Hematology Flashcards
Value of ferritin to R/O IDA
> 100
To confirm IDA
Low IRON
High TIBC
TRansferrin Sat < 15
Ferritin < 14
How long should be continued oral iron after normalization of Hb
3-6 months
Anemia of inflammation
High ferritin
Low iron & TIBC
TF sat normal / low
Normocytic , over time microcytic
Fo ESRD , erythropoietin indicated if ?
What’s the target Hb
Hb < 10
Hb Should not exceed 11.5 to avoid adverse effects: uncollected HTN, volume overload, thrombosis
Ferritin & TF sat in patient taking erythropoietin?
Ferritin > 100
TF sat > 20
Vit B12 > …….? To exclude B12 deficiency
221
Best indicator of B12 deficiency
High methylmalonic acid & Homocysteine
Best indicator of folate deficiency
Normal methylmalonic acid
High Homocysteine
What’s the preferred test when folate deficiency suspended despite of normal serum folate level ?
RBC folate
Or
High Homocysteine
Drug indicated for ttt of anemia in patient who requires regular RBC transfusion
Luspatercept
Cure treatment of sickle cell disease
allogeneic HSCT
Hydroxyurea indicated in SCD with
- Frequent pain event > 3 per year
- Severe symptomatic anemia
- Previous acute chest syndrome
- Stroke
Agents approved for ttt and prevention of painful events in SCL
- L- glutamine
- Crizanlizumab
Acute Chest syndrome treatment
Empiric antibiotic
Hydration
O2
Spirometry
Analgesia
RBC transfusion either with PRBC or as exchange transfusion
Sildenafil in SCD
Increase painful crises
Preoperative transfusion target Hb
10
Hb target in SCD to avoid problem with blood hyperviscosity
Hb < 10
Management of hereditary spherocytosis
Acute hemolytic crisis:
1. supportive
2. transfusion if needed
Longer term treatment:
1. Folic acid replacement
2. Splenectomy
Hereditary spherocytosis labs
High MCHC , osmotic fragility
Flow cytometry : reduced EMA binding to band 3 on erythrocytes
Warm hemolytic anemia treatment
- Steroids
- Rituximab
- Splenectomy
Cold hemolytic anemia treatment
1.Avoid cold exposure
2. Steroids
3. Splenectomy
4. Rituximab + fludarabine or bendamustine
PNH diagnosis
Flow cytometry : loss of CD 55 and CD 59
PNH treatment
Eculizumab and ravulizumab
Which vaccine should be received before using Eculizumab and ravulizumab?
Meningococcal vaccination
Hemochromatosis diagnosis
Ferritin > 200
TRF sat > 45
What is Factor V Leiden
Mutated Factor V is resistant to cleavage by activated Protein C, leading to a prothrombotic state.
How to diagnose factory V Leiden
FVL genetic testing or activated protein C resistance test
What is the most common inherited thrombophilia type
FVL
Prothrombin G20210A Gene Mutation ?
Increase production of prothrombin ( F II)
What is the natural anticoagulant?
- Antithrombin III
- Protein C
- Protein S
What’s the main role of AT III ?
Inhibit thrombin & activated factor IX
AT III deficiency is high risk in …?
Pregnancy, leading to VTE related pregnancy loss & pregnancy morbidity
Common causes of Acquired decreased anticoagulant factor levels AT III, protein S and Protein C
- Acute thrombosis
- Liver disease
- DIC
- Nephrotic syndrome
- Protein losing enteropathy
Acquired decreased anticoagulant factor
protein S and Protein C only
- Warfarin therapy
- Vit K deficiency
Acquired decreased anticoagulant factor
protein S only
- Inflammatory states
- Estrogen
- HIV
- L asparaginase chemotherapy
Acquired decreased anticoagulant factor
levels AT III only
ECMO
Hemodialysis
Severe trauma or burns
L asparaginase chemotherapy
Risk of DVT after Hip fracture surgery
60 %
Risk of DVT in patient undergo general surgery
15-30 %
APLAS evaluation includes?
anticardiolipin antibodies
anti–β2-glycoprotein antibodies
lupus anticoagulant
in patients with confirmed APLAS being managed with anticoagulant therapy, the 2021 CHEST guideline suggests adjusted-dose vitamin K antagonist , Target INR
target INR 2.5
adjusted-dose vitamin K antagonist over DOAC therapy during the treatment phase
Signs of DVT
- Unilateral swelling
- Pain
- Warmth
- Erythema
What’s the indication for IVC filter
Acute proximal DVT or Acute PE
With contraindication to anticoagulation
Thrombolytic therapy indication in DVT
Massive DVT leading to impaired venous drainage, severe edema , acute limb ischemia
Thrombolytic therapy indication in PE
Massive PE and shock from cardiac output
What to do if INR > 4.5 - 10 on warfarin
Withhold warfarin
What to do if INR > 10 without bleeding on warfarin
Oral Vit K
What to do if INR high And there is life threatening bleeding on warfarin
Iv Vit K and 4 factor prothrombin complex concentrate
Leukemia with splenomegaly + pancytopenia+ dry tap on aspiration
Hairy cell leukemia
APML t ( ……;…..)
t ( 15;17)
ALL t ( ……;…..)
T(12;21)
T(8;4)
T(4;11)
Diagnosis of AML by cytogenetic
T ( 15;17) , t ( 8;21 ) , inv ( 16)
Favorable AML
T ( 8;21)
T( 15;17)
Inv (16)
Unfavorable AML
Monosomal karyotype
11q23
T( 6;9) , t ( 9;22) , inv (3) , -5/5q , -7/7q, TP53 mutation
Unfavorable prognostications for AML
-Age > 60
-Poor performance status
-WBC > 100
-Prior disease of bone marrow ( MDS , MPLD )
Leukemia + infiltrate gums
Acute monocytic leukemia
Pancytopenia + hypo cellular marrow
Aplastic anemia
Pancytopenia + hyper cellular marrow
MDS
Treatment of aplastic anemia
- Age > 50 or younger but no suitable stem cell donor , antithymocyte globulin , cyclosporine , prednisone, eltrombopag
- Younger patient, allogeneic HSCT
- Asymptomatic with mild , close monitoring without immediate treatment
Idiopathic Pure red cell aplasia treatment
Prednisone, cyclosporine , cyclophosphamide
Pure red cell aplasia treatment due to thymoma
Thmectomy
Pure red cell aplasia treatment in immunocompromised
Iv Ig
Evaluation of polycythemia To R/O 2ry causes
1.Pulse oxygenation
2.sleep study
3.Echo
4.kidney U/S
Treatment of PV?
HCT level target?
- Phlebotomy : HCT < 45%
- ASA
- Cytoreductive therapy: Hydroxyurea & interferon alpha
- JAK1/2 inhibitor: ruxolitinib
- Patient with venous thrombosis will require lifelong anticoagulation
Indication of cytoreductive therapy in PV
- Age > 60
- Hx of MI, stroke , venous thrombosis
Indication of JAK1/2 inhibitor
Ruxolitinib
If resistant or can’t tolerate first line
causes of 2ry thrombocytosis
1.IDA
2.chronic bleeding
3.splenectomy
4.infection
5.inflammatory condition
treatment of ET of low risk
Patients at low risk (age <60 years, JAK2 negative, no venous or arterial thrombosis) can be monitored without therapy or
aspirin if vasomotor symptoms are present, such as headache or erythromelalgia.
treatment of ET of high risk
age > 60 years and who are JAK2 positive (with or without venous or arterial thrombosis) and should be treated with
1.aspirin and
2. cytoreductive therapy with hydroxyurea or Interferon-α should be considered in younger patients and during pregnancy
- hydroxyurea is potentially teratogenic.
Anagrelide is effective at decreasing platelet counts; however, it is typically reserved for patients who cannot tolerate or do not respond to hydroxyurea.
patients with acute venous thromboembolism will require lifelong anticoagulation.
treatment of PMF
1.allogeneic HSCT
2.Asymptomatic patients with low-risk disease can be monitored
however, most patients have some degree of symptomatology and may benefit from supportive medications. JAK1/2 inhibitor ( Ruxolitinib, Fedratinib) that has been shown to significantly improve constitutional symptoms in patients with PMF and reduce spleen volume
Causes of eosinophilia
can be recalled by the mnemonic CHINA
C collagen vascular disease ( eosinophailic granulomatosis with polyangitis )
H h=Helminitic (parasite ) infection (strongyloides)
I idiopathic hypereosinophilic syndrme
N neoplasia (PMF,AML,Lymphomas)
A allergy
Treatment of primary HES
low doses of imatinib
Glucocorticoids are effective, but it is imperative to rule out active Strongyloides infection first because glucocorticoids can cause dissemination, which can be fatal.
VWD investigation
- Prolonged bleeding time & Maybe aPTT
- F VIII level may be moderately reduced
Diagnosis of mastocytosis
1.Skin biopsy for cutaneous mastocytosis
- BM biopsy for systemic mastocytosis
Treatment of mastocytosis
Anti histamine & Anti leukotriene to control symptoms
Cytoreductive therapy for aggressive forms
MDS treatment
1.Asymptomatic low risk can be monitored
- Allogeneic HSCT is curative option but not in old age
Hypomethylating agents ( azacytidine & decitabine ) decrease transfusion dependence & risk of conversion to AML
Low risk MDS with the 5q cytogenetic abnormality can be treated with
Lenalidomide if they are transfusion dependent
Treatment of hemochromatosis
Phlebotomy one or twice weekly then tapering to maintain a target ferritin 50-100
Once reached the target, phlebotomy 2-6 times yearly and check ferritin 3-6 months
Screening for HCC in hemochromatosis
Patient with cirrhosis ( stage 4 )
Indication for autologous HSCT
MM
Relapsed non Hodgkin lymphoma
Frequent transfusions may lead to?
alloimmunization, increasing the risk of hemolytic disease of the newborn
Painful vaso-occlusive crises increase during pregnancy; pain should be managed with
opioid analgesics,
NSAIDs should be avoided after the 30th week of gestation to avoid premature closure of the fetal PDA
gestational thrombocytopenia?
no history of thrombocytopenia, and
the platelet count does not decrease until late in gestation
Differentiating ITP from gestational thrombocytopenia may be difficult, but features suggesting ITP include
1.earlier presentation (first trimester)
2.lower platelet count nadir <70 × 109/L,
3.history of thrombocytopenia before pregnancy (even if it was only mild)
Treatment of ITP in pregnancy
Patients should receive iv Ig
or
glucocorticoids at any time during pregnancy if the platelet count decreases to < 30 .
Platelets targets for delivery (vaginal or cesarean section)
Plt > 50
therapy should begin approximately 1 week before the expected delivery date to achieve that goal.
Neuraxial anesthesia requires a platelet count > 80 .
acquired asymptomatic increase in neutrophil count, including
- asplenia
- chronic inflammatory conditions such as IBD,
- obesity
4.vigorous exercise
Screen for………. in survivors of pediatric leukemia.
1.dyslipidemia
- DM
whole-body low-dose CT scan negative for smoldering myeloma should be followed by …..?
whole-body MRI.
Which DOAC approved in ESRD?
Apixaban