Hematology Flashcards
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
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
What is the indication of flow cytometry in preipheral blood smear
In any patient CBC if you found sphereocyte , next step???
Comb test
Challenge time!!
Type of inheritance
1- hereditary spherocytosis ?
2- Sickle cell
3- G6PD
4- hereditary spherocytosis + hereditary elliptocytosis
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
Child with anemia + spleenomegaly + pigmented gallstone + negative combs
How to confirm
And how to treat
CBC shown
Eosin-5-maleimide binding test (EMA binding test)
Hereditary spherocystosis
Indication of exchange transfusion in SCD
Young SCD with decrease in HB (>2g ) with thrombocytopenia and reticulocytosis, dx and mx
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
What is the lower accepted Hb level in SCD patient going to surgery
10 Hb
Indication of urgent blood transfusion in SCD ?
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.
Most common mutation in primary polychythemia rupra vera ?
JAK2
Ttt of polychythemia rupra vera
1 procedure ?
2 drugs?
Approach to isolated thrombocytopenia?
Anticoagulant in patient with HIT (heparin induced thrombocytopenia)
argatroban , danaparoid
bivalirudin, fondaparinux) and direct oral anticoagulants (DOACs) .
RDW +Menztor index is high in iron deficiency anemia or thalassemia ?
Ttt of
-minor B-thalassemia
- major B- thalassemia
-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
Ttt of anemia of chronic disease?
مافيه هههههههههه😝
Inflammatory anemia is usually not severe and rarely requires therapy.
Patient on electrophoresis has high A2, Dx?
Thalassemia minor
When to do spleenectomy as therapy for thalassemia
Regarding a splenectomy as a management, which of the following
diseases can be CURED after splenectomy?
Management of HUS and TTP
+ سمعي قصه حصه وامها
Tumor lysis syndrome electrolyte ?
K
PO4
Urate
Ca
How to treat hyperkalemis in tumor lysis syndrome ?
1?
2?
3?
4?
Prophylaxis of tumor lysis syndrome
Treatment of
TTP
HUS?
plasma exchange
HUS, is supportaive , give abx only if the infection presisnt
What is the difference btw TTP and DIC in lab
D-dimer + PT, PTT normal in TTP and abnormal in DIC
Best tool to stage “T” in gastric cancer?
endoscopic US
Signs of compansated shock???
Mention 2
Tachycardia
And peripheral vasoconstriction ( pale periphary )
Critically ill patient in ICU after septic shock + now have high ALT AST total bilirubin
Dx
Tx?
Ischemic hepatitis
Self limited , no specific ttt
First line therapy in patient with urticaria after IV contrast?
Epinephrine
Type of inncoent murmur that present in all anemia ?
Systolic murmur
Restless leg syndrome found in which type of anemia?
IDA
77 old male with iron deficiency anemia , next step?
Colonoscopy for colon cancer
Young patient treated from IDA with oral iron with no improvement , what to suspect ?
Celiac
Dx?
Indication of IV iron in IDA?
IDA in patient with CKD on dialysis , what to give?
Erythropoietin (HB target 10-12 )
Mood of inheritance in thalassemia?
Type and location of mutation??.
Autosomal reccessive
In electrophoresis of thalassemia
Which will be absent
And which will be increased ?
HbA
HbF
Ttt of iron oveload in
Thalssemia?
Hemochromatosis
Iron chelating agent
Phlebectomy
Confirmatory test for hereditary hemochromatosis?
Liver biopsy + genitic testing (HFE gene)
Type and location of mutation in SCD ?
Autosomal recessive point mutation of B-chain in 6th position
The difference in lab btw aplastic vs splenic crisis ?
Retics count
SCD prophylaxis?
Thresholds and situations to consider platelet transfusion for epidural anasthesia ? Neurosurgery? Central line ?
80 000
100 000
20 000
Child with Isolated thrombocytopenia (50 000 ) + epistaxis
Ttt?
Of female intermenstraul bleeding with PLT (50 000 )
Ttt?
Steroid
This is ITP case
Female with sudden anemia + schisocyte + low platelet + purpura + headache 🤕 + fever
Definitive test?
Ttt if no major hemorrhage
Ttt if major hemorrhage
Dx: TTP 👩😰😭😭💔👨🦰❌
ADAM TS 13 LEVEL
plasma exchange
Platelet transfusion
Child after GI infection had
Anemia with schisocyte
Renal impairment
Low PLT
Dx and mx?
HUS
Typical HUS: supportive only
Atypical HUS: eculizumab ( causes fulminant meningococcal meningitis, screen first )
This little cute baby and his brothers had
Atopic dermatitis + infection + bleeding
Patient had massive postpartum hemorrhage + blood oozing from IV cannula site
And now her labs are
⬇️PLT
⬆️ PT
⬆️ PTT
⬆️ D-dimer
⬇️ fibrogen
Dx
Mx?
DIC
Train of severe aortic stenosis + acquired vWD + GI bleed
Dx ?
Mx
Hedye syndrome
Aortic valve replacement
Female and all her sisters had epistaxis and purpura +
Prolonged bleeding time
Dx and type of inheritance ?
Mx?
VWD ( Autosomal dominant )
Dx by vWF level and activity + VIII level + multi timer study to determine the subtype
Ttt by desmopressin
The only 5️⃣ indication of iron injection instead of oral are?
A2 in thalassemia should be more than?
All B-thalassemia types
A2> 3.5%
If less it is not thalassemia
Patient with shoulder and ankle arthritis ( strange location 🤔)
+ high feritin
Dx
Mx
Hereditary Hemochromatosis (all hemochromatosis should have HIGH FERETIN
Phlebotomy
Patient with bruises + normal platelet count + prolonged Bleeding time
Dx?
Patient with thrombocytopenia + shisocyte
Dex?
TTP
HUS
DIC
Patient with recurrent abortions + prolonged PTT
With negative mixing study
Dx?
Patient with severe platelet drop (>50%) with skin necrosis after 7 days of heparin therapy
Best investigation?
Management ?
Patient after massive tranfusion of pRBC only, developed oozing blood from NGT and cannula site
Dx?
Dilutional thrombocytopenia
Timing of blood transition reaction?
First 10 min?
First 30 min?
2-10 days
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,
Most common mutation found in polycythemia rubra vera
JAK2
Spleenomegaly found in primary or secondary polycythemia or both?
Primary only
Gold standard to diagnose polycythemia rubra
JAK2 Mutation
What to add if phlebotomy failed to control Polycythema rubra vera (45%) ?
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
Patient with resistant level of PLT >450 000 + DVT hx + spleenomegaly
Dx
Most common mutation?
Ttt?
Essential thrombocytosis
JAK2
Very low risk = observe
Low risk = daily low dose aspirin
High risk = cytoreductive drug (hydroxy urea)
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
Myelogibrosis
BM trephine biopsy → Markedly ↑ fibrosis
How to confirm IDA
Low ferritin
Hollwel-jolly body + target cell + sickle cell
Dx?
SCD
In pernicious anemia there is atrophic gastritis , this is increase the risk of??
تذكري اسمها بالعربي الانيما الخبيثه يعني بتحيب شي خبييييث
gastric cancer
Patient with
Short stature and microcephaly + cafe-eu-late spot + malformation of thumb
And developed aplastic anemia
Dx?
Fanconi anemia
Gold standard to diagnose aplastic anemia?
BM biopsy showing hypocellular BM and replaced by fat
Which hematology parameter will be normal in HEELP syndrome?
Normal coagulation
HEELP+N
تخليي وحده قيصيمه تنطق كلمة هيلب راح تقول هليبببنننن
Hemolytic anemia (MAHA ) + Elevated liver enzyme + Low Platelet + Normal coagulation
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?
Paroxysmal noctural hemoglobinuria
Flow cytometry fonfirm abscence of CD55-59
HbH
Found in which anemia?
A-thalassemia
Ttt of thalassemia major ( tranfusion dependant thalassemia)
5 things
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
Best initial and most accurate test in SCD
Autoimmune hemolytic anemia (warm ) + immune mediated thrombocytopenia
In patient with high WBC and lymphocytosis with signs of infection
Dx and mx?
Evans syndrome in CLL
Steroid
Autoimmune hemolytic anemia (warm ) + immune mediated thrombocytopenia
In patient with high WBC and lymphocytosis with signs of infection
Dx and mx?
Evans syndrome in CLL
Steroid
Patient with painless lymphadenopathy
But it become painful when drinking 🍺 🤨🤨🤨
Dx?
Dx
يستاهل
Hodgkin lymphoma
You found painless enlarged lymph node, next step?
Excisional biopsy
NOT FNA
Best investigation to monitor disease activity in lymphoma
