Hematology Flashcards by A A

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

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What is the indication of flow cytometry in preipheral blood smear

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

Comb test

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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

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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

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Indication of exchange transfusion in SCD

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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

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

10 Hb

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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.

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Most common mutation in primary polychythemia rupra vera ?

JAK2

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Ttt of polychythemia rupra vera
1 procedure ?
2 drugs?

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Approach to isolated thrombocytopenia?

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Anticoagulant in patient with HIT (heparin induced thrombocytopenia)

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

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

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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

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Ttt of anemia of chronic disease?

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

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Patient on electrophoresis has high A2, Dx?

Thalassemia minor

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When to do spleenectomy as therapy for thalassemia

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

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Management of HUS and TTP
+ سمعي قصه حصه وامها

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Tumor lysis syndrome electrolyte ?
K
PO4
Urate
Ca

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

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Prophylaxis of tumor lysis syndrome

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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

Hematology Flashcards

(82 cards)