hematolgy 2 Flashcards

1
Q

When to suspect hereditary thrombophilia?

A

age < 45 w/o another risk factor

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2
Q

causes?

A

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

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3
Q

actor V leiden mutasion?

A

MCC

activated resistance to protein C

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4
Q

prothrombin mutation?

A

2nd MCC

Increase prothrombin

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5
Q

antithrombin deficiency?

A

Inherited form rare

acquired: cirrhosis, nephrotic syndrome and DIC

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6
Q

Protein C and S deficiency?

A

decrease inactivation of factor V and VIII

warfarin induced skin necrosis

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7
Q

Triads of hereditary spherocytosis?

A
Jaundice
Splenomegaly
Hemolytic anemia
Hemoglobinuria
Increase MCHC
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8
Q

cancer-related anorexia/cachexia syndrome?

A

anorexia
wt loss
decrease skeletal muscle mass

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9
Q

management?

A
progestrone analog(megestrol acetate)-1st line
corticosteroid:2nd line B/C side effect
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10
Q

what about cannabinoids?

A

Effective for HIV related anorexia

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11
Q

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

A

Age-appropriate cancer screening

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12
Q

Lab finding in antiphospholipid syndrome?

A

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

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13
Q

CLL?

A

tumor due to mature B cell proliferation

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14
Q

CM?

A

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

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15
Q

Diagnosis?

A

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

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16
Q

complication?

A

Infection
autoimmune hemolytic anemia
secondary malignancy

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17
Q

Infection?

A

due to hypogammaglobulinemia and defective cell signaling

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18
Q

secondary malignancy?

A

Richter transformation

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19
Q

Richter transformation

A

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.

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20
Q

Prognosis?

A
Median survival is 10 year
Poor prognosis sign
multiple chain LDP
Hepatosplenomegaly
anemia and thrombocytopnia
21
Q

Cause of microcytic anemia?

A

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

22
Q

IDA and Lead poisoning similar laboratory findings?

A

Low iron and TIBC will be low

23
Q

If iron and TIBC will be normal?

A

Suscepect Thalassemia and Heme structure defect

24
Q

what to do then?

A

electrophoresis

25
Q

Epidemiology of ITP?

A

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

26
Q

Manifestation?

A

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

27
Q

Diagnosis?

A

diagnosis of exclusion

28
Q

What to exclude?

A
Drug
liver disease
hypersplenism
Microangiopathic disorder 
BM disorder
29
Q

the drug should be given with phenytoin?

A

Folic acid
Ca
Vit D

30
Q

management of ITP?

A

depends on the platelet count

31
Q

> 30,000

A

observation

32
Q

< 30,000

A

corticosteroid

33
Q

massive bleeding?

A

Iv Ig and platelet transfusion

34
Q

transfusion and hypocalcemia?

A
blood contain citrate which can chelate Ca and cause hypocalcemia
massive transfusion(> 1 volume) per 24 hour
35
Q

Traumatic macrovascular hemolysis sign?

A
anemia
jaundice
hepatosplenomegaly(work hyperthrophy)
mild thrombocytopenia
schistocytes on pheripherial morphology
36
Q

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

A

It has low sensitivity in time of acute crisis

37
Q

what do we use to diagnose at this time?

A

sign of hemolytic anemia
inciting event
bite cell and Henze body

38
Q

most common origin of PE thrombi?

A

pelvic/femoral vein

39
Q

giving the only folate in case of cobalamin deficiency?

A

Improve anemia but precipitate the neurologic symptoms.

40
Q

Febrile non-hemolytic transfusion rxn?

A

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

41
Q

prevention?

A

Lukoreduction for blood storage

42
Q

Pathogenesis

A

cytokine released by leukocytes in donor blood/

43
Q

other benefits of leukoreduction?

A

Prevent HLA alloimmunization

CMV transmission

44
Q

AML?

A

MC adult leukemia

Median age 65

45
Q

CM?

A
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)
46
Q

Laboratory?

A

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

47
Q

Diagnosis?

A

BM biopsy: Hypercelular with myeloid blast

48
Q

why MM patients are at risk of infection?

A

defective Ab production

49
Q

What will be RDW and RSC count in thalassemia?

A

Normal