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Internal Medicine- Areej > Hematology > Flashcards

Hematology Flashcards

1
Q

18 Y/O Boy with

abdominal pain vomiting and fever .. + splenomegaly and mild jaundice , US revealed
pigmented gallstones, negative comb test dx ?
How to confirm

A

PNH
Patoxysmal = episodc jaundice
Nucturial hemoglubinuria = Episodes of hemoglobinuria causing pink/red/dark urine which usually occurs in the morning due to the concentration of urine overnight.
Confirm dx by flow cytometry

Ttt: wait and watch

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2
Q

What is the indication of flow cytometry in preipheral blood smear

A
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3
Q

In any patient CBC if you found sphereocyte , next step???

A

Comb test

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4
Q

Challenge time!!
Type of inheritance
1- hereditary spherocytosis ?
2- Sickle cell
3- G6PD
4- hereditary spherocytosis + hereditary elliptocytosis

A

1-Autosomal dominant
2- autosomal recessive in Chromosome 11 beta-chain in 6th position glutamate is replaced by valine
3- x-linked
4- autosomal dominant

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5
Q

Child with anemia + spleenomegaly + pigmented gallstone + negative combs
How to confirm
And how to treat
CBC shown

A

Eosin-5-maleimide binding test (EMA binding test)
Hereditary spherocystosis

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6
Q

Indication of exchange transfusion in SCD

A
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7
Q

Young SCD with decrease in HB (>2g ) with thrombocytopenia and reticulocytosis, dx and mx

A

Splenic sequestration
Mx:
In case of life-threatening low HB (< 6.5 ) intial management is RBC transfusion
Not : give 1RBC pack with 1 unit of fluid to avoid hyperviscosity syndrome

If not life-threatening intial management is fluid then transfuse

Goal of Hb: transfuse untill it reach 10 only , never over correct

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8
Q

What is the lower accepted Hb level in SCD patient going to surgery

A

10 Hb

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9
Q

Indication of urgent blood transfusion in SCD ?

A

transfuse if the hemoglobin is at least 2 g/dL
below their baseline, with acute clinical symptoms, signs of hemodynamic compromise, or
increased respiratory effort or oxygen requirement to keep the oxygen saturation above 92
percent.

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10
Q

Most common mutation in primary polychythemia rupra vera ?

A

JAK2

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11
Q

Ttt of polychythemia rupra vera
1 procedure ?
2 drugs?

A
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12
Q

Approach to isolated thrombocytopenia?

A
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13
Q

Anticoagulant in patient with HIT (heparin induced thrombocytopenia)

A

argatroban , danaparoid
bivalirudin, fondaparinux) and direct oral anticoagulants (DOACs) .

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14
Q

RDW +Menztor index is high in iron deficiency anemia or thalassemia ?

A
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15
Q

Ttt of
-minor B-thalassemia
- major B- thalassemia

A

-minor = no ttt
- major = early lifelong blood transfusion + iron chelation if serum ferritin concentrations exceed 1000 ng/mL. + HSCT is indicated for severe β-thalassemia major

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16
Q

Ttt of anemia of chronic disease?

A

مافيه هههههههههه😝
Inflammatory anemia is usually not severe and rarely requires therapy.

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17
Q

Patient on electrophoresis has high A2, Dx?

A

Thalassemia minor

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18
Q

When to do spleenectomy as therapy for thalassemia

A
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19
Q
A
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20
Q

Regarding a splenectomy as a management, which of the following
diseases can be CURED after splenectomy?

A
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21
Q

Management of HUS and TTP
+ سمعي قصه حصه وامها

A
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22
Q

Tumor lysis syndrome electrolyte ?
K
PO4
Urate
Ca

A
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23
Q

How to treat hyperkalemis in tumor lysis syndrome ?
1?
2?
3?
4?

A
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24
Q

Prophylaxis of tumor lysis syndrome

A
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25
Q

Treatment of
TTP
HUS?

A

plasma exchange
HUS, is supportaive , give abx only if the infection presisnt

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26
Q

What is the difference btw TTP and DIC in lab

A

D-dimer + PT, PTT normal in TTP and abnormal in DIC

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27
Q

Best tool to stage “T” in gastric cancer?

A

endoscopic US

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28
Q

Signs of compansated shock???
Mention 2

A

Tachycardia
And peripheral vasoconstriction ( pale periphary )

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29
Q

Critically ill patient in ICU after septic shock + now have high ALT AST total bilirubin
Dx
Tx?

A

Ischemic hepatitis
Self limited , no specific ttt

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30
Q

First line therapy in patient with urticaria after IV contrast?

A

Epinephrine

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31
Q

Type of inncoent murmur that present in all anemia ?

A

Systolic murmur

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32
Q

Restless leg syndrome found in which type of anemia?

A

IDA

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33
Q

77 old male with iron deficiency anemia , next step?

A

Colonoscopy for colon cancer

34
Q

Young patient treated from IDA with oral iron with no improvement , what to suspect ?

A

Celiac

35
Q

Dx?

A
36
Q

Indication of IV iron in IDA?

A
37
Q

IDA in patient with CKD on dialysis , what to give?

A

Erythropoietin (HB target 10-12 )

38
Q

Mood of inheritance in thalassemia?
Type and location of mutation??.

A

Autosomal reccessive

39
Q

In electrophoresis of thalassemia
Which will be absent
And which will be increased ?

A

HbA
HbF

40
Q

Ttt of iron oveload in
Thalssemia?
Hemochromatosis

A

Iron chelating agent
Phlebectomy

41
Q

Confirmatory test for hereditary hemochromatosis?

A

Liver biopsy + genitic testing (HFE gene)

42
Q

Type and location of mutation in SCD ?

A

Autosomal recessive point mutation of B-chain in 6th position

43
Q

The difference in lab btw aplastic vs splenic crisis ?

A

Retics count

44
Q

SCD prophylaxis?

A
45
Q

Thresholds and situations to consider platelet transfusion for epidural anasthesia ? Neurosurgery? Central line ?

A

80 000
100 000
20 000

46
Q

Child with Isolated thrombocytopenia (50 000 ) + epistaxis
Ttt?
Of female intermenstraul bleeding with PLT (50 000 )
Ttt?

A

Steroid
This is ITP case

47
Q

Female with sudden anemia + schisocyte + low platelet + purpura + headache 🤕 + fever
Definitive test?
Ttt if no major hemorrhage
Ttt if major hemorrhage

A

Dx: TTP 👩😰😭😭💔👨‍🦰❌
ADAM TS 13 LEVEL
plasma exchange
Platelet transfusion

48
Q

Child after GI infection had
Anemia with schisocyte
Renal impairment
Low PLT
Dx and mx?

A

HUS
Typical HUS: supportive only
Atypical HUS: eculizumab ( causes fulminant meningococcal meningitis, screen first )

49
Q

This little cute baby and his brothers had
Atopic dermatitis + infection + bleeding

A
50
Q

Patient had massive postpartum hemorrhage + blood oozing from IV cannula site
And now her labs are

⬇️PLT
⬆️ PT
⬆️ PTT
⬆️ D-dimer
⬇️ fibrogen

Dx
Mx?

A

DIC

51
Q

Train of severe aortic stenosis + acquired vWD + GI bleed
Dx ?
Mx

A

Hedye syndrome
Aortic valve replacement

52
Q

Female and all her sisters had epistaxis and purpura +
Prolonged bleeding time
Dx and type of inheritance ?
Mx?

A

VWD ( Autosomal dominant )
Dx by vWF level and activity + VIII level + multi timer study to determine the subtype

Ttt by desmopressin

53
Q

The only 5️⃣ indication of iron injection instead of oral are?

A
54
Q

A2 in thalassemia should be more than?

A

All B-thalassemia types
A2> 3.5%
If less it is not thalassemia

55
Q

Patient with shoulder and ankle arthritis ( strange location 🤔)
+ high feritin
Dx
Mx

A

Hereditary Hemochromatosis (all hemochromatosis should have HIGH FERETIN
Phlebotomy

56
Q

Patient with bruises + normal platelet count + prolonged Bleeding time
Dx?

A
57
Q

Patient with thrombocytopenia + shisocyte
Dex?

A

TTP
HUS
DIC

58
Q

Patient with recurrent abortions + prolonged PTT
With negative mixing study

Dx?

A
59
Q

Patient with severe platelet drop (>50%) with skin necrosis after 7 days of heparin therapy

Best investigation?
Management ?

A
60
Q

Patient after massive tranfusion of pRBC only, developed oozing blood from NGT and cannula site
Dx?

A

Dilutional thrombocytopenia

61
Q

Timing of blood transition reaction?
First 10 min?
First 30 min?
2-10 days

A

First 10 min ➡️ anaphylactic ( common in IgA deficient patient )
First 30 min or first 1 hour ➡️ ABO incompatibility,
1-6 hours➡️ non-hemolytic febrile reaction (MOST COMMON )
2-10 days➡️ delayed hemolytic reaction,

62
Q

Most common mutation found in polycythemia rubra vera

A

JAK2

63
Q

Spleenomegaly found in primary or secondary polycythemia or both?

A

Primary only

64
Q

Gold standard to diagnose polycythemia rubra

A

JAK2 Mutation

65
Q

What to add if phlebotomy failed to control Polycythema rubra vera (45%) ?

A

Cytoreductive drug 💊
1- hydroxyurea ( in young male ), if no improvement = rituximab
2- interferon a (child bearing female )
3- Rituximab (old 👵 👴 )

All patient should have
Phlebotomy + low dose daily aspirin

66
Q

Patient with resistant level of PLT >450 000 + DVT hx + spleenomegaly
Dx
Most common mutation?
Ttt?

A

Essential thrombocytosis
JAK2
Very low risk = observe
Low risk = daily low dose aspirin
High risk = cytoreductive drug (hydroxy urea)

67
Q

Patient diagnosed 5 years ago q
With CML
Now developed anemia, recurrent infections, and purpura + spleenomegaly
Now PBS show Dacrycyte ( tear drop cell)
No phladilphia chromosome
Dx and most definitive test

A

Myelogibrosis

BM trephine biopsy → Markedly ↑ fibrosis

68
Q

How to confirm IDA

A

Low ferritin

69
Q

Hollwel-jolly body + target cell + sickle cell

Dx?

A

SCD

70
Q

In pernicious anemia there is atrophic gastritis , this is increase the risk of??
تذكري اسمها بالعربي الانيما الخبيثه يعني بتحيب شي خبييييث

A

gastric cancer

71
Q

Patient with
Short stature and microcephaly + cafe-eu-late spot + malformation of thumb
And developed aplastic anemia
Dx?

A

Fanconi anemia

72
Q

Gold standard to diagnose aplastic anemia?

A

BM biopsy showing hypocellular BM and replaced by fat

73
Q

Which hematology parameter will be normal in HEELP syndrome?

A

Normal coagulation

HEELP+N
تخليي وحده قيصيمه تنطق كلمة هيلب راح تقول هليبببنننن

Hemolytic anemia (MAHA ) + Elevated liver enzyme + Low Platelet + Normal coagulation

74
Q

WARNING ⚠️ 🚨🚨🚨🚨🚨 LIFE-THREATENING CASE
Patient with intermittent Jaundice and dark urine at morning with venous thrombosis at unusual locations (hepatic and abdominal Veins)
CBC: pancytopia

Dx?
Definitive test?

A

Paroxysmal noctural hemoglobinuria

Flow cytometry fonfirm abscence of CD55-59

75
Q

HbH
Found in which anemia?

A

A-thalassemia

76
Q

Ttt of thalassemia major ( tranfusion dependant thalassemia)
5 things

A

1️⃣ Regular life-long transfusions to keep Hb >9g/dL → suppress ineffective haematopoiesis + allow growth
2️⃣ Folic
3️⃣ Iron Chelating agent as Desferroxamine
4️⃣ Splenectomy May be done after 5y
5️⃣ Definitive Rx: BM transplant

77
Q

Best initial and most accurate test in SCD

A
78
Q

Autoimmune hemolytic anemia (warm ) + immune mediated thrombocytopenia
In patient with high WBC and lymphocytosis with signs of infection
Dx and mx?

A

Evans syndrome in CLL
Steroid

79
Q

Autoimmune hemolytic anemia (warm ) + immune mediated thrombocytopenia
In patient with high WBC and lymphocytosis with signs of infection
Dx and mx?

A

Evans syndrome in CLL
Steroid

80
Q

Patient with painless lymphadenopathy
But it become painful when drinking 🍺 🤨🤨🤨
Dx?

A

Dx
يستاهل
Hodgkin lymphoma

81
Q

You found painless enlarged lymph node, next step?

A

Excisional biopsy
NOT FNA

82
Q

Best investigation to monitor disease activity in lymphoma

A

Internal Medicine- Areej (6 decks)

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