המטולוגיה Flashcards
Definition of physiologic anemia in newborn?
peak in 6-12 weeks
Hb valure around 10-11 mg/dL
no Tx needed
גורמי סיכון לאנמיה מחסר ברזל
- כלכלה טבעונית
- תזונה עם חלב פרה
- מחזור
- מחלות כרוניות
which type of anemia is microcytic hypochromic with Oval RBS and high RDW?
Iron deficiency.
TAILS mnemonic for microcytic:
T- thalasemia
A- anemia of chronic disease
I- iron
L- lead
S- sidaroblast
Tx for iron def.?
ברזל אלמנטלי 3-6 מ”ג./ק”ג ליום, 2-3 חודשים למילוי המאגרים
definiton of Transient erythroblastopenia of chilhood
and what will be seen on lab results?
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.
Which medications can cause G6PD hemolytic anemia?
Nitropoentin
Primaquin
Sulfonemid
aspirin
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?
Heraditery sphirocytosis
AD
, המוליזה אקסטרא-ווסקולרית, צהבת, טחול מוגדל ולעיתים אבני מרה
almost the only disease with high MCHC
Dgx of herditery sphyrocytosis?
family Hx
splenomegaly
sphyrocytes in blood smear
high MCHC
Which test can raise a suspicion of Heraditery sphyrocytosis?
Osmotic fragile test
not sensetive/ specific can be positve in other diseases.
Tx for heraditery sphyrocytosis?
Folate
severe cases- splenectomy
which type of malignancy are high fanconi anemia pt are at high risk?
MDS
AML
SSC of head, neck , vulva
What is the pathophysiologic behind fanconi anemia
mutation in DNA reparing enzymes
what is the inherited form of aplastic anemia?
Fanconi syndrome
AR or X-linked
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?
Fanconi anemia
a/w other organs anomalies- kidney, heart, GI, head and eyes
Dgx of fanconi anemia?
and what is the definite Tx?
fragility test when exposue to DEB +
eleveted alpha-feto protein
or genetic
TX- BM transplant -= definite
Which AD syndrome (anemia) is a/w only red line hypoplasia (lack of precursors)?
Diamond blackfan anemia
Triphalangeal thubs is a/w which type of syncrome?
an what we will see in lab results?
Diamond blackfan anemia
macrocytic anemia
reticolucytopenia
Tx for blackfan diamond syndrome?
Steroids for life- recommend after first year of life
Which types of infections Sickle cell anemia is most prone to
- Osteomylaitis - salmonella
- Aplastic crisis- pravo B19
- incapsulate pathogens- S. pneumonia, HiB, Nissereha menengitis
which life threting complication can occure in sickle cell and a/w fever triger
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
what is the most common to be the first episode of sickle cell pt ?
and ehat is the Tx?
Dactelyitis
due to tissue ischemia
Tx- Paracetamol > NSAIDS > opiates > morphine IV
Tx for neurological complications in sickle cell disease?
Blood transfusion in 1 hr.
Targeted Hb = 10
+
Oxygen
Which sickle cell complication is menifest as HTN, headace, seziures, visual disturbance?
PRES
post. reversible leukoencephalopathy syndrome
what are the criteria to Dgx of acute chest syndrome (sickle cell disease)
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
chronic Tx for sickle cell disease?
- hydroxyurea > 9 month
- Penecillin PPx- until age of 5 unless Hx of pneumoccocal inf.
- Vaccination- Pneumoccoc, influenza, meningoccoc
- BM transplant- heal’s.
when we will consider BM transplant in sickle cell pt?
Reccurent episode of ACS, Strokes, abnormal TCD
Which Hb type is the dominant in delivery and when we will expcet it to change?
HbF ~ 70% at delivery (2alpha + 2 gamma)
around 6 month of life- will completly change to HbA (2 alpha + 2 Beta)
How to differiante between thalasemmia and Iron deficeincy?
MCV/ RBC < 13 thalasemmia
MCV/RBC > 13 iron def.
Thalasemmia- Target cells
Dgx of beta thalasemmia?
high levels of:
HbA2
+
HbF
Clinical menefidtstion of Beta thalasemmia major + electrophoresis?
Tx?
complications
clinical presentation:
Hemolytic anemia»_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
how many damage allele needed for:
* creation of Hb H?
* HbH disease?
- creation of Hb H- 2 damage alleles
- HbH disease- 3 damage allels
3 causes of MAHA?
- HUS
- DIC
- TTP
Tx for DIC?
- FFP- for coagulation factors
- מתן קריופרסיפיטט – Factor VIII, wVF, fibrinogen > 150
What is the most common hertiant bleeding disease ?
vWF disease (AD)
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?
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»_space; therefore can be present with slighlt eleveted PTT time
What are the main 2 conditions of baby that born with thrombocytopenia?
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.
ITP definition
autoimmune thrombocytopenia (isoleted)
mainly age 1-4
most common cause of isolated Thrombocytopenia in kids?
ITP
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?
Dgx- ITP
Tx- IVIG (tx of choice ) or steroids
in Rh+ pt- can treat with anti D
clinical dgx.
When we will not Treat ITP?
PLT > 20K
+
no bleeding story
Which infections are a/w ITP?
EBV. HIV, SLE, HP or vaccine
What we will see in blood smear of ITP?
low PLT + megakaryocytes
What are the indications for BM biopsy in ITP?
- hepatosplenomegaly
- joint or bone pain
- LAP
- WBC abnormalites
- Unexplain anemia
ITP:
How many % will have fully resolved?
how many will become chronic?
80% fully resolve w/in 6 months
chronic- 20%
Which syndrome is a/w
Eczme, Thrombocytopenia, immunodef.
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
Which type of malignancy are Wiskott aldrich syndrome a/w?
Lymphoproliferative (15%)
Which Ig will be low and which will be high in Wiskott aldrich syndrome
low/ normal IgM + IgG (M like W = low)
high IgA + IgE
Tx for Wiskott aldrich syndrome
Splenectomy
definitve Tx- BM transplant
What is the deficient in Glanzmann Thrombasthenia
Labs of disease?
GPIIbIIIa def. = fibronogen cant bind PLT - no activation
long bleeding time + normal PLT
What is tested by ristocin test?
fucntion of vwF and GP1b
if positive = normal
abnormal in wVF diseas and bernards soulier
Triad of HUS?
common pathogen and timline
- MAHA- microangiopathic hemplysis
- Thrombocytopenia
- AKD
E.coli 157:O7»_space; shiga toxin
after 5-7 days of ganstroenteritis with bloody diarrhea
What is the inheritance pattern of Hemophilia?
A,B,C
all X-linked ressesive
A- 8
B- 9
C-11
most common inherited thrombosis syndrome?
ans what is the pathophysiology?
factor V leiden
factor C cant bind mutant factor V»_space; increase risk for VENUOS thrombosis
AD
Use of which medication can increase the risk for thrombosis?
OCP use
(also pregnancy)
Which 2 main complications can be seen in A-splenism?
- capsular pathogens infections.
- 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
A child present with lymphadnitis. on exmination there is unilateral LAD submaidibular.
the LN is painless and labile.
what is the most likely pathogen?
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
Tx for lympahdenitis from bacterial source
cover Strep + SA with cheplaosporin first generation (Cefazolin / Cefalaxin)
how can we prevent febrile non hemolytic transfusion reaction ?
מנה מסוננת מלויקוציטים
השכיחה ביותר, בגלל נוגדנים של המקבל ללויקוציטים של התורם. בד”כ רק חום וצמרמורות
