Hematology Flashcards by Lucy Soudek

What is the mentzer index and how do you use it to determine if microcytic anemia is due to iron deficiency or thalassemia?

Mentzer index = MCV/RBC
<13 suggests thalassemia
>13 suggests iron deficiency

How well did you know this?

Not at all

Perfectly

What globin chains make up:
HgbA
HgbA2
HgbF
HgbBarts
HgbH
HgbS
HgbSS

A: a2b2. (95-98%)
A2: a2o2 (2-3%)
F: a2y2 (0.8-2%)
Barts: y4 (hydrops fettles, 4 mutations)
H: b4 (3 mutations)
S: b2S2
SS: S4

How well did you know this?

Not at all

Perfectly

What do you see on bloodwork in kids with AIHA? what is the tx?

hemolysis with DAT+
increased retics
increased unconjugated bilirubin
low haptoglobin
increased LDH
urine dip shows free hemoglobin

Tx= steroids 2mg/kg/day and wean over 4-6 weeks

How well did you know this?

Not at all

Perfectly

What are 2 causes of pure red cell aplasia?

Diamond Blackman anemia
Transient erythroblastopenia of childhood (TEC)

How well did you know this?

Not at all

Perfectly

definition of pure red cell aplasia

isolated anemia with VERY LOW RETICS

often post viral (ex. Parvo)

How well did you know this?

Not at all

Perfectly

presentation of TEC

well child b/w 6months-3 yrs, acute reticulopenic anemia, recovery 1-2 months

How well did you know this?

Not at all

Perfectly

presentation of Diamond blackfan anemia

+/- congenital abnormalities
presents less than age 2
ELEVATED ADA
transfusion dependent
risk of malignancy
low retics

How well did you know this?

Not at all

Perfectly

Presentation of beta thalassemia major

Skeletal findings/face
HSM
para-aortic masses
Asian, African, mediterranean
Dx: hemoglobin analysis (No A, have A2 and F)
Tx: transfusion, iron chelation

How well did you know this?

Not at all

Perfectly

what test do they use to diagnoses hemoglobinopathies?

high performance liquid chromatography

How well did you know this?

Not at all

Perfectly

2 congenital causes of thrombocytopenia

wiskott Aldrich (small platelets, eczema, immunodeficiency)
thrombocytopenia/absent radii (TAR)

How well did you know this?

Not at all

Perfectly

Red flag for ITP (aka something else going on) (7)

age <12 months
family history of ITP
congenital anomalies
consanguinity
constitutional symptoms
sig lymphadenopathy +/- HSM
lack of response to first line therapy

How well did you know this?

Not at all

Perfectly

Definition of ITP

platelets <100
newly dignosed <3 months
persistent 3-12 months
chronic >12 months

How well did you know this?

Not at all

Perfectly

What type of platelets do you transfuse in NAIT?

random pooled platelets (fastest)

How well did you know this?

Not at all

Perfectly

in which infants should you automatically check platelets at birth and daily for a few days?

all infants to mothers with known autoimmune disease

How well did you know this?

Not at all

Perfectly

3 types of von willebrand disease

Type1: most common, AD, milder quantitative defect, can treat with DDAVP

Type2: Rare, disorder of VWF function, DDAVP with caution, can make it worse

Type3: Severe, AR, very little/no VWF, act like hemophilia patients (joint and muscle bleeds), no use for DDAVP, needs VWF replacement

How well did you know this?

Not at all

Perfectly

Intrinsic pathway factors (4)

12, 11, 9, 8

How well did you know this?

Not at all

Perfectly

Extrinsic pathway factor (1)

How well did you know this?

Not at all

Perfectly

Common pathway factors (4)

10, 5, 2 (prothrombin), 1(fibrinogen)

How well did you know this?

Not at all

Perfectly

what factor affected in hemophilia A and hemophilia B?

8 in A
9 in B

How well did you know this?

Not at all

Perfectly

cause of early hemorrhagic disease of the newborn

early = in first 24 hours
maternal anticonvulsants or antiTB meds
prevented by giving bit K to mother in late pregnancy

How well did you know this?

Not at all

Perfectly

tx for type III vWF disease

factor VIII/von willebrand’s concentrate

How well did you know this?

Not at all

Perfectly

vitamin K affects which factors

2,7,9,10 therefore intrinsic, extrinsic and common pathways

How well did you know this?

Not at all

Perfectly

What globin chains make up:
HgbA

A: a2b2. (95-98%)

hemoglobin A2 made up of what chains

A2o2 (2-3%)

HgbF

a2gamma2

HgbBarts

y4 (hydrops fetalis)

Hgb H

Beta4 (3 mutations)

HgbS

B2S2

HgbSS

lab abnormalities in TEC

mod-severe anemia MVC normal low retics may see neutropenia platelets may be normal or increased

red flag feature of TEC

if >1 RBC transfusion, think of alternative diagnosis

common oxidants causing G6PD exacerbations

sulfas nitrofurantoin dapsone naphthalene (moth balls) anti-malarials rasburicase fava beans aspirin infections DKA hepatitis

true or false: splenectomy is treatment for G6PD

false --not useful. Tx is supportive care and avoidance of triggers

HgbA > HbS = ?

sickle cell trait

HgbS >> HgbF and no hgbA = ?

sickle cell disease --either SCD-SS or SCD-SBo

HgbS >> Hgb A and Hgb F =?

most likely SCD-SB+

HgbS and HgbC = ?

SCD-SC

what are risk factors for stroke in SCD?

SS genotype low baseline Hb previous TIA high blood pressure rate of and recent episode of acute chest crisis

What is the treatment of stroke prevention in SCD?

chronic blood transfusion to maintain HgbS <30%

treatment of gallstones in SCD?

elective cholecystectomy

3 indications for exchange transfusion in sickle cell disease

stroke severe ACS pre-op for major surgeries

indicators of poor prognosis is SCD

dactylics before age 1 hgb concentration <7g/dL leukocytosis without infection

2 electrolyte abnormalities caused by massive blood transfusion

hyperkalemia hypocalcemia (citrate in blood product chelates calcium ions)

Most common infection from blood transfusion

HepB (1 in 1.1-1.7 million)

causes of elevated PT/INR AND PTT (4)

DIC vit K deficiency liver disease heparin

how to differentiate PT/PTT prolongation from vit K deficiency vs DIC

low platelets in DIC

Does hemophilia A affected PT PTT or both?

PTT, factor 8 deficiency, extrinsic pathway

somebody who doesn't respond to heparin might have what disease?

antithrombin deficiency

_________ is valuable and the diagnostic test of choice to detect a PE

Helical or spiral CT with an intravenous contrast agent is valuable and the diagnostic test of choice to detect a PE

What 3 lab abnormalities might you see in Kasabach-Merritt Sydrome

severe thrombocytopenia, hypofibrinogenemia, elevated D dimer (caused by large hemangioma causing intravascular coagulation with thrombocytopenia and hypofibrinogenemia)

Red flags suggesting alternate diagnosis in ITP (ie. need bone marrow biopsy)

B symptoms bone pain recurrent thrombocytopenia poor treatment response lymphadenopathy HSM child is "unwell" signs of chronic disease low hb (unless mildly low and explained by bleeding) high MCV abnormal WBC/neutrophil Abnormal cellular morphology

classification of severity of ITP and management

Left supraclavicular lymph node due to what?

metastatic malignancy from the abdomen

Right supraclavicular lymph node due to what?

malignancy from the mediastinum

what does presence of megakaryoblasts on peripheral smear indicate?

transient myelproliferative disorder

7 syndromes the predispose to leukemia

Trisomy 21 NF1 Ataxia Telangiectasia Fanconi Anemia Li-Fraumeni Syndrome Bloom syndrome Noonan syndrome

4 syndromes associated with wilm's tumor

WAGR, Denys-Drash, NF1, Beckwith-Wiedemann