo lymphocytosis, lymph node +, smudge cells, AIHA, infxns
Lymphocytes - small mature smudge cells
Hypogammaglobulinemia - recurrent infxns
Autoantibodies- autoimmune hemolytic anemia
Lymphocytes express T cell marker CD5 and B cell marker CD20/23 (rituximab)
Tx
Asymptomatic : none
Symptomatic - fludarabine, chlorambucil, rituximab
IVIgG for infections

254
Q

62yo M with URI WBC 18, lymphocytes predominant - smear with mature lymphs and smudge cells dx?

A

CLL - lymph 80%, sudge cells, PMN 10%

255
Q

Pt p.w WBC 40, lymph 80% dx?

A

CLL
Indication for CLL tx
Lymphocytosis alone - observation
Lymphocytosis + anemia Hg<100 - treat

256
Q

Pt with CLL - Hg <100 management?

A

Chemo

257
Q

70yo with PNA twic in 1 year, h/o CLL dx 2 ya - told tx not needed as Hg 13, plt 140 - now Hg 12, retic 3 LDH 250, polychromasia - most likely cause recurrent PNA?

A

Hypogammaglobulinemia

Prevention: monthly IVIG

258
Q

Reason for CLL pt with sudden onset anemia and spherocytes on smear

A

Warm autoimmunit hemolytic anemia
Tx - steroids
If no response then - immunosupp agents - dexamethasone, cyclophosphamide, rituximab

259
Q

Hairy cell leukemia

A

Pancytopenia, hairycells - TRAP+ (tartrate resistant acid phosphatase)
Elderly pt with pancytopenia, marked splenomegaly
Smear - filamentous hairy cells
BM: fibrosis
Tx: Cladribine-> rituximab (clairy with long hair)
Complications - infxn, vasculitis

260
Q

Which most likely to got AML

A

CML

261
Q

BLister/bite cell

A

G6PD deficiency

262
Q

Acanthocyte/spurr cell

A

etoh anemia, liver dz

263
Q

Echiniocyte/burr cell

A

uremia anemia - dec epo

264
Q

Howell jolly bodies

A

splenectomy/fxn asplenia

265
Q

Normoblast

A

severe hemolysis/myelofibrosis

266
Q

Polychromatophilia (reticulocytes

A

hemolysis, blood loss, BM working

267
Q

Schistocytes

A

TTP, HUS, HELLP, preeclampsia, fatty liver, DIC, prosthetic valves

268
Q

Spherocytes

A

hereditary spherocytosis, autoimmune hemolytic anemia (warm)

269
Q

Target cells

A

thalassemia, sickle cell, HCV, HEV, etoh

270
Q

Tear drops

A

myeloid metaplasia with myelofibrosis

thalassemia

271
Q

Sideroblasts

A

myelodysplasia
etoh abuse
Vit B6 def
Sideroblastic anemia

272
Q

dRVVT +

A

common pathway defect
if just deficiency in clotting factor then clotting time normalizes with normal plasma mixing study then neg, if still prolonged likely lupus anticoagulant - should normalize with addition of phospholipid to mixture (antiphopholipid/lupus ac should be overcome)

2018 ABIM Awesome Review (14 decks)

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