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מבחן גמר בילדים > המטולוגיה > Flashcards

המטולוגיה Flashcards

1
Q

Definition of physiologic anemia in newborn?

A

peak in 6-12 weeks
Hb valure around 10-11 mg/dL

no Tx needed

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2
Q

גורמי סיכון לאנמיה מחסר ברזל

A
  • כלכלה טבעונית
  • תזונה עם חלב פרה
  • מחזור
  • מחלות כרוניות
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3
Q

which type of anemia is microcytic hypochromic with Oval RBS and high RDW?

A

Iron deficiency.

TAILS mnemonic for microcytic:
T- thalasemia
A- anemia of chronic disease
I- iron
L- lead
S- sidaroblast

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4
Q

Tx for iron def.?

A

ברזל אלמנטלי 3-6 מ”ג./ק”ג ליום, 2-3 חודשים למילוי המאגרים

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5
Q

definiton of Transient erythroblastopenia of chilhood

and what will be seen on lab results?

A

דיכוי זמני של אריתרופואזיס לאחר מחלה ויראלית
בד”כ ילדים בגילאי 6 חודשים- 3 שנים
self limited

labs
Reticulocytopenia
normocytic anemia (mild-severe)
neutropenia (20% of cases)
normal MCV- different to iron deff.

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6
Q

Which medications can cause G6PD hemolytic anemia?

A

Nitropoentin
Primaquin
Sulfonemid
aspirin

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7
Q

a 5 year old boy present with RUQ pain. on US gallbladder bones are seen , and labs there is anemia, high MCV, jaundive in rticulocytosis.

his perents tell you that is uncle has splenomegaly and also pigment bile stones.

what is the most likley disease?

A

Heraditery sphirocytosis
AD

, המוליזה אקסטרא-ווסקולרית, צהבת, טחול מוגדל ולעיתים אבני מרה

almost the only disease with high MCHC

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8
Q

Dgx of herditery sphyrocytosis?

A

family Hx
splenomegaly
sphyrocytes in blood smear
high MCHC

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9
Q

Which test can raise a suspicion of Heraditery sphyrocytosis?

A

Osmotic fragile test

not sensetive/ specific can be positve in other diseases.

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10
Q

Tx for heraditery sphyrocytosis?

A

Folate

severe cases- splenectomy

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11
Q

which type of malignancy are high fanconi anemia pt are at high risk?

A

MDS
AML
SSC of head, neck , vulva

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12
Q

What is the pathophysiologic behind fanconi anemia

A

mutation in DNA reparing enzymes

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13
Q

what is the inherited form of aplastic anemia?

A

Fanconi syndrome
AR or X-linked

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14
Q

A 6 year ols peresnt with wierd thumbs, cafe-au liet and short sature

on blood exams there are high blastocytes. with pancytopenia and macrocytic anemia

what is the most likey Dgx?

A

Fanconi anemia

a/w other organs anomalies- kidney, heart, GI, head and eyes

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15
Q

Dgx of fanconi anemia?

and what is the definite Tx?

A

fragility test when exposue to DEB +
eleveted alpha-feto protein

or genetic

TX- BM transplant -= definite

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16
Q

Which AD syndrome (anemia) is a/w only red line hypoplasia (lack of precursors)?

A

Diamond blackfan anemia

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17
Q

Triphalangeal thubs is a/w which type of syncrome?

an what we will see in lab results?

A

Diamond blackfan anemia

macrocytic anemia
reticolucytopenia

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18
Q

Tx for blackfan diamond syndrome?

A

Steroids for life- recommend after first year of life

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19
Q

Which types of infections Sickle cell anemia is most prone to

A
  1. Osteomylaitis - salmonella
  2. Aplastic crisis- pravo B19
  3. incapsulate pathogens- S. pneumonia, HiB, Nissereha menengitis
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20
Q

which life threting complication can occure in sickle cell and a/w fever triger

A

splenic sequestration
הגדלה של הטחול + ירידה חדה בהמוגלובין. לרוב טריגר של חום , בקטרמיה או זיהום כלשהו

הישנות 66% לרוב תוך 6 חודשים מהאירוע הקודם

מתייצג ילד עם כאב בטן שמאלית ואי יציבות המודינאמית

טיפול דפיניטיבי- הסרת הטחול

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21
Q

what is the most common to be the first episode of sickle cell pt ?

and ehat is the Tx?

A

Dactelyitis
due to tissue ischemia

Tx- Paracetamol > NSAIDS > opiates > morphine IV

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22
Q

Tx for neurological complications in sickle cell disease?

A

Blood transfusion in 1 hr.
Targeted Hb = 10
+
Oxygen

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23
Q

Which sickle cell complication is menifest as HTN, headace, seziures, visual disturbance?

A

PRES
post. reversible leukoencephalopathy syndrome

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24
Q

what are the criteria to Dgx of acute chest syndrome (sickle cell disease)

A

new shadow on CXR + 2 of the following
1. fever
2. dyspnea
3. hypoxemia
4. cough
5. chest pain

ACS- סיבוך מסכן חיים. חייב לבצע צל”ח בכל ילד קטן עם חום ללא סימנים רספירטורים

25
Q

chronic Tx for sickle cell disease?

A
  • hydroxyurea > 9 month
  • Penecillin PPx- until age of 5 unless Hx of pneumoccocal inf.
  • Vaccination- Pneumoccoc, influenza, meningoccoc
  • BM transplant- heal’s.
26
Q

when we will consider BM transplant in sickle cell pt?

A

Reccurent episode of ACS, Strokes, abnormal TCD

27
Q

Which Hb type is the dominant in delivery and when we will expcet it to change?

A

HbF ~ 70% at delivery (2alpha + 2 gamma)
around 6 month of life- will completly change to HbA (2 alpha + 2 Beta)

28
Q

How to differiante between thalasemmia and Iron deficeincy?

A

MCV/ RBC < 13 thalasemmia
MCV/RBC > 13 iron def.

Thalasemmia- Target cells

29
Q

Dgx of beta thalasemmia?

A

high levels of:
HbA2
+
HbF

30
Q

Clinical menefidtstion of Beta thalasemmia major + electrophoresis?

Tx?

complications

A

clinical presentation:
Hemolytic anemia&raquo_space; jaundice
CHF- first 6 months of life
cheepmonke face- forehead and cheeks are prominant

electrophoresis
high levels of HbF

Tx- reccurent blood transfusion, BM transplant

complication- iron excess > liver MRI from age 10

31
Q

how many damage allele needed for:
* creation of Hb H?
* HbH disease?

A
  • creation of Hb H- 2 damage alleles
  • HbH disease- 3 damage allels
32
Q

3 causes of MAHA?

A
  1. HUS
  2. DIC
  3. TTP
33
Q

Tx for DIC?

A
  • FFP- for coagulation factors
  • מתן קריופרסיפיטט – Factor VIII, wVF, fibrinogen > 150
34
Q

What is the most common hertiant bleeding disease ?

A

vWF disease (AD)

35
Q

A girl age 12 presemt with reccurent bleeding and epistaxis. on exmination the pt tells that she have also severe menses and on Labs there are normal PT, normal PTT and prolong bleeding time. which test can confirm your dgx? what is the Tx?

A

Von willibrand disease

Dgx- Ristocin test will show now aggregation (negetive test)

Tx- Desmopressin (elevete vWF) or given loss substance in severe cases

Desmopresin - also Tx for central DI, and primary night wetting

dont forget VWF is also a carrier of factor VIII&raquo_space; therefore can be present with slighlt eleveted PTT time

36
Q

What are the main 2 conditions of baby that born with thrombocytopenia?

A

1. NATP = neonatal alloimmune thrombocytopenia - maternal Ab’s produce against PLT of paternal origion. severe disease that can worsen with each preganancy
* Tx- in pregnent women- IVIG, after delivery in newborn- maternal origion PLT

2. mother with ITP
* Tx- during pregnanct- steroids, tx in newborn- IVIG and sometimes steroids.

37
Q

ITP definition

A

autoimmune thrombocytopenia (isoleted)
mainly age 1-4

38
Q

most common cause of isolated Thrombocytopenia in kids?

A

ITP

39
Q

A 3 year old girl present with pathcias and mucosal bledding. on exmination her mother mention that she had a viral inf. 3 weeks ago
on labs- PLT < 100K

what is the most likley dgx and tx?

A

Dgx- ITP
Tx- IVIG (tx of choice ) or steroids
in Rh+ pt- can treat with anti D

clinical dgx.

40
Q

When we will not Treat ITP?

A

PLT > 20K
+
no bleeding story

41
Q

Which infections are a/w ITP?

A

EBV. HIV, SLE, HP or vaccine

42
Q

What we will see in blood smear of ITP?

A

low PLT + megakaryocytes

43
Q

What are the indications for BM biopsy in ITP?

A
  1. hepatosplenomegaly
  2. joint or bone pain
  3. LAP
  4. WBC abnormalites
  5. Unexplain anemia
44
Q

ITP:
How many % will have fully resolved?
how many will become chronic?

A

80% fully resolve w/in 6 months

chronic- 20%

45
Q

Which syndrome is a/w

Eczme, Thrombocytopenia, immunodef.

A

Wiskkot Aulbrich syndrome
WATER:
W-wiskkot
A-aulbrich
T-thromboytopenia (micro PLT)
E- Eczme
R- reccurent infection

combine T & B dysfunction

X-link ressecive, WAS mutation

46
Q

Which type of malignancy are Wiskott aldrich syndrome a/w?

A

Lymphoproliferative (15%)

47
Q

Which Ig will be low and which will be high in Wiskott aldrich syndrome

A

low/ normal IgM + IgG (M like W = low)
high IgA + IgE

48
Q

Tx for Wiskott aldrich syndrome

A

Splenectomy

definitve Tx- BM transplant

49
Q

What is the deficient in Glanzmann Thrombasthenia

Labs of disease?

A

GPIIbIIIa def. = fibronogen cant bind PLT - no activation

long bleeding time + normal PLT

50
Q

What is tested by ristocin test?

A

fucntion of vwF and GP1b
if positive = normal

abnormal in wVF diseas and bernards soulier

51
Q

Triad of HUS?

common pathogen and timline

A
  • MAHA- microangiopathic hemplysis
  • Thrombocytopenia
  • AKD

E.coli 157:O7&raquo_space; shiga toxin

after 5-7 days of ganstroenteritis with bloody diarrhea

52
Q

What is the inheritance pattern of Hemophilia?
A,B,C

A

all X-linked ressesive
A- 8
B- 9
C-11

53
Q

most common inherited thrombosis syndrome?

ans what is the pathophysiology?

A

factor V leiden

factor C cant bind mutant factor V&raquo_space; increase risk for VENUOS thrombosis

AD

54
Q

Use of which medication can increase the risk for thrombosis?

A

OCP use

(also pregnancy)

55
Q

Which 2 main complications can be seen in A-splenism?

A
  1. capsular pathogens infections.
  2. Thromboembolic complications

must give vaccination of capsular pathogens at least > 14 days before splenectomy (prevner + pneumovax fromage 2, and second dose after 5 yrs),
no C/I for any vaccine

56
Q

A child present with lymphadnitis. on exmination there is unilateral LAD submaidibular.
the LN is painless and labile.

what is the most likely pathogen?

A

non TB mycobacteria

אזור סאב מנדיבולרי / פרה-אאיקליץ
הבלוטה ניידת ולא כואבת > מתרככת ומתנזלת > צבע העור משתנה לכחול > ניקוז חיצוני

אם לא מתנקזת- יש להוציא בשלמות ואם לא ניתן- טיפול תרופתי

57
Q

Tx for lympahdenitis from bacterial source

A

cover Strep + SA with cheplaosporin first generation (Cefazolin / Cefalaxin)

58
Q

how can we prevent febrile non hemolytic transfusion reaction ?

A

מנה מסוננת מלויקוציטים

השכיחה ביותר, בגלל נוגדנים של המקבל ללויקוציטים של התורם. בד”כ רק חום וצמרמורות

מבחן גמר בילדים (23 decks)

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