Hematology Flashcards
When do consider partial exchange transfusion for polycythemic newborns?
Hct > 70%
Or lower w/ symp.
If you are giving a partial exchange transfusion- what fluid as diluent?
Normal Saline
How much blood do you give to decrease Hct from 0.75 to 0.5 in 2kg BB?
BB Blood vol= 80 mL x kg
Volume exchange (mL)
= Blood vol x (observed-desired Hct)/ Observed Hct
= (80x2) x (0.75-.05)/0.75
= 53 mL
Or generally 10-20 cc/kg.
What are suggested Transfusion level for infants with anemia of prem?
*start at 115 w/ resp support and minus 15 if none= 100
For each wk start w/ last no resp support (100 for wk 2) threshold for resp support and minus 15.
> Wk 1: Resp support= 115; No resp= 100
> Wk 2= 100 w/ resp support; 85 if not
> Wk 3= 85 or 75 no resp
List 3 AE to RBC transfusion:
> AE of leukocyte
- anaphylaxis
- graft vs. host
- TRALI (transfusion related lung injury)
> Acute volume or lyte
- TACO (transfusion related circulatory overload)
> transfusion infection
- virus, bacterial, parasite
> blood group in compatible
- aute hemolytic rxn
Which blood group do you give neo in ED?
Group O Rh (-)
T or F: you try to maintain Hgb of 75 in prem.
True; regardless of age.
T or F: infants with resp or cyanotic heart dx may need higher transfusion threshold.
True
Most common RBC transfusion rxn?
- Febrile non hemolytic rxn
- Allergic rxn
- TACO (circulatory overload)
Most common cause of febrile rxn in blood transfusion?
Sensitization to WBC
> due to presence of cytokine made by passenger leuk
NOT hemolysis.
Child getting blood. Become febrile and chills. Management?
- stop it; run TKVO
- stop it; steroids
- continue at slow rate
- continue w/ methylpred
Stop it and run TKVO
+/- antipyretic if fever bothersome
+/- ensure no other cause (hemolytic rxn, sepsis)
Which infection is most at risk to get from pRBC?
- Hep B
- Hep C
- HIV
- Parvo B19
Parvo B19
HIV: 1 in 8 million
Hep C: 1 in 5 million
Hep B: 1 in 1 million
Parvo: 1 in 20 K
Child with that major thal and recurrent transfusion. On desfuroxime. Most serious complication?
Cardiac hemosiderosis
** transfusion induced
Fe deposited; liver, endo, cardiac after 10 yr
How are thalassemias inherited?
AR
What are Beta thal minor (trait), intermedia, major.
Beta Thal= beta lacking.
Minor/Trait= 1 abN allele
B/B+, B/B0
= Silent; No Tx
Beta Intermedia
B+/B+, B+/B0
= two abnormal allele
may need transfusion
Beta Major= Cooley
= homozygous
B0__/B0__
> CC: 6-12 mon. when HbA replace HbF= anemia, jaundice, DD, HSM
Tx: transfusion, iron chelation, bone marrow transplant
What Hgb do you find in beta thal major patients?
Hb F
0-10% HbA only
What can you expect on beta thal major BW?
- anemia
BUT low retic (unstable erythroid precursor die) - ++ HbF
Which ethnicity is beta thal dominant in?
Mediterranean
Indian
What ethnicity is alpha-thal dominant in?
S.E Asia
Africa
Describe the 4 types of alpha Thal
aa/-a= 1 bad gene= silent
- a/-a= 2 bad gene= trait
- a/–= Hb H= 3 bad gene
–/– = Hb Barts/ hydrops fetalis
What do you see with HbH:
- inclusion body
- heinz body
- howell-jolly body
Inclusion body
(Hgb= beta 4 because three alpha missing= seen in cells)
Heinz Body= G6PD
Howell-Jolly= asplenia (sickle cell), amyloid, hemolytic anemia, prem infant
How do you a dx a Thalassemia?
Hemoglobin electrophoresis
(i.e. HbA2 and HbF up= beta chain missing)
Confirmation: Gene mapping
What type of treatment is required for Hb Barts?
Intrauterine transfusion
as no alpha gene
= fetal hydrops otherwise
What is gold standard for beta thal major?
Transfusion
+ Hydroxyurea (increase Hb F)
+/- hematopoietic stem cell transplant curative
What do you need to be aware of with beta thal major transfusions?
Keep Hgb > 90.
Transfuse q4wk. 4 life.
Watch for Fe overload (screening serum ferritin and MRI)
Watch liver, endo, cardiac deposits
Start Iron cheating as soon as overload seen (deferoxamine)
T or F: hematopoietic stem cell is CURATIVE for beta thal major?
True
Girl with microcytic anemia. Ferritin and iron normal. Next test? DDX?
Hgb electrophoresis
Microcytic Anemia
- Iron deficiency
- Thalassemia (AR dx)
- Sideroblastic (granular deposit Fe in mitochondria; high transferrin, ferritin, Fe)
- Lead intoxication (old paint)
- Anemia of chronic dx
What is the Mentzer Index?
MCV/ RBC count
> 13= Fe deficiency
< 13= beta thal likely
What is the BW for thal?
Microcytic anemia
High RBC
Low or normal retic
Mentzer (MCV/RBC) < 13
Normocytic anemia ddx.
Retic high= Hemolysis
Bleeding
Hypersplenism
In the RBC
Hemoglobin….
>Thalassemia
> Sickle Cell
Enzyme….
> G6PD
> Pyruvate Kinase Deficiency
Memb….
> Hereditary spherocytosis
> Eliptocytosis
Outside RBC (antibody) > Immune = Coomb + - AI hemolytic anemia
> Non immune= Coom (-)
- HUS (uremic syn)
- TTP (thrombotic thrombocytopenia purpura)
- DIC (disseminated intravascular coag)
- Hemangioma (Kasabach-Merrit syn)
Retic low/normal
- Acute bleed
- Anemia of chronic dx (RA, SLE, TB, malignancy)
- Low cell lines= leukaemia, aplastic anemia
- High Lines= infection
- Mixed WBC + Plt= RF, meds
- Normal WBC + Plt= TEC, early Fe deficiency
What is TEC anemia?
Transient erythroblastopenia of childhood
- temp cessation in RBC production
- healthy
- normocytic
- low retic
- usually 1 mo-6 year
- last 1-2 month
- recover
- typically bone marrow as dx of exclusion (r/o bone marrow infiltrative dx)
2 yo. well. Pallor. Hgb 80. Normocytic. Low Retic. Next investigation:
- Bone marrow aspirate
- Hgb electrophoresis
- Coombs
- Serum ferritin
Bone marrow aspirate (or ferritin?)
Even if TEC need to R/O bone marrow infiltrative like leukaemia.
DDX:
- Acute bleed
- Anemia of chronic dx (RA, SLE, TB, malignancy)
- Low WBC + Plt= leuk, aplastic anemia
- High WBC + Plt= infection
- Mixed WBC + Plt= RF, meds
- Normal WBC + Plt= TEC, early Fe deficiency
list three features on CBC consistent with Iron deficiency:
- Microcytic hypo chromic **anemia (Hgb, MCV low)
- Low ferritin (low iron stores)
- Serum iron low
- Increased TIBC and low transferrin
- **RDW increased
- ** RBC low
- Retic normal or moderately up
- **WBC normal
- +/- Plt up
Low energy. Pallor. Viral illness 1-2 wk ago. Sclera yellow. Hgb 70. Norm WBC. Norm Plt. Retic 24%. Type of anemia? Test to confirm? Tx?
Hemolytic normocytic anemia.
Likely: Acute idiopathic autoimmune hemolytic anemia
Test to confirm: DAT
Other: Smear (spherocytosis etc.)
Tx: Prednisone and IVIG
(If Hgb < 100 and Retic > 10%)
What is fanconi Anemia?
AR or X linked Most common inherited bone marrow failure (aplastic anemia sun) Low IQ Small eye, abN ear CAM, hypo pigmented, Absent or abN thumb
List long-term consequence of fanconi anemia?
Pancytopenia
AML
Squamous cell carcinoma of head and neck
Aplastic Anemia + Hypopigmented skin or CAM + abnormal Thumbs
Fanconi Anemia
Note: aplastic anemia= bone marrow aplasia= pancytopenia.
- Fanconi one congenital cause
- Shwachman-Diamond (pancreatic, bone marrow, skeletal)
All are features of iron deficiency anemia EXCEPT:
- pica
- koilonychia
- cheilosis
- mild scleral icterus
- psychomotor retardation
Icterus= hemolysis + jaundice.
CC:
- pallor of palm, creases, conjunctive
- skull bosing
- angular cheilitis
- koilonychia (spoon nail)
- pica, pagophagia (ice)
- irritable, anorexia
- tachy HR and high CO
- cognitive defect
What is the progression of Iron deficiency anemia BW?
Reduced serum ferritin (iron storage protein)
Serum iron down
Binding capacity of serum (serum transferrin) UP
Transferrin sat DROP
Not enough iron for heme= Anemia
Less Hgb in each= Microcytosis
Variation in cell size= RDW
Decrease in MCV
Blood smear of iron deficiency of anemia show all BUT:
- Heinz body
- Poikilocytes
- Microcytosis
- Hypochromasia
- Normal or low retic
Heinz body (inclusion in RBC due to damaged Hgb i.e. G6PD)
- normal/high retic but low when iron substrate for RBC completely gone
- poikilocyte (general term for abnormal shape RBC= can see elliptocytes/cigar shape)
List 3 DDX on micro anemia that fail to respond to oral iron
- **poor compliance
- **poor dose
- **malabsorption of administered iron (taking with milk)
- ongoing blood loss
- B12 or folate deficiency
- **other: thal, anemia of chronic dx, lead poisoning etc.
Kid with high intake milk. CBC show normocytic anemia with Hgb AS (sickle trait). Likely dx:
- TEC
- Iron deficiency
- sickle cell dx
TEC
Transient erythroblastopenia of childhood
Full term. Mom O(+). Well but pale. Hgb 70. Stable. Likely dx:
- ABO incompatible
- Chronic fetal maternal hemm
- RH incompability
ABO incompatible
Term BB. Midwife. Present at 7d. Hgb 70. MCV 112. Plt normal. Likely dx?
Vitamin K deficiency leading to hemorrhagic dx of newborn.
List 4 ppt of neonatal hemophilia:
- *1. bleed w/ circumcision
- *2. Prolonged bleeding with BW (heel stick or venipuncture)
3. Cephalohematoma - *4. Subgaleal Hemorrhage
- *5. Intracranial hemorrhage