Chapter 7 BOMB GM MTB Step 3 Hematology Flashcards
1
Q
1 Anemia (in general) -gen cp -severe Sx
A
“-ALL forms of anemia: *fatigue*/tired/malaise with subj ““loss of energy””.
-severe Sx: SOB, lightheaded, AMS/confusion”
2
Q
Anemia DDx
A
MICH
Methemoglobinemia
Ischemic heart Dz/CAD
CO (carbon monoxide) poisoning
Hypoxia
3
Q
Q describes craving for Pt. to chew on ice or dirt - think?
A
*pica* 2/t *iron def* anemia
4
Q
on CCS, what pe should you pick for anemia w/u? potential positive findings? T/F: no unqiue physical findings in anemia to allow a specific Dx
A
1) gen app: *pallor*
2) CV: *flow murmur* (usu 1-2/4 systolic murmur)
3) chest: *no (+) but just do bc of SOB to help r/o other causes*
4) ext: pallor
5) HEENT: *pale conjunctiva*, possibly jaundice, scleral icterus
T: no finding is sp enough to key in to Dx
5
Q
Anemia -BI Dx test -additional initial tests (10) -most imp Dx test -MA Dx test
A
- BI: *CBC* w/ periph smear
- other initial (10): retic ct., haptoglobin, LDH, total/direct bili, TSH w/ T4, B12/folate lvl, iron studies + UA w/ micro
- most *imp: iron studies*
- MA: *BM biopsy*
6
Q
iron studies values (5)
A
- Fe (iron in blood)
- ferritin (stored iron)
- TIBC (total iron binding capacity aka thirst for iron)
- Fe sat (opposite of TIBC; amt of iron that is saturated w/ Hb)
- RDW (width; measures range of variation of RBC’s)
7
Q
how to categorize anemia
A
from CBC, ck
1) ck Hb/HCt (H/H): F 37-42; M 45-50
*2) MCV* (mean corpuscular volume, or mean cell V): measure of the avg V of a RBC/corpuscle
-tells you *micro, macro or normocytic* (SIZE)
*3) MCHC* (mean corp [Hb]): measure of [Hb] in a given V of pRBC’s
- tells you if problem w/ synth of Hb
- tells you *hypo, hyper or normochromic* (COLOR)
8
Q
i) MICROCYTIC ANEMIA -DDx
A
-*TAILS: T*hallasemia, *A*nemia of Chronic Dz (ACD), *I*ron deficiency, *L*ead poisoning, *S*ideroblastic
9
Q
if H&P says: *blood loss* (hmrg) OR *elevated platelet ct.* - think this type of anemia? -BIMA Dx test? -^expected values? -Tx
A
- *iron def*
- BI Dx: *iron studies*; MA: BM biopsy
- lo Fe, *lo ferritin, hi TIBC*, lo Fe-sat, *hi RDW*
- Tx: PO ferrous sulfate
10
Q
only anemia assoc w/ incr RDW CCS: T/F do BM biopsy
A
*iron def*: since less iron, new cells are prog smaller so RBC width changes over time
F: do not do. just bc its MA doesn’t mean its clinically done.
11
Q
if H&P says: *Hx of RA or ESRD* - think this type of anemia? -problem -BIMA Dx test? -^expected values? -Tx
A
*ACD*
- iron stores are hi but constipated so cant release
- BI Dx: *iron studies*; MA: *none*
- lo Fe, *hi ferritin, lo TIBC*, nl/lo Fe-sat, lo RDW
- correct underlying Dz
12
Q
T/F: any chronic infectious, inflm or CT Dz can lead to ACD
A
T: it can
13
Q
if H&P says: *a-Sx-ic Pt. w/ lo MCV and smear shows target cells* - think this type of anemia? -problem -most common -BIMA Dx test? -^expected values? -Tx
A
*Thalassemia*
- problem with alpha or beta globin chain prod
- common is B-thal minor
- BI Dx: *iron studies*; MA: *Hb electrophoresis*
- iron studies *NL*
- none
14
Q
Hb electrophoresis results
A
if B-thal: *incr HbA2 & HbF*
if alpha: *nl* results (bc *MA* Dx’d by *DNA sequencing* instead)
15
Q
T/F: alpha vs beta thal have a diff MA test
A
T:
*beta*-thal: *Hb electrophoresis* (shows incr HbA2, HbF)
*alpha*-thal: *DNA sequencing* (needs more hardcore test to pick up this less common thal)
16
Q
lead poisoning/exposure gets categorized under this anemia -other possible causes (2)? -problem -BIMA Dx test? -^expected values? -Tx
A
*Sideroblastic Anemia*
- *EtOH* (alcoholic) or *INH* use
- problem: iron build up inside Mt of RBC
- BI Dx: *iron studies*; MA: *Prussian blue stain* (stains + for Fe, helpful bc there’s an excess here)
- iron studies: *hi Fe* (excess)
- if minor: Rx *pyridoxine supp*; major: remove toxin
17
Q
IRON STUDIES RESULTS: match results with possible micro anemia -hi RDW -nl iron studies -hi ferritin, lo TIBC -lo ferritin & Fe, hi TIBC -hi Fe
A
- hi RDW: iron def
- nl: Thal
- hi ferritin: ACD
- lo ferritin/Fe: iron def
- hi Fe: sideroblastic anemia
18
Q
T/F: oral ferrous sulfate can turn stool black when can this be confusing?
A
T: confusing if Pt. on this but there’s a concern for GI bleed (ie blk stools), *don’t* jump to *colonoscopy* right away. instead do guaiac testing (hemoccult) bc it can diff b/w ferrous sulfate and elemental iron. (only Fe in Hb or Mb can make stool guaiac card +)
19
Q
HbH Dz -what is it -structure -gen problem -Cx finding
A
- Hemoglobin H disease (HbH): form of *alpha-thal* in which moderate to severe anemia develops 2/t decr prod of alpha globin chains -> excess beta globin chains then combine with each other to form hemoglobin H
- beta-4 tetrads (one missing)
- 3-gene del
- only *micro anemia w/ hi retic ct.*
20
Q
micro anemia w/ hi retic ct.
A
HbH Dz/a-thal major
21
Q
70 yo F CBC shows decr HCt (32; nl 37-42) with decr MCV 70 (nl 80-100). NBS? then?
A
NBS: ck stool via hemoccult. if (+) colonoscopy, *if (-) STILL DO COLONOSCOPY*: she is > 50 so do it anyway for routine screening. that plus fact she has micro anemia (cont to next Q)
22
Q
unexplained micro anemia in Pt. > 50 most likely 2/t
A
*colon cancer* so ck *colonoscopy*
23
Q
why is sigmoidoscopy usu wrong answer when ck for colon cancer? when is capsule endoscopy done?
A
bc does NOT inspect R colon; miss 40% of cancers
done to eval bleeding when upper & lower endoscopy are nl & source of bleeding is still beleived to be in SI
24
Q
only form of ACD that reliably reponds to EPO is 2/t?
A
ESRD (bc kidney makes EPO)
25
extravascular hemolysis - what is it?
hemolysis that occurs outside vessels (ie \*spleen & liver\*) so you \*CAN'T\* see it on smear
26
ii) MACROCYTIC ANEMIA -cp -DDx -BIMA Dx
- like ALL anemia: fatigue. diff seen with each type
- DDx: if \*megaloblastic, B12 or folate def\*
- BI Dx: \*CBC w/ periph blood smear (hyper-segmented PMN's & oval cells\*); MA: \*B12 or folate lvl\* (will be low)
27
megaloblastic anemia def'n
smear showing hyper-segmented PMN's (avg lobe # on nl WBC = 3.5. \*if \> 4\* or if more than \*5% have \> 5 lobes\*, Pt. has \*MEGALOBLASTIC ANEMIA\* as well as macrocytic anemia (macrocytosis ie big cells ie incr MCV).
28
CCS: what to order if suspect B12/folate def (4)
CBC w/ periph smear (BI)
also (3): bili lvl & LDH (both incr) + retic ct. (decr)
29
T/F: up to 30% of Pt w/ B12 def can have NL B12 lvl.
T: bc trans-cobalamin in acute phase reactant so any stress can cause its elevation leading to false incr of B12. continue to next Q for NBS
30
^NBS (if you suspect B12 def but B12 lvl is nl)
order \*METHYLMALONIC ACID LVL\* (INCR only in B12)
- rmr stockton (B\*12\*) and \*MALONE\*
- also rmr \*homocysteine (HC)\* incr in both B12 and folate so \*not\* helpful
31
NBS to confirm \*etiology\* of B12 def
order \*anti-parietal cell Ab's\* & \*anti-IFx Ab's\*
-both help confirm or r/o \*pernicious anemia\* (allergy to parietal cells so type of AI d/o against part of stomach) as etiology of B12 def.
32
T/F: Schilling's Test is rarely done
T: very old. this method NOT NEC if auto-Ab's present
33
a) vitamin B12 def -cp T/F: neuro problems resolve w/ Tx T/F: least common neuro problem is periph neuropathy
-fatigue + \*Neuro findings\* (usu \*periph neuropathy\* but any Sx can develop that fx PNS or even CNS); also has smooth tongue (\*glossitis\*) & \*diarrhea\*
T: do resolve w/ Tx \*if\* have been present for short period of time
F: periph neuropathy is \*MOST\* common; \*least\* common is \*dementia\*
34
after B12 replacement Tx, order of improvement
FIRST: retic ct. improves
THEN: neuro Sx improve LAST
35
vit B12 def often seen with this Rx
\*METFORMIN\*: blocks B12 absorption so body is effectively deficient
think metformin/DM also has periph neuropathy
36
b) folate def -cp
-fatigue \*w/o \*Neuro findings (rmr vit \*B9\* def is \*BENIGN\*)
37
T/F: both B12 and folate Tx'd w/ replacement
T: both. folate will even correct BLOOD problems of B12 def \*BUT\* worsen neuro Sx.
38
watch for this when Tx-ing B12 def
watch for \*hypoK\* when Tx-ing B12 def
39
iii) HEMOLYTIC ANEMIA -cp -labs (4)
- sudden onset wknss/fatigue
- hi (3): indirect bili, retic ct., LDH; \*lo: haptoglobin\*
40
diff b/w extravascular & \*intravascular\* lab abnormalities
INTRA has all the same ab-nl of EXTRA but ALSO shows following (3):
- ab-nl \*smear\* bc intravascular (schistocytes, helmet cells, fragmented cells)
- Hb-uria
- hemosiderinuria (metabolic, oxidized prod of Hb in urine)
41
for CCS, in suspected case of EV hemolysis order these (5)
BI: CBC w/ periph smear
also (4): LDH, bili, retic ct., haptoglobin lvl
42
if you suspect hemolytic anemia, first thing you want to improve
\*1st improve retic ct.\*, then try to eliminate hyper-segmented PMN's
43
iii) SICKLE CELL ANEMIA \*(SCA) IMP\* -cp
-severe pain in: chest, back, thighs
44
must do this (A) in sickle cell Pt., esp if this (B) happens
- must do phys exam
- if Pt. has fever AFTER you give O2, fluids, analgesics & Abx
45
possible pe findings in SCA
- HEENT: \*retinal infarct\*
- CV: flow murmur 2/t anemia
- resp/chest: \*rales\*/crackles (velcro, crumpled paper sound) 2/t infxn (PNA consolidation?) or \*infarct\*
- ab/GI: \*splenomegaly\* in kids; \*asplenia\* in adults
- ext: skin ulcers, \*aseptic necrosis of hip\*
- neuro: \*stroke\* (current of prev)
46
"when to do ""exchange transfusion"" in SCA Pt. (4 indications)?"
ck results of pe (prev Q) to see evidence of sickle cell \*crisis\* aka vaso-occlusive crisis
1) eye: \*visual Sx\* 2/t retinal infarct
2) resp/chest: \*pleuritic pain & ab-nl CXR\* 2/t pulm infarct
3) penis: \*priapism\* 2/t prostatic venous plexus infarct
4) neuro: \*STROKE\*
47
SCA Tx -NBS/1LTx if suspected (3) -game changer - NBS?
- 1LTx (3): O2, NS/IVF, analgesia
- if \*fever\*: give 1/3 \*Abx (ceftriaxone, levo or moxi)
48
why is fever in SCA Pt. emergency? in case of: most urgent NBS? CCS: order what next (6)?
bc Pt. has \*NO SPLEEN\*
urgent NBS: give \*Abx\* (MORE IMP than waiting for results of testing)
- CCS Dx (5): BCx, UA, retic ct., CBC, CXR
- CCS Tx (1): 3 Abx above (ceftriaxone, levo or moxi - rmr \*give BEFORE doing Dx tests\*)
49
F w/ hemoglobinopathy (eg \*SCA crisis\*) has sudden drop in HCt (34 to 26) + incr in retic ct. (2%) over 2d in hosp -Dx? -MA test? -Tx?
- Dx: \*parvovirus B19\* (infxn that invades BM -\> stops prod of RBC's @ lvl of pronormoblast) OR \*folate def\*
- MA for parvo: \*PCR for DNA of parvovirus\*
- Tx: \*transfusion + IV Ig\*
50
SCA Pt. should be universally placed on this & why
\*folate\*: helps produce RBC's in BM; folic acid levels decr in SCA Pt. bc RBC's die too quickly.
-also folate def presents ~ SCA crisis (crisis Sx (ie pain) + lower HCt + incr retic ct.) so if Pt. on replacement Tx of B9, then Dx shifts to parvo B19
51
when SCA Pt. ready for d/c, Rx: \*IMP\* (3)
1) folate replacement
2) pneumococcal vax
3) hydroxyurea (HU): prevents further crises (if crisis occurs \> 4x/yr)
52
milder version of SCA/sicker cell Dz? -sim/diff? -most common cp? -most common comp? -Tx
\*Hb Sickle Cell Dz\*
- fewer crises (no painful crises occur at all) \*but\* visual disturbance just as freq
- common: \*visual Sx\*
- comp: \*renal Sx\* (hematuria, isothenuria (inability to concentrate/dilute urine), UTI)
- Tx: NONE
-don't confuse w/ sickle cell \*TRAIT\* which ONLY has renal Sx (hematuria + concentrating defect) - no visual Sx
53
#2 Hemolysis (in gen)
1 ANEMIA
2 HEMOLYTIC SYNDROMES
54
a) hemolytic anemia (iv and extravascular)
Q #39-42
55
a) AI Hemolysis -Pt Hx will also have (3) -cp -labs
- Hx will include: 1) other \*AI\* Dz (SLE or RA); 2) Hx of \*CLL\* or lymphoma; 3) use of \*Rx\*: PCN, alpha-methyldopa, quinine or \*sulfa\*
- cp (same as hemolytic anemia #39): sudden onset wknss/fatigue
- labs (same as hemolytic anemia #39): \*hi\* (3): indirect bili, retic ct., LDH \*incr in ALL\* forms of hemolysis; \*lo: haptoglobin\*
56
T/F: haptogLObin can be LO in BOTH IV & EV hemolysis
T: both
57
AI Hemolysis -BIMA Dx -BIME Tx
- BI: CBC & \*periph smear w/ poss spherocytes\* (schisotocytes in IV hemolysis only); MA: \*Coombs test\*
- BI: CS (\*prednisone\*); Most Effective: (if \*recurrent: splenectomy\*)
58
Ab's found in Coombs aka? ex? T/F: only IgG Ab;s respond to Tx for AI hemolysis
"\*""WARM""\* Ab's which are only \*IgG\*; ex: idiopathic, SLE
T: \*only IgG\* responds to CS & splenectomy"
59
CCS: case of severe hemolysis (non-responsive to CS or repeated blood transfusions) - NBS to \*stop acute episodes\* of hemolysis?
use \*IV Ig\*
60
b) Cold-induced Hemolysis (CIH) -aka -Hx (2) -expected test results (2) -Cx cp -Tx
- aka Cold Agglutinins
- Hx of \*mycoplasma or EBV\* infxn
- Coombs (-) but C' (+)
- cp: \*NO\* response to CS, splenectomy or IV Ig
- Tx: rituximab
61
T/F: CIH is Dx of exclusion
T: we r/o warm 1st to settle Dx on CIH
62
c) G6PD-def -cp -gen -cause
- M w/ sudden onset of severe hemolysis
- X-linked d/o (rmr more in M)
- ox stresses: \*infxn, Rx\* (sulfa, primaquine, dapsone), \*fava beans\*
63
G6PD-def BIMA Dx
- BI Dx (2): \*Heinz body\* test: collections of oxidized precipitated Hb in RBC membrane; & \*bite cells\*: pieces of RBC membrane removed by spleen
- MA: \*G6PD lvl\* - not BI bc incr only after \*2 mos.\*
64
T/F: nl lvl of G6PD def after an episode of hemolysis r/o G6PD def
F: it DOES NOT exclude G6PD-def bc on the day of the hemolysis, the most deficient cells have already been destroyed so lvl of G6PD is nl
65
G6PD-def Tx
no specific Tx just \*avoid ox stress\*
66
d) Pyruvate Kinase def -cp -cause
- \*same as G6PD-def\* (in terms of hemolysis) \*except diff causes\*:
- not provoked by Rx or fava beans - cause here is unclear
67
e) Hereditary Spherocytosis -cp -Cx Dx -MA Dx test -Tx
- cp (3): \*recurrent\* episodes of hemolysis, splenomegaly, bili gallstones
- Dx: \*incr MCHC\* (mean corp Hb conc)
- MA Dx: \*osmotic fragility test\*
- Tx: \*splenectomy\* (prevents future hemolysis since cells are normally destroyed in spleen).
68
f) \*HUS & TTP\* (Hemolytic Uremic Syndrome & Thrombotic Thrombocytopenic Purpura) -Hx for each -cp of each
HUS
- Hx: \*E. coli\* 0157:H7 infxn
- Sx: \*triad = ART\* (\*A\*I hemolysis shows IV hemolysis w/ abnormal smear; \*R\*enal failure; \*T\*hrombocytopenia)
TTP
- Hx: Rx use (\*ticlopidine\* = antiplatelet, ADP-R inh ~ clopidogrel/Plavix)
- Sx: \*pentad = FAT RN\* (ART + \*F\*ever + \*N\*euro Sx)
69
HUS/TTP Tx
most cases \*self-resolving\*. if \*severe = plasmapheresis\*
70
T/F: no specific Dx test for either HUS or TTP. T/F: never use plt in HUS or TTP T/F: do no use CS for HUS/TTP Tx T/F: do no use Abx for HUS Tx
T: Dx based on clinical Sx. check triad in prev Q's to Dx.
T: don't use. platelet transfusion def make it worse.
T: don't use. steroids do not help.
T: Abx for HUS make it worse
71
g) \*PNH\* (paroxysmal nocturnal hemoglobinuria) -cp -#1 cause of death -2 common sequelae -BIMA Dx tests (2)
- cp: pancytopenia, recurrent episodes of \*dark urine\* esp in \*morning\*
- large vessel venous clot (ex: \*portal v. thrombosis\*)
- (2): \*aplastic anemia & AML\*
- BI: old screen tests: sugar water/Ham's test (activate C' to see if it leads to hemolysis); \*MA: CD 55/59 Ab\* (aka Decay Accelerating Fx)
72
PNH Tx: short-term vs long-term
BI Tx: CS (prednisone)
if severe (ie transfusion-dependent), long-term Tx = \*eclizumab\*: inh C-5 -\> prevents C' activation
73
preg woman, 35th wk, cp: wknss labs: incr LFT's, PT nl, smear: fragmented RBC's, plt low -Dx? -Tx
- Dx: \*HELLP\* syndrome: variant or complication of pre-eclampsia; \*H\*emolysis, \*E\*levated \*L\*FT's, \*L\*ow \*P\*LT
- Tx: \*deliver baby\*!!!
74
HELLP vs DIC - main diff?
HELLP has normal coag studies: \*ab-nl PT/PTT in DIC\*
platelets low in both
75
h) Met-Hb (Methemoglobinemia) -cp -DDx -pathophys -main cause -Tx
- cp: \*SOB for no clear reason\* w/ \*clear lungs\* on pe/CXR. will see some \*brown blood\* at some pt in Hx
- r/o PE
- pp: blood/Hb is locked in an oxidized state (ferric Fe3+) so it cannot pick up O2
- Rx (\*nitrates\*: NG, amyl nitrate, nitoprusside; \*dapsone\*, anesthetic drugs ending in \*-caine\* (lido, bupiva, tetra))
- Tx: \*meth\*ylene blue
76
T/F: Methemoglobinemia can occur with as little exposure as topical anesthetic admin to mucus membrane
T: it can. believe it. plz blv betch
77
#3 Transfusion Rxn's: organize by time
"1) \*IgA def\*: as soon as Pt. receives transfusion ie \*ASAP\*
2) \*ABO\* incompatibility: sometimes \*during\* transfusion
3) \*TRALI\* (transfusion-related acute lung injury) aka ""leuko-agglutination rxn"": \*20 mins after\*
4) \*febrile non-hemolytic rxn:\* few \*hrs\* after
5) \*minor/Rh incompatibility\*: few \*days\* after"
78
give rxn for each (5) transfusion rxn
"1) \*IgA def: anaphylaxis\* ASAP (htn, SOB, tachy; LFT's nl)
2) \*ABO\* incompatibility: \*acute hemolytic Sx\*: htn, tachy w/ back pn/cp, dark urine, haptoglobin lo, LDH/bili hi
3) \*TRALI\* (transfusion-related acute lung injury) aka ""leuko-agglutination rxn"": SOB, CXR: transient infiltrates
4) \*febrile non-hemolytic rxn\*: fever w/o hemolysis Sx
5) \*minor/Rh incompatibility\*: Pt. w/ delayed jaundice"
79
Tx for each transfusion rxn
"1) \*IgA def: washed RBC's\* or blood donation from IgA def donor
2) \*ABO\* incompatibility: stop transfusion; look next Q
3) \*TRALI\* (transfusion-related acute lung injury) aka ""leuko-agglutination rxn"": no Tx, resolves spont
4) \*febrile non-hemolytic rxn: filter\*: APAP 4 fever, \*leukoreduction\* in future (\*filters WBC's\* from transfusion)
5) \*minor/Rh incompatibility\*: none"
80
ABO-incompatibility induced Acute hemolytic transfusion reaction - Tx?
1) stop transfusion
2) Px measures: decr ARF risk via low-dose DA (incr RBF), IVF w/ crystalloid solutions & osmotic diuresis w/ mannitol
3) DIC Tx (next Q's)
81
DIC basic problem
Disseminated: Spread widely, scattered; so DIC = scattered blood clots w/in the vessels
body stressed --\> \*clotting becomes overactive\* --\> coag cascade haywire -\> forms clots in un-needed places
irony: body uses so many plt/clot Fx to form \*unnec clots\*, there's not enough leftover to prevent bleeding elsewhere in body so Pt. has 1) blood clots that block blood flow but also 2) severe bleeding elsewhere (can be internal or ext like purpura/petechiae)
82
7 main causes of DIC
trauma/surg
preg
sepsis
cancer
liver Dz/pancreatitis
organ transplant rjxn
incompatible blood transfusion
all conditions have systemic involvement which is why clotting is scattered thruout body
83
T/F: DIC is a lab-based Dx?
NO! Cx Hx imp bc severe liver disease can essentially have the same laboratory findings as DIC. so need a consistent history of an illness known to cause DIC
84
Dx DIC - labs
Dx: constellation of laboratory markers = (5)
1) \*prolonged PT/PTT\*: consumption and impaired synthesis of coagulation cascade.
2) \*low fibinrogen\*: implies consumptive process (ie systemic activation of clotting system) but rmr Fibrinogen is an acute phase reactant so can be nl/incr d/t underlying inflm condition; fibrinogen + thrombin -\> fibrin = jizzs
3) \*low plt ct\*: rmr disseminated intra-vasc \*coagulation\* so plt being used up, just like fibrinogen
4) \*incr D-d\*: D-d is fibrin degradation product which is found 2/t intense fibrinolytic activity (fibrin = jizzs on clot)
5) \*peripheral blood smear w/ fragmented RBC's\*: aka schistocytes; 2/t shear stress from thrombi; not sn/sp 4 DIC
85
how to manage DIC
transfusions of (3) FFP, pooled cryoprecipitates for fibrinogen, and/or platelet concentrates
86
washed RBC's is Tx - think \*F\*ilter transfusion in \*F\*uture Tx - think
IgA def
\*F\*ebrile: aka leukoreduction
87
as soon as Pt. receives transfusion - Sx - think? sometimes during transfusion - Sx - think? few hours after - think few days after - think
immediate: IgA def (Anaphylaxis)
ABO incompatibility
hours: febrile
days: minor blood group (Rh) incompatibility
88
#4 LEUKEMIAs
i) myelodysplasia/MDS (pre-leukemia)
ii) acute leukemia (ALL & AML/APL; myelo/lympho-cytic)
iii) myeloproliferative d/o = chronic leukemia's (CML/CLL), hairy cell, myelofibrosis, PCV, ET (ess. thrombocythemia)
iv) plasma cell d/o: MM, MGUS, Waldenstrom's
v) Aplastic Anemia
89
lymphoid vs myeloid cells
L: NK cells, T-lymphocytes, B-lymphocytes/plasma cells/Ab
M: MKC/plt, RBC, mast cell, myeloblasts (BEN + monocyte/Mø)
-if it's not a lymphocyte, think myeloid cells
90
1) myelodysplasia (MDS) -best way to think of this -cp -labs (4) -
- mild, slowly prog \*pre-leukemia\* syndrome (~ how cervical dysplasia CAN prog to cervical cancer)
- \*old\* Pt. w/:
1) \*pancytopenia\*
2) \*hi MCV\* (ie \*macro-ovalocytes\* bc marrow making abnormal cells as engine sputters)
3) \*lo retic ct\* (retic = immature RBC's which are being decr)
4) \*nl B 12 lvl\* (imp bc you have macro-ovalocytes)
91
how to diff MDS vs macrocytic anemia 2/t B12/B9 def
rmr both have macro-ovalocytes but:
1) B12/9 def: \*Megaloblastic anemia\*
- dysfxnal RBC's (megaloblasts) in BM + \*hypersegmented PMN's (5+\* nuclear lobes/segments; 4 = nl) + ab-nl B12/9 lvl
2) MDS: \*nl B12\* + special PMN (\*Pelger-Huet\* cell w/ only \*2 lobes\*)
92
how to diff MDS from acute leukemia
MDS has a \*small # of blasts\* - not enough to be considered AML/ALL
93
most common cause of death in MDS? MDS Tx?
NOT leukemia - \*infxn\* or \*bleeding\* out
Tx: supp + \*transfusions PRN + Azacitadine\* (specific Tx)
-if 5q minus syndrome, Tx w/ lenalidomide (decr transfusion dependence in MDS)
94
2) Acute Leukemia -cp -BI Dx test
- pancytopenia: \*fatigue\* (lo RBC), \*bleeding\* (lo plt) & \*infxns\* (lo WBC) so Pt. w/ \*fxnal immunodef\*
- periph \*smear\*: showing \*blasts\* (hi #; blasts bc acute)
95
BI Tx for AML (2) adjunct for APL (ie AML M3) adjunct for ALL
chemo: \*idarubicin\* (or \*daunorubicin\*) + \*cytosine arabinoside\* (\*ndn & arab\* - ron/kam - go after \*amal\*)
APL: 2 above + \*ATRA\* (all-trans retinoic acid)
ALL: 2 above + \*intra-thecal MTX\* (MTX when ALL else fails)
96
most imp prog Fx in acute leukemia is? tells us what? if Pt. @ risk for relapse after chemo, NBS
cytogenic/karyotypic ab-nl: the more the cell is dev from baseline, more (P) for relapse
relapse risk: \*BM transplant\* as soon as chemo induces remisson
97
Auer rods, think
\*AML\* M2/M3
-clumps of azurophilic granules that form elongated needles seen in the cytoplasm of leukemic blasts.
98
AML M3 aka? -Dz assoc? \*KNOW\*
\*APL\*: acute promyelocytic leukemia
-\*DIC\*
99
cp: sob, AMS, blurry vision. WBC \> 100 K -Dx? -why these Sx -BI Tx?
- Dx: \*AML\* (lymphocytes are much smaller and do not occlude vessels + we know it's acute if smear: hi blasts)
- very hi WBC (ie \> 100K) -\> sluding of blood cells in vessels of brain, eyes & lungs -\> Sx on cp
- BI Tx (for \*leukostasis\*): \*leukapheresis\* (removes WBC via blood centrifugation) + \*hydroxyurea\* (lowers WBC ct)
100
3) Myeloproliferative d/o: a) \*CML\* (chronic myelogenous leukemia) -Cx labs -cp -BIMA Dx
-lab: hi WBC (\*predominantly PMN\*, rmr neutrophil = myeloid cell)
-\*splenomegaly\*
-BI Dx: lo \*LAP score\* (leukocute alkaline phosphatase)
MA Dx: \*Phl Chr\* (9;22)
101
using LAP score
hi PMN w/ \*lo LAP = CML\*
hi PMN w/ hi LAP = reactive, 2/t infxn
102
CML BIME (most effective) Tx
BI Tx: \*imatinib\* (gleevec) (MOA = tk-inh)
-backups: dastinib, nilotinib (also tyrosine kinase inh)
ME Tx: \*BM transplant\* (most curative, but not BI bc imatnib leads to 90% hematologic remission w/o A/E)
103
WRONG ANSWERS 4 CML Tx
1) interferon: less efficacious, A/E = flu-like Sx
2) hydroxyurea: never nullifies Phl Chr
3) busulfan: \*never right\* for anything unless Q asks what causes \*pulm fibrosis\*
104
un-Tx'd CML has highest risk of this when considering all forms of myeloproliferative d/o
\*transformation\* to acute leukemia
105
3) Myeloproliferative d/o: b) \*CLL\* (chronic lymphocytic leukemia) -cp -BI Dx -Tx: depends on?
"-cp: \*\> 50\* yo w/ \*incr WBC found on ""routine"" test\*. describes as \*""nl appearing lymphocytes""\*. otherwise a-Sx.
- BI Dx: periph blood smear shows \*""smudge""\* cells (~ squished jelly donut = ruptured nuclei of lymphocytes)
- Tx: depends on stage"
106
stages of CLL (5)
stage 0: hi WBC alone (only lymphocytes)
1: enlarged LN's (still lymphocytes)
2: splenomegaly (now myeloid cells - RBC)
3: anemia
4: low plt (highest stage bc now transcended lymphocytes & now involving myelogenous cells so transformed to CML)
107
T/F: CLL Tx is \*entirely\* based on stage of Dz T/F: do NOT Tx a-Sx-ic elevations in WBC in CLL
T: early stage (0/1) \*no Tx\* req'd
-2+ stage: Tx w/ fludarabine + rituximab
T: do NOT Tx
108
CLL Tx: which Tx most likely to extend survival in advanced stage Dz (2-4)? adjunct added to ^? CLL Tx: alemtuzumab - better than what? MOA?
\*fludarabine\*
add \*rituximab\*: adds significant benefit
alemtuzumab better than chlorambucil. MOA: anti-CD52
109
c) Hairy Cell Leukemia -cp -MA Dx -BI Tx
"-cp: 50 y/o w/ pancytopenia & \*massive splenomegaly\*
- MA Dx: \*incr TRAP lvl\* (tartrate resistant acid phosphatase) + smear showing \*""hairy cells""\*
- BI Tx: \*cladribine\* (2-CDA)"
110
d) Myelofibrosis -cp -MA Dx: -BIME Tx
- cp: same as hairy cell (50 y/o w/ pancytopenia & splenomegaly) \*but nl TRAP\*
- MA Dx: \*smear\* showing \*teardrop\*-shaped cells
- BI Tx: lenalidomide or thalidomide (\*if transplant not possible\*)
- ME Tx: BM transplant
111
e) PCV (polycythemia vera) -cp -
-cp: HA, \*blurred vision\*, dizziness, fatigue, \*pruritus in hot shower\* (2/t histamine release from basophils), splenomegaly
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