hematolgy 2

Question 1

When to suspect hereditary thrombophilia?

Answer 1

age < 45 w/o another risk factor

Question 2

causes?

Answer 2

actor V leiden mutasion
prothrombin mutation
antithrombin deficiency
protein c and s deficiency

Question 3

actor V leiden mutasion?

Answer 3

MCC

activated resistance to protein C

Question 4

prothrombin mutation?

Answer 4

2nd MCC

Increase prothrombin

Question 5

antithrombin deficiency?

Answer 5

Inherited form rare

acquired: cirrhosis, nephrotic syndrome and DIC

Question 6

Protein C and S deficiency?

Answer 6

decrease inactivation of factor V and VIII

warfarin induced skin necrosis

Question 7

Triads of hereditary spherocytosis?

Answer 7

Jaundice
Splenomegaly
Hemolytic anemia
Hemoglobinuria
Increase MCHC

Question 8

cancer-related anorexia/cachexia syndrome?

Answer 8

anorexia
wt loss
decrease skeletal muscle mass

Question 9

management?

Answer 9

progestrone analog(megestrol acetate)-1st line
corticosteroid:2nd line B/C side effect

Question 10

what about cannabinoids?

Answer 10

Effective for HIV related anorexia

Question 11

age >45 with no identified risk factor for TE develop TE first test?

Answer 11

Age-appropriate cancer screening

Question 12

Lab finding in antiphospholipid syndrome?

Answer 12

antiphospholipid antibody
increase PTT not respond to plasma mixing
respond to phospholipid mixing that bind adequately to Ab

Question 13

CLL?

Answer 13

tumor due to mature B cell proliferation

Question 14

CM?

Answer 14

lymphadenopathy(cervical, supraclavicular and axillary)
Hepatosplenomegaly
mild thrombocytopenia and anemia

Question 15

Diagnosis?

Answer 15

Sever lymphocytosis
Smug cell
Flow cytometry
LN and BM biopsy not needed

Question 16

complication?

Answer 16

Infection
autoimmune hemolytic anemia
secondary malignancy

Question 17

Infection?

Answer 17

due to hypogammaglobulinemia and defective cell signaling

Question 18

secondary malignancy?

Answer 18

Richter transformation

Question 19

Richter transformation

Answer 19

also known as Richter’s syndrome (RS),
is a transformation of B cell chronic lymphocytic leukemia (CLL) or hairy cell leukemia into a fast-growing diffuse large B cell lymphoma, a variety of non-Hodgkin lymphoma which is refractory to treatment and carries a bad prognosis.

Question 20

Prognosis?

Answer 20

Median survival is 10 year
Poor prognosis sign
multiple chain LDP
Hepatosplenomegaly
anemia and thrombocytopnia

Question 21

Cause of microcytic anemia?

Answer 21

IDA
Thalassemia
Heme structure defect(HC,S,CS) disease
Lead poisoning

Question 22

IDA and Lead poisoning similar laboratory findings?

Answer 22

Low iron and TIBC will be low

Question 23

If iron and TIBC will be normal?

Answer 23

Suscepect Thalassemia and Heme structure defect

Question 24

what to do then?

Answer 24

electrophoresis

Question 25

Epidemiology of ITP?

Answer 25

commonly acquired auto Ab formation recent viral infection and comorbidity(HCV

Question 26

Manifestation?

Answer 26

Usually asymptomatic Mucocutaneous bleeding ecchymosis,petichia and purpura severe hemorrhage is rare

Question 27

Diagnosis?

Answer 27

diagnosis of exclusion

Question 28

What to exclude?

Answer 28

``` Drug liver disease hypersplenism Microangiopathic disorder BM disorder ```

Question 29

the drug should be given with phenytoin?

Answer 29

Folic acid Ca Vit D

Question 30

management of ITP?

Answer 30

depends on the platelet count

Question 31

>30,000

Answer 31

observation

Question 32

< 30,000

Answer 32

corticosteroid

Question 33

massive bleeding?

Answer 33

Iv Ig and platelet transfusion

Question 34

transfusion and hypocalcemia?

Answer 34

``` blood contain citrate which can chelate Ca and cause hypocalcemia massive transfusion(> 1 volume) per 24 hour ```

Question 35

Traumatic macrovascular hemolysis sign?

Answer 35

``` anemia jaundice hepatosplenomegaly(work hyperthrophy) mild thrombocytopenia schistocytes on pheripherial morphology ```

Question 36

why G6PD should be considered in times of crisis even with a negative G6PD enzyme assay?

Answer 36

It has low sensitivity in time of acute crisis

Question 37

what do we use to diagnose at this time?

Answer 37

sign of hemolytic anemia inciting event bite cell and Henze body

Question 38

most common origin of PE thrombi?

Answer 38

pelvic/femoral vein

Question 39

giving the only folate in case of cobalamin deficiency?

Answer 39

Improve anemia but precipitate the neurologic symptoms.

Question 40

Febrile non-hemolytic transfusion rxn?

Answer 40

fever, chills, and rigor with 1-6 Hr after transfusion Transient respond to antipyretic

Question 41

prevention?

Answer 41

Lukoreduction for blood storage

Question 42

Pathogenesis

Answer 42

cytokine released by leukocytes in donor blood/

Question 43

other benefits of leukoreduction?

Answer 43

Prevent HLA alloimmunization | CMV transmission

Question 44

AML?

Answer 44

MC adult leukemia | Median age 65

Question 45

CM?

Answer 45

``` fatigue(B-Symptom rare) symptom of cytopenia anemia-fatigue and weakness bleeding and bruising from thrombocytopenia infection(granulocytopnia) hepatosplenomegaly and LDP rare DIC(If APL) ```

Question 46

Laboratory?

Answer 46

Cytopenias? maybe leukocytosis Elevated LDH Pheripherial smear: promyelocyte elevated PT and PTT

Question 47

Diagnosis?

Answer 47

BM biopsy: Hypercelular with myeloid blast

Question 48

why MM patients are at risk of infection?

Answer 48

defective Ab production

Question 49

What will be RDW and RSC count in thalassemia?

Answer 49

Normal