Hematology Flashcards
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
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
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
What are 2 causes of pure red cell aplasia?
Diamond Blackman anemia
Transient erythroblastopenia of childhood (TEC)
definition of pure red cell aplasia
isolated anemia with VERY LOW RETICS
often post viral (ex. Parvo)
presentation of TEC
well child b/w 6months-3 yrs, acute reticulopenic anemia, recovery 1-2 months
presentation of Diamond blackfan anemia
+/- congenital abnormalities
presents less than age 2
ELEVATED ADA
transfusion dependent
risk of malignancy
low retics
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
what test do they use to diagnoses hemoglobinopathies?
high performance liquid chromatography
2 congenital causes of thrombocytopenia
wiskott Aldrich (small platelets, eczema, immunodeficiency)
thrombocytopenia/absent radii (TAR)
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
Definition of ITP
platelets <100
newly dignosed <3 months
persistent 3-12 months
chronic >12 months
What type of platelets do you transfuse in NAIT?
random pooled platelets (fastest)
in which infants should you automatically check platelets at birth and daily for a few days?
all infants to mothers with known autoimmune disease
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
Intrinsic pathway factors (4)
12, 11, 9, 8
Extrinsic pathway factor (1)
7
Common pathway factors (4)
10, 5, 2 (prothrombin), 1(fibrinogen)
what factor affected in hemophilia A and hemophilia B?
8 in A
9 in B
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
tx for type III vWF disease
factor VIII/von willebrand’s concentrate
vitamin K affects which factors
2,7,9,10 therefore intrinsic, extrinsic and common pathways
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
S4
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»_space; HgbF and no hgbA = ?
sickle cell disease –either SCD-SS or SCD-SBo
HgbS»_space; 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
