Heme

Question 1

effect of PTT in lupus anticoag

Answer 1

prolonged

Question 2

first dx step towards SVC syndrome?

Answer 2

CXR

-most commonly caused by malignancy

Question 3

trousseaus sign

Answer 3

migratory thrombophblebitis most highly assoc with pancreatic cancer but can be others as well

Question 4

CML vs leukomoid reaction

Answer 4

CML has a low LAP and more myelocytes

• CML has absolutely basophilia
• CML has extreme leukocytosis*****
• philadelphia csome

polycycthemia vera and leukomoid reaction both show elevated LAP score

Question 5

features of CLL

Answer 5

• 70
• fatigue, splenomegaly
• mature lymphocytsis and smudge cells on smear
• if found incidentally and asx, don’t treat –> very slow growing
• thrombocytopenia –> late finding, poor px because shows impaired BM fx

Question 6

tumor lysis syndrome labs, tx

Answer 6

• INC K, Phos
• dec Ca
• AKI

first line is IV fluids, then allopurinol

Question 7

physical exam signs of hemolysis

Answer 7

splenomegally
jaundice
scleral icterus

Question 8

AML

Answer 8

age ~60

• can have variable white count
• inc myeloblasts with pancytopenia
• myeloperoxidase +
• auer rods
• 15;17
• all trans retinoic acid

Question 9

Young guy with no clot risk develops DVT. You give high dose heparin for DVT in patient with prolonged aPTT but nothing changes, including aPTT. What’s happening?

Answer 9

ATIII deficiency

• AD
• heparin requires ATIII to work –> then deactivates II, X

Question 10

first diagnostic steps in malaria?

Answer 10

thick smear –> followed by thin smear

Question 11

exercise hemoglobinuria is what type of hemolysis?

Answer 11

intravascular

***of note, haptoglobin is usu normal in extravascular hemolysis

Question 12

Kehr’s sign

Answer 12

referred left shoulder pain from splenic rupture

Question 13

philadelphia csome

Answer 13

9;22
tx with imatinib (tyrosine kinase inhibitor)

> 90% in CML but can be in ALL with kiddos so watch out

Question 14

acute trauma coagulopathy

Answer 14

admin pRBC, FFP and plt in 1:1:1

Question 15

dactylitis

Answer 15

swelling of hand or feet assoc with vasoocclussive crisis

also seen in RA, sarcoid

Question 16

p falciparum proph

Answer 16

atovoquone-proguanil

Falcip is resistant to chloraquine in endemic areas

***ring inclusions in RBC

Question 17

Coombs results

Answer 17

positive= HS!

Negative= AIHA

**they both have spherocytes on smear

Question 18

bilateral patchy infiltrates on CXR, dry cough, hemolysis labs? dx? test?

Answer 18

cold, IgM, AIHA

positive direct coombs

Question 19

isolated fever within hour or two of recieving blood products

Answer 19

normal labs = non hemolytic transfusion rxn, can give acetaminophen

next step–> check coombs

Question 20

parvovirus B19

Answer 20

pancytopenia

hypocellular, fatty marrow on biopsy

Question 21

thrombocytopenia in cirhossis most likely caused by?

Answer 21

hypersplenism

spleen gets large due to inc portal pressures –> sequestrs platelets

Question 22

tx for refractory ITP that does not respond to IVIG/ steroids

Answer 22

splenectomy

Question 23

PTT is what pathway

Answer 23

intrinsic

*affected by heparin

Question 24

what is THE BEST TEST EVER for hereditary spherocytosis

• also, what should you give them as proph?
• what pathology are that at risk for at a younger age then usual?

Answer 24

ema (eosin-5-m…) binding test, it uses flow

characteristics of HS: inc RDW, inc MCHC, hemolysis signs

• proph with folic acid to prevent megaloblastic anemia
• gallstones

Question 25

safe malaria proph in pregnancy

Answer 25

mefloquin | atovaquone- proguanil

Question 26

paroxysmal nocturnal hemoglobinuria

Answer 26

genect defect in anchoring protein at risk form thrombosis in atyplica locations test= CD55/CD59

Question 27

polycythemia vera

Answer 27

hyperviscosity sx - can cause essetnail thrombocytosis - can cause 2nd gout tx= phlebotomy

Question 28

characteristics of hodgkins (don't forget the paraneoplastic syndrome)

Answer 28

- reed sternberg cells= polynuclear giant cell, CD15/30+ - EBV - alcohol induced pain in lymph nodes - painless cervical lymphadenopathy - elevated ESR is assoc with poor px - paraneoplastic syn: inc Ca++ via ectopic 1a-OH production tx with chemo

Question 29

malarial endemic regions

Answer 29

asia, africa, south america signs can look like hemolysis so look at history

Question 30

diamond blackfin anemia

Answer 30

- dx at <1 year --> definitive is bone marrow bx - macrocytic anemia (lack other cytopenias) - triphalangeal thumbs

Question 31

tx of CLL

Answer 31

if asyx --> observe and monitor progression if sx --> rituximab (anti CD20)

Question 32

PE signs assoc with Fe deficiency

Answer 32

koilonychia- SCOOPED NAILs | angular chelitis

Question 33

tx of ALL

Answer 33

(ab= CD10, CD19, tdt) - aggressive chemo with vincristine, imatinib if 9;22 - CNS proph with intrathecal chemo

Question 34

differentiate megaloblastic from simply macrocytic

Answer 34

megaloblastic = hypersegmented neutrophils -can only be caused by b12/ folate defi

Question 35

antibiotics in sickle cell crisis/ sepsis?

Answer 35

IV ceft --> improved mortality think of coverage for staph, salmonelloa, e coli, s pneumo (functional asplemia)

Question 36

presentatin seems consistent with G6pd and HS. How do you differentiate?

Answer 36

G6PD is more acute, 2-3 days after oxidative stress insult, like abx HS happens a few weeks later and has ** inc MCHC**

Question 37

hair cell luekemia

Answer 37

common in older men with similar presentation to CLL - massive splenomegaly - TRAP+ (tart tresistant acid phos), CD11c - dry BM tap - tx= cladribine

Question 38

vWD labs and tx

Answer 38

inc PTT (dec factor VIII) **hemophilia will also have long ptt inc bleeding time nl plt tx with desmopressin (VIII is part of extrinsic pathway, requires vwb)

Question 39

more rare side effect of TMP-SMX that you don't think of as often?

Answer 39

agranulocytosis

Question 40

lab disturbances that can be seq of gestational dm in newborns?

Answer 40

hypoglycemia hypocalcemia polycythemia

Question 41

ringed sideroblasts, microcytic vs macrocytic anemia

Answer 41

microcytic= lead poisoning macrocytic= myelodysplastic syn

Question 42

warf vs DOAC in acute DVT?

Answer 42

DOAC acts instantly

Question 43

warf induced skin necrosis

Answer 43

due to relative Prot C deficiency which is the first factor to decrease

Question 44

heme effect of isoniazid

Answer 44

acq sideroblastic anemia that ,masquerades as fe def. - inhibits pyridoxine req for protoporphyrin synth - dimorphic rbcs - INC fe, DEC tibc

Question 45

lab values in vaso-occlusive crisis

Answer 45

aka splenic sequestration - reticulocytosis - thrombocytopenia - normocytic anemia