Hematology 6% Flashcards
Erythrocyte lineage
Stem cell-> proerythroblast->normoblast (nucleus)->reticulocyte (bluish cells)->RBC
Inc’d retic count (BM working properly)
etio: blood loss - GI, vaginal, hemorrhage inc'd destruction Extrinsic - DIC, hemolysis Intrinsic - membrane protein - hereditary spherocytosis Metabolic enzyme - G6PD Globinopathies - SS, Hb
Dec’d Retic count
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
Microcytic (MCV<80)
iron def anemia
anemia of inflamm (MCV 78,79,80)
Thalassemia
Sideroblatic anemia
Macrocytic MCV >100
Vit B12/folate (MCV 110) myelodysplasia etoh liver dz liver dz blood loss hypothyroid inc retic hemolysis, blood loss
Normocytic MCV 80-100
renal failure
hypothyroid (98-103)
anemia of inflamm (81,82,83)
aplastic anemia
Iron study - normal
Iron 50-160, transferrin sat 20-45%, TIC 22-420 Ferritin >20 (station wagon 1/3 full)
Iron def anemia -
now school bus very empty
iron <15%, high TIBC (more seats), low ferritin (low iron=low ferritin)
Anemia of inflamm
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
Hemochormatosis
screening test - transferrin saturation is most specific - high iron high ferritin low TIBC (occupied)
Most sensitive assay for IDA -
ratio soluble transferrin/log ferritin
>2=IDA
<1 anemia of inflammation
Chronic alcoholism
inc’d carbohydrate free transferrin level
Celiac sprue
oily stools, itch skin lesions (dermatitis herpetiformis - tx with dapsone) - TTG+, IDA
R/O IDA
ferritin >100
Paroxysmal nocturnal hematuria PNH
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
Anemia of inflammation (chronic dz)
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)
Pagophagia
eating ice in IDA
Hg 10, MCV 85, TIBC low, TF sat 22% ferritin low 120 - BM shows?
dec sideroblasts and inc iron filled macrophages
70 M or post meno F with Hg9.5, MCV 70, low ferritin - wtd?
GI w/u
Young - EGD
Old - colonoscopy
25yo F Hg 9, MCV 63 Ferritin low RDW 17 - hypochromic microcytosis, target cells, pencil/cigar cells dx?
Menstrual loss or preg with inc need
Tx - PO iron (IV only with renal failure)
When to check retic count during IDA tx
5 days
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?
start iron supp - too low to make cells
Hg 10.5, MCV 80, peripheral smear normal - uniform size cells RDW 14
inc plasma volume
Hg 10.5m MCV 75, hypochormic microcytic cells RDW 17%
IDA
Elderly man p/w n/abd pain diarrhea - slight confusion and gaste distrubance dec biv sense - Hg 9 , MCV78,
check urine for heavy metals (lead)
Sideroblastic anemia
INH witout B6, etoh abuse, batt workers, porcellin workers
Elderly man works at factor making batteries or lives in old buiding or alcoholic p/w anemai Hg 5, MCV 80
Lead tox
Hg A (normal)
2 alpha, 2 beta
Hg A2 (small amts ok)
2 alpha, 2 delta
HgF
2 alpha, 2 gamma - good prognostic factor - hyroxyurea makes Hg F
dec alpha
alpha thal
dec Beta
Beta thal
Point mut of B chains - SCDz
African americans
B thal Trait
very low MCV (<75) mild anemia, inc HgA2, inc RBC, RDW normal
Hemogl electrophoresis
alpha thal NORMAL
+ in beta thal
B thal Intermedia
low MCV, mod anemia lots of Hg A1, non-transf dependent
B thal Major (cooley’s anemai)
Hemolysis, transfusion dep - iron overload, hepatoslenomegaly, target cells, tear drop cells, alpha cahins - ppt - heinz bodies
Young woman or man for reg checkup - Hg 12.2, MCV 70 RDW 13.5, spear with hyochromic cells, target cells +
Hemoglobin electrophoresis -> b thal
Pt from asia p/w Hg 12, MCV 75 Hg electrophoresis NORMAL - blood refused while trying to donate blood - most likely dx
Alpha thal
Sickle cell dz
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
Hg SS -> B1 G->V, B2 G->V
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
Hg SC - B1 G-V B2 G-> Lysine
Hg SC dz - aspetic necrosis femur/hum, retinal infarct, vaso occlusion CAN occur
AA male p.w SC anemia, joint pain, fever, Gh 10 LDH ele, retic elev, normla ferritin dx?
Vasoocculisive crisis - hydration, alanlesics, oxygen - PRBC if Hg still low
Prev - hydroxuria - more HgF
Pt with severe sicle cell anemia recurrent crisis - what can reduce circulating sickle cells
reduce intensity conditioning regiment followed by peripher stem cell allogenic tx from HLA matched donor
don’t use meperidine for analesia - causes seizures
AA SC dz right hip pain - no truama - hip xray diffuse articular sclerosis, decalcif - dx?
Avascular necrosis of femur - MRI confirmation
Sickels cell anemia with stroke
exchange tx
Sicke cell anemia with CP, fever, no PMNs, CXR infiltrates, morphine given
Acute chest - leading cause morality in SC pts - give abx, O2, PRBC - exhange tx if still hypoxic
Etio - microvasc infarction, fat embolism
Pt sickle cell trait LUQ pain - enlarging spleen, dropping hg orthostasis - dx?
Splenic sequestation crissi - IVF, PRBC
Pt with Sickle cell dz, joint pains, rash on trunk Hg 6, plt and WBC normal - retic 0%, ANA +, anticardiopan +, parvovirus +
Parvovirus B12 aplastic crisis - Dx IgM Ab
Tx - IVIG
Daycare worker with arthalgias - Hg 8 no h/o bleeds, no rash, retic count 0.2
Parvovirus B19 (don’t see rash in adults)
Preg teacher pw worriy of Hep A outbreat or parvoivurs B12 in school -
IgM-, IgG_ ok protected
Cause of osteomyelitis in SCdz
Salmonella>staph 2:1 - need gram neg coverage
Bthal
HgA, little HgF and HgA2
B thal major
Hg A (little) HgF (LOTS), HgA2
SS trait
Lot HgA - little HgF, some Hg S
SS dz
no HgA, little HgF, lots HgS, little HgA2
Hg S/C (thal)
little HgA, some HgF, lots HgS, some HgA
Pt Hg 13 MCV 70 - Electrophoresis HgA 75%, HgS 25% Hg F0.3%
SS trait and alpha thal
Macrocytic anemia
> 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)
Folic acid def causes
etoh, methotrexate, trop sprue, pyrimethamine, pynytoin, herdi sphercytosis, chronic hemolysis
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
dilantin induced folate def
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
continue folic aid - known to inc plts
PCP starts 60yo with MCV 120, Gh 10 on folic acid - years later Hg 11.2 - has mild dementia and balancing problems - wtd
check B12
folate normal, B12 300
check MMA if inc’d still has B12 def
74yo F sx of forgetfulness - romberg sign +, B12 310 - gh 13 MCV 103 wtd?
Check MMA
Which medication dec’s absorption of Vit B12
metformin (lactic acidosis)
tx - oral B12
B12 vs B6 def
high MCV = B12 def
With any chronic hemolytic anemia (SCDz, Hered spherocytosis) pt needs to be on?
Folate
HgA1c falsyy low (high cell turnover)
Long time dok worker 80yo pw improper gait and forgetfullness - romberg pos, dec vibration, HCV 118 - B12 275 - MMA elevated dx?
Vit B12 defieicnecy (ele MMA)
Folate deficeincy
only homocysteine high
Vit B12 def
MMA and Homocystein high
Vit B6 def
only homocystiene high
Myelodyplastic syndromes
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
MDS Tx
55 Azathacine+supportive blood tx, GCSF in WBC
EPO500 - antithyoctye globulin (ATG) - if can’t tol ATG then lenaidomide
5q gene del - lenadolmide
Hemochromatosis
Iron o/s with transferrin sat >45%
best screening tool serum transferrin sat
Dx HFE gene testing
Sx hemochrom
pancrease - DM skin - hyperpiz - porphyria cutanea tarda heart - CM LFT cirrhosis, hepatoma, elel lfts pituitary - hypogonadism Joints - arthropathy (MCP/wrist joint)
Ferritin >1000 - want to check iron o/l extent -
liver bx
Tx for hemochromatosis
Lphlebotomy>deferoxamine
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?
Dx: Hemochromatosis
Screenign - transferrin saturation
Aplastic Anemia
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
Pancytopenia
MDS B12 def Fanconi syndrome RTA II Viral hep B,C HIV, hyperslpensim, hairy cell leuk, bactrim copper def
40 yo ecchymosis pancytopenia - c/w
viral hepatitis
Uremia, low Hg, peripheral smear with burr cells
anemia of renal failure - low epo
Tx - epo
Pt with etoh cirrhosis, worsening anemia - elev bilirubin, elev etic - no evid of bleeding, RBC with irr spiculations
Spur cell anemia of liver dz -
Pt unstable angina started on nitrates - or post EGD turns cyanotic, SOB - EKG no schanges - pulse ox 85% PO2 96
Dx Methhemogobinemia
Tx: methylene blue, vit C
Hemolytic anemias
Extracellular def
Acquired intracell
Inherited intracell
Extracell defects
Immune hemolytic anemias
Autoimmune - Warm, cold aggultins, paroxysmal cold hemoglobinuria
trauamtic microangiopathyic hemolytic anemia
Acquired intracellular defets
Paroxysmal noctural hemoglobinuria
CD55/59
Inherited intracellular defets
membrane abnormalities - hereditary spherocytosis
Enzyme abnormalities - G6PD def, hemoglobinopathies
Coombs test
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
PCN/cephalosporin/methydopa
Warm Ab (antiIgG)
SLE - type I RTA
Warm Ab
CLL/lyphoma
Warm and cold
Quinidine
COld anti-IgM
Infxn - mycoplams, amono)
Cold ab
Role of steroids in tx
Warm only
Influenza
Paroxysmal cold hemoglobinuria
43yo on bactrim for UTI - develops anemia - LDH and retic inc’d - myoplasma titer inc - cold agglutin + - cause of anemia
myoplasma induced hemolysis
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?
paroxysmal nocturnal hemolobinuria
Dx: DAF assay/Flow cytometry CD55/59
Tx Allogenic BM tx/eculizumab
Give meningiococcal vaccine 2 weeks prior
Hereditary spherocytosis
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)
Hereditary spherocytosis dx -
osmotic fragility testing
Tx - folic acid - splenectomy
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?
Hereditary spherocytosis - cytoskeletal spectrin membrane defect
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?
Autoimmune hemolytic anemia (warm)
G6PD def -
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
Hemolysis - TTP
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)
Renal - HUS
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
Liver HELLP
Hemolytic anemia
Elevated liver enzymes
Low Plts
3rd trim or post partum
Diffuse - DIC
Involves clotting factors elev PT/PTT
Schisto only
Prosthetic valves - normal
Pregnancy
HELLP, pre-eclampsia, acute fatty liver of preg, TTP
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?
HUS
Premature ejaculation
SSRI
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?
HELLP
32 wk - early delivery
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?
TTP
Hemolysis, anemia, thrombocytopenia, neuro sx, renal failure
Tx: plasmaphoresis (if no machine then plasma exch / infusion)
Pt with plt 50K wit PNA - how to get TTP dx
Peripheral smear - schistocytes
Which condition will you see schistocyte?
DIC, HUS, HELLP, TTP
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?
Major hemolytic rxn - ABO incompatilbiity
usually clerical error
Post transplant blood tx
irradiated, CMV neg blood
Rh incompatibility
delayed rxn - day 6 palor, icterus
IgA def
PRBC with IgA - anaphylaxis
Need WASHED PRBC
Pt gets 2U PRBC during surgery - returns 1 wk later with dark urine and mild icterus - Hg 11 to 9
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
After massive tx - which electrolyte decreased
Calcium
After massive tx - pt has seizure - cause?
Citrate toxicity
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 -
Coombs test
Pt with h/o urticaria and allergies but needs blood tx - wtd?
Washed PRBCs
Pt w/ fever chills every time tx’d, no drop in h/h dx?
febrile rxn from leukocytes -
get leukocyte poor PRBCs - prevents NON-hemolytic rxns
Pt gets PRBC - few minutes later HR inc, BP drops, SOB, RR high swelling lips - temp 99
IgA deficiency - anaphalaxis from IgA in PRBC (never seen IgA b4)
tx: Tx with WASHED PRBC
Pt get plt tx - 2 hours later fever, hypotension, h/a, CP olgiuria bu tno red urine - dx?
bacterial contamination (plt’s stored…)
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?
TRALI - transf assoc acute lung injury
Anti-leukocyte ab from donor
18yo to get solid organ tx - father insists on donating blood -
needs HLA matched WBC in donor blood - prev GVHD
irradiate fathers blood to prevent GVHD
Pt with aplastic anemia gets allogenic BM tx and needs blood
tx irradiated PRBC
Pt s/p BM tx, CMV neg needs blood - wtd?
irradiated, CMV neg blood
25yo F 3 kids with severe menorrhagia low hg given blood tx - 1 week later with diffuse purpura - PT/PTT normal low plts
Post tx purpura
Etio - PLA-1 ab - alloimmunized with prior tx or preg
Tx - IVIG
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?
dilutional thrombocyotpenia
etio - massive prbc tx without plts - give plts next time
45yo acute GIB with orthostatic hypotension - 8 unts PRBC and vigorous hydration then bleeds again several hours later with prolonged PT/PTT etio?
Plasma washout
tx - FFP
55yo scheduled fo relective CABG - consult for AC intraop and post op PPX - had DVT in past was tx’d - HIT neg - best management
IV hep durign surgery + SQ fondaparinex post op
Pt with severe MVA needs massive tx -
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)
Primary hemostasis
Platelets
immediate,
Superficial - petechiae, ecchymossi, purpura,
inc’d bleeding time, NORMAL PT/PTT
Secondary hemostasis
Clotting factors Delayed Joint bleeds, hematomas Normal bleeding time Inc'd PT - extrinsic pathway In'd PTT - intrinsic pathway
Primary Hemostasis - Dysfxn plts
Dysfxn plts Von Willedbrand Bernard Soulier (giant plts) COX inhib Glanzmann's dz MM RF
Primary Hemostasis Dec’d plts
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
Microangiopathic anemia
HIT dec'd by 50% no matter what level it starts at plot 4 hep complex antigenic plts cry for help thormbosis
Plt dysfxn d/o vWF dz
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
Bernard Soulier dz
Giant plts, inc’d BT
COX inhib
from ASA/NSAIDs
Glanzmann’s dz
GP Ib defect (rct for vWF)- abn plt aggregation
inc BT, plt count normal
GP IIb/IIIa inhib
abcimimab/eptifibatide/tirofiban
U/A
post PTCA
Pt with inc BT, inc PTT dec ristocetin facto rassay
vWF dz
Pt withinc BT normal PTT, giant plts
Bernard Soulier
Pt with inc BT, nl PTT, nl Plts, abn plt aggregation
Glanzman's dz Ib defect (rct for vWF)
40yo F recently started on ASA develops heavy menses dx?
vWF dz
Pt with mucous membrane bleeds, protein 9.5, Ca 12.5, BUN/Cr 40/4.5 plt 95 cause of MM bleeds is likely…
MM/renal failure
Pt withi ESRD with MM bleeds - etiology?
plt dyfxn from ESRD
Tx: DDAVP
24yo 3rd trim plt 85
Gestational thrombocytopenia
benign - monito rplts q102wks no steroids no IVIG
ITP
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
Pt with large ecchymotic area 8x12 on thigh, plts 12, PT/PTT normal - smear with low plts and megakarycyte
Bernard soulier -> prednisone
Pt with multiple bruises, plt 15, Pt/PTT nromal wtd?
Prednisone
Preg pt with ITP on steroids - plt 9 - wtd?
IVIG + steroids
Young female dx with ITP this year with menorrhagia Hg 9 multiple bruises on leg - plt 9 - wtd?
IVIG
Pt’s with throbocytopenia need what checked?
HIV/ HCV ab
1 unit plts will inc plts 1 hr later by…?
5K
Pt scheduled for splenectomy - wtd prior
pneumococcal / flu shot - (also before TNF alpha, rituximab
Pt s/p hip replacement on SQ hep for DVT ppx - 4 days later pain in r calf - +DVT dx?
HIT -
Tx - stop heaprin start argatroban or lepirudin (DTI)
Pt with angina on heparin - plts 250, on 4th day plt 100 - no bleeding or thrombus - wtd?
d/c hep and start DTI (argatroban)
Low GFR which DTI ok?
Argatroban - cleared by liver ok in RF
Lepiriduin cleared in kidney
Pt with DVT - on heparin and warfarin - 4th day HIT + wtd?
d/c hep, d/c warfarin start DTI (Lepirudin) start coumadin when Cr normalizes
Pt with UA on hep - 200 plts - 3 days late r145 - wtd?
still ok c/w heparin
Can LMWH be used in HIT
NO - cross reacts with heparin
Hep-coumadin bridge
protein C decreases faster than thrombin so clots - need heparin to bridge a/c until coumadin catches up (knock off thrombin)
Pt with plt 250, next day 5K no sx, no bleed, no drop in Hg, PT/PTT nl, LDH nl what happened?
repeat manula plt count
EDTA in blodo tube clumping - artifact
Pt with DVT start on A/C with hep 2 days later hypotensive - Na124, K5.6 dx?
b/l adrenal hemorrhage 2/2 heparin - hydrocortisone, has
Pt on heparin for DVT ppx - 10 days later serum Cr 1.4 but K 4.5 to 6 wtd?
change to fondaparinux (arixtra)
Secondary hemostasis Intrinsic pathway (PTT)
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
Xa inhibitors - inhibit thrombin formation and thrombi formation
fondoparinux (arixtra) LMWH apixiban, rivaroxiban
Extrinsic pathway (PT - vit K dep)
Tissue factor->VIII->
Direct thrombin inhibitors
Lepiruden, argatroban, bivilirudin (angiomax), Dagitroban (pradaxa)
Russel vipor venum test
mixing study - if clotting times continues to be prolonged then not just clotting factor problem - there is lupus anticoagulant (antiphospholipid ab)
Inc PTT, nl Pt
on hep - yes normal no bleed - factor XII def mild or rare bleed -> XI def mod bleed IX def severe bleed - VII def
inc PTT bleed corrected b 1:1 mix normal plasma
factor deficiency
in PTT bleed NOT corrected with mixing study
inhibitor (aquired)
inc’d PTT no bleed NOT corrected by mixing study and inc’d r/o thrombosis/abortions
lupus A/C syndorme - check dRVVT
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
antiphospolipid Ab (lupus AC) PT INR 2-3
Pt on exam PTT 75s surgery 2 ya no complciations - FXI levels low but mixing study did not correct, dRVVT prolonged dx?
Lupus anti-coagulant syndrome
Inc’d PT, nl PTT
vit K def
F VII def
on coumadin
Inc PT/PTT
common pathway def multiple factor def DIC Liver dz Coumadin brodifacoum (super warfarin posining)
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
factor XIII def
Pt for pre-op - PTT 90 s, PT nromal - PTT corrected with normal plasma
dx F XII, XI, IX, or VIII def
Pt post op in ICU with inc PT INR 2.4 not on coumadin admissioni INR nromal -
F VII def
Which factor def NOT have excessive bleeding
F XII
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
Brodifacoum super warfarin poisoning
tx: vit K for months, monitor PTT
Hemophilia A (fact VIII def
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)
Pt with mild hemophilia A 10% factor VIII - dental extraction wtd?
desmopressin spray +- aminocaproic acid swich (antifibrinolytic agent protects clot
Pt with hemophilia A with head truama
Factor VIII concentrate keep level >50% x 2weeks
Pt with hemophilia A for major surgery wtd
Factor VIII concentrate prior to surgery and up to 72hrs later
Pt with hemophiia dev ab to FVIII has trauma
Factor VIIa concentrates - indirectly inc’s thrombin
Pt with factor IX def - sustains trauma with fracture of femur wtd?
F IX conc or FFP
Prescribe warfarin for pt with afib and high chad score - finds out its rat poson wtd?
continue warfarin, check PT/INR
Pt with afib on coumadin INR 5 no active bleed - wtd
lower dose and hold one dose
INR 5 to 9 no active bleed
hold coumadin - low dose vit K - recheck INR when drops to 3 start coumadin at lower dose
INR>9 wtd (on coumadin)
hold coumadin high dose vit K +-FFP recheck INR when drops to 3 then restart coumadin at lower dose
INR 3 with acute bleed like GIB - wtd?
high dose vit K and FFP
Pt shceduled for surgery and has afib - warfarin stopped to 3 days ago - morning of surger INR 1.6 wtd
clear for surgery
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?
FFP 4-6 hrs prior to procedure
DIC
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
Pt with metastatic lung CA dev sudden fluish toes and finger plits dec, PT/PTT normal/slightly elev, LFT nl, Fibringoen nl
Chronic DIC 2/2 cancer
Tx - if thrombi - heparin
Pt with abruptio placentae with retained dead fetus, dec hg, dec plt, inc PT/PTT wtd?
extraction of fetus
Woman with ecessive bleed after D&C - plt ct 45 d dimer elv - follow pt with ?
D-dimer - good NPV, dec prob PE
Throbotic d/o
Congenital FV leiden (activ ptoein C resistance (>50yo) FaII 20210 defect Protein C/S - 20 to 50yo ATIII Dysfibrinogenemia, inc homocysteine
Thrombotic d/o aquired
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)
35yo M plans travel from tokyo to NY h/o FV leiden defect
recommend - hydration and leg excercises during flight, no A/C
55yo M NY to Hawaii - h/o DVT in past - best management along with hydration and leg excercise
Graduated knee high compression stockings (if >40yo)
Pt h/o FV leiden or protein C def with DVT management?
Coumadin - 6mo (first time)
Pt develops 2nd DVT - wtd?
AC for life (coumadin)
55yo causcasian p/w leg pain -> DVT after flgiht from hong kong to LA most likely cause?
FV leiden or activated protein C resistance
Pt dx with DVT started on coumadin INR 3 - 3 days later wk left side
Protein C def (pro-coagulant period - needed heparin or lovenox bridge)
When to check protein C after DVT/tx
once 3 to 6 mo AC done and coumadin stopped x 2 weeks
Pt with prolongued PTT, does not correct mixing or adding regular plasma - corrects when adding phopholipids - no DVT
monito rif pt has DVT then start heparin and coumadin
Pt history of Breast CA->DVT wtd
long term LMWH (better than coumadin for onc)
25yo preg F with DVT SOB - wtd
LMWH throughout preg and up to 6 weeks after delivery
26yo F preg had DVT after knee surgery 5 ya - tx’ed with ac for 6 months
Only 6 weeks LMWH during post partum period (most thrombotic period) - heparin or warfarin
Woman with prothetic valve on warfarin becomes preg and on 5th week
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
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?
Heparin or LMWH plus ASA antepartum and postpartum
Rev cause of DVT (OCP)
warfarin x 3 months
F V leiden or protein C def with DVT
coumadin x 6 months
Cancer with DVT
extended LMWH
Lupus anticoagular with elev PTT/ +DVT
coumadin indefinetly
Lupus A/C elev PTT NO DVT
observe
4 days after d/c for large DVT in leg/thigh PT/INR 1.1 on 5mg coumadin - wtd?
begin LMWH, inc warfarin to 7.5
Transitioning of LMWH to warfarin
5 days LMWH and warfarin with target INR of 2 for 24 hrs
complication - warfarin skin necrosis
Pt with skin necrosis what does pt have?
Protein C deficiency
tx? FFP - repletes protein C
Myeloproliferative d/o’s
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
Polycythemia Vera
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
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?
Myelofibrosis
see bizarre cells (tear drop cells)
see erythroid precursors (nucleated RBCs)
How to manage pt with PCV then myelofibrosis with sever anemia
Blood transfusion
65yo M dyspnea/headaches - chornic smoker - 35pk years - Hg 23, MCV 78, plt 550, alk phos normal sao2 normal, B12 high - tx?
Phlebotomy + low dose ASA
MC seen with PCV
splenomegaly
Secondary erythrocytosis
Hypoxia - COPD, R->L shunts, high altitute
Neoplasm - retinal tumors, cerebellar tumors
Other - polycystic dz, steroids, androgens
Essendtial Thrombocytosis
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
Reactive thrombocytosis
plt <800, secondary factor usually pressent etio iron def chronic infxn/inflamm neoplasm chornic blood loss splenectomy post megaloblastic anemia tx
20yo F fatigue, palor - menses heavy - Hg 9, plt 800 MCV64 - most likely cause of thrombocytosis?
Reactive thrombocytosis from IDA
25yo F routine physical - no complaints and no meds plts 800 wtd?
Observation….
Pt h/o PCV p/w burning sensation in foot - erythema/warmth, tenderness, gangren of toe
erythromelagia - cuase plts (essential thormbocytosis) or RBCs (PCV)
Tx: ASA
CML - chronic <2% blasts - better formed cells
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
Leukemias
ALL55
Dec LAP in…
CML, PNH, wilson’s dz (neuropsych, tremor)
Myelofibrosis with myeloid metaplasia
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
AML acute myelogenous leukemia
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
M3 AML - acute pro myelocytic leukemia
t15,17 Aur rods - good prognosis -
tx ATRA all trans retinoic acid
AML bad prognosis
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
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?
ATRA differential syndrome
Tx Dexamethasone, d/c ATRA (cytokine leak from differentiating cells)
You would fine myeloperoxidase stain + in which of following
Acute PML (M3 AML), microsopic polyanteritis angiitis
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?
Sweet syndrome (acute febrile neutropenic dermatosis) skin dz with fever, leukocytosis Bm bx - cytogenics study a/w leukemias - mainly AML Tx: Steroids
CLL
> 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
62yo M with URI WBC 18, lymphocytes predominant - smear with mature lymphs and smudge cells dx?
CLL - lymph 80%, sudge cells, PMN 10%
Pt p.w WBC 40, lymph 80% dx?
CLL
Indication for CLL tx
Lymphocytosis alone - observation
Lymphocytosis + anemia Hg<100 - treat
Pt with CLL - Hg <100 management?
Chemo
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?
Hypogammaglobulinemia
Prevention: monthly IVIG
Reason for CLL pt with sudden onset anemia and spherocytes on smear
Warm autoimmunit hemolytic anemia
Tx - steroids
If no response then - immunosupp agents - dexamethasone, cyclophosphamide, rituximab
Hairy cell leukemia
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
Which most likely to got AML
CML
BLister/bite cell
G6PD deficiency
Acanthocyte/spurr cell
etoh anemia, liver dz
Echiniocyte/burr cell
uremia anemia - dec epo
Howell jolly bodies
splenectomy/fxn asplenia
Normoblast
severe hemolysis/myelofibrosis
Polychromatophilia (reticulocytes
hemolysis, blood loss, BM working
Schistocytes
TTP, HUS, HELLP, preeclampsia, fatty liver, DIC, prosthetic valves
Spherocytes
hereditary spherocytosis, autoimmune hemolytic anemia (warm)
Target cells
thalassemia, sickle cell, HCV, HEV, etoh
Tear drops
myeloid metaplasia with myelofibrosis
thalassemia
Sideroblasts
myelodysplasia
etoh abuse
Vit B6 def
Sideroblastic anemia
dRVVT +
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)
