Hematology Flashcards
1
Q
When do consider partial exchange transfusion for polycythemic newborns?
A
Hct > 70%
Or lower w/ symp.
2
Q
If you are giving a partial exchange transfusion- what fluid as diluent?
A
Normal Saline
3
Q
How much blood do you give to decrease Hct from 0.75 to 0.5 in 2kg BB?
A
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.
4
Q
What are suggested Transfusion level for infants with anemia of prem?
A
*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
5
Q
List 3 AE to RBC transfusion:
A
> 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
6
Q
Which blood group do you give neo in ED?
A
Group O Rh (-)
7
Q
T or F: you try to maintain Hgb of 75 in prem.
A
True; regardless of age.
8
Q
T or F: infants with resp or cyanotic heart dx may need higher transfusion threshold.
A
True
9
Q
Most common RBC transfusion rxn?
A
- Febrile non hemolytic rxn
- Allergic rxn
- TACO (circulatory overload)
10
Q
Most common cause of febrile rxn in blood transfusion?
A
Sensitization to WBC
> due to presence of cytokine made by passenger leuk
NOT hemolysis.
11
Q
Child getting blood. Become febrile and chills. Management?
- stop it; run TKVO
- stop it; steroids
- continue at slow rate
- continue w/ methylpred
A
Stop it and run TKVO
+/- antipyretic if fever bothersome
+/- ensure no other cause (hemolytic rxn, sepsis)
12
Q
Which infection is most at risk to get from pRBC?
- Hep B
- Hep C
- HIV
- Parvo B19
A
Parvo B19
HIV: 1 in 8 million
Hep C: 1 in 5 million
Hep B: 1 in 1 million
Parvo: 1 in 20 K
13
Q
Child with that major thal and recurrent transfusion. On desfuroxime. Most serious complication?
A
Cardiac hemosiderosis
** transfusion induced
Fe deposited; liver, endo, cardiac after 10 yr
14
Q
How are thalassemias inherited?
A
AR
15
Q
What are Beta thal minor (trait), intermedia, major.
A
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
16
Q
What Hgb do you find in beta thal major patients?
A
Hb F
0-10% HbA only
17
Q
What can you expect on beta thal major BW?
A
- anemia
BUT low retic (unstable erythroid precursor die) - ++ HbF
18
Q
Which ethnicity is beta thal dominant in?
A
Mediterranean
Indian
19
Q
What ethnicity is alpha-thal dominant in?
A
S.E Asia
Africa
20
Q
Describe the 4 types of alpha Thal
A
aa/-a= 1 bad gene= silent
- a/-a= 2 bad gene= trait
- a/–= Hb H= 3 bad gene
–/– = Hb Barts/ hydrops fetalis
21
Q
What do you see with HbH:
- inclusion body
- heinz body
- howell-jolly body
A
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
22
Q
How do you a dx a Thalassemia?
A
Hemoglobin electrophoresis
(i.e. HbA2 and HbF up= beta chain missing)
Confirmation: Gene mapping
23
Q
What type of treatment is required for Hb Barts?
A
Intrauterine transfusion
as no alpha gene
= fetal hydrops otherwise
24
Q
What is gold standard for beta thal major?
A
Transfusion
+ Hydroxyurea (increase Hb F)
+/- hematopoietic stem cell transplant curative
25
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)
26
T or F: hematopoietic stem cell is CURATIVE for beta thal major?
True
27
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
28
What is the Mentzer Index?
MCV/ RBC count
> 13= Fe deficiency
< 13= beta thal likely
29
What is the BW for thal?
Microcytic anemia
High RBC
Low or normal retic
Mentzer (MCV/RBC) < 13
30
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
31
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)
32
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
33
list three features on CBC consistent with Iron deficiency:
1. Microcytic hypo chromic **anemia (Hgb, MCV low)
2. Low ferritin (low iron stores)
3. Serum iron low
4. Increased TIBC and low transferrin
5. **RDW increased
6. ** RBC low
7. Retic normal or moderately up
8. **WBC normal
9. +/- Plt up
34
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%)
35
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
```
36
List long-term consequence of fanconi anemia?
Pancytopenia
AML
Squamous cell carcinoma of head and neck
37
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)
38
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
39
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
40
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)
41
List 3 DDX on micro anemia that fail to respond to oral iron
1. **poor compliance
2. **poor dose
3. **malabsorption of administered iron (taking with milk)
4. ongoing blood loss
5. B12 or folate deficiency
6. **other: thal, anemia of chronic dx, lead poisoning etc.
42
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
43
Full term. Mom O(+). Well but pale. Hgb 70. Stable. Likely dx:
- ABO incompatible
- Chronic fetal maternal hemm
- RH incompability
ABO incompatible
44
Term BB. Midwife. Present at 7d. Hgb 70. MCV 112. Plt normal. Likely dx?
Vitamin K deficiency leading to hemorrhagic dx of newborn.
45
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
46
What is Hemophilia A and B. Inheritance?
```
Hemophilia A= Factor 8
Hemophilia B= Factor 9
X chromosome (*M)
```
= low # or defective
= impaired secondary hemostasis
47
Which BW is abnormal in hemophilia:
- Plt
- Bleeding Time
- INR
- aPTT
- specific assay for Factor 8 and 9
PTT= UP
AND
Specific assay Fact 8+/- 9
48
Who do you treat Hemophilia acutely and prophylaxis?
Acute bleeding= fresh frozen plasma (clotting factor) + Cryoprecipitate (factor 8C, vWF, factor 13)
Known: factor 8 or 9 concentrate
Prophylaxis infusions only if severe dx
*No ASA + NSAID
49
4 etiology for non hemorrhagic (clot) stroke
Congenital heart dx
Inherited prothrombotic
> factor 5 leiden
> prothombin gene mutation
Acquired prothrombotic
> Protein C or S deficiency
> Antithrombin 3 deficiency
```
Other older:
> Sickle cell anemia
> Antiphospholipid antibodies
> OCP
> Pregnancy
```
50
What is one serious complication of severe ITP:
CNS intracranial bleed
51
What is an indication for bone marrow bx in child with ITP w/ plt 12.
- ANC < 1
- Previous steroid
- Hgb < 115
- Fever > 39
ANC < 1
Bx if R/O malignancy, bone marrow failure.
Steroid not indication for bx.
52
Term baby with petechiae and bruising. Plt 12; CBC otherwise normal. Given plt and repeat 16. Mom plt 80. Best tx:
- PLA-1 negative plt
- IVIG
- Washed maternal plt
IVIG
Reminder: PLA-1 negative plt NAIT and mom plt then normal.
53
In which is plt transfusion indicated:
- aplastic anemia
- acute ITP
- chronic ITP
- HUS
- ITP due to quinidine toxicity
Aplastic anemia
| acute ITP only if intracranial bleed
54
When do you infuse plt
1. Overt bleeding and < 50
2. Major procedure < 50
3. Minor (LP, IV) <25
4. Bone marrow failure and high risk (infection, organ failure, clot, mucosal bleeding) + < 20
5. Bone marrow failure + low risk + < 10
55
List 3 causes of aplastic anemia:
= bone marrow failure = pancytopenia
1. Fanconi anemia
2. Shwachman-Diamond Syn
3. Sickle cell infected by parvovirus B19
56
What is ITP?
Immune destruction of normal plt (usually < 100)
| *autoantibody against plt membrane antigen
57
T or F: ITP is the most common acute cause of thrombocytopenia in well kids.
True.
58
T or F: young child with ITP more likely to have chronic ITP or systemic cause (like SLE).
False.
Teens with insidious onset likely to have chronic ITP or systemic dx.
Classic age: 1-4 y.o.
59
Sudden bruising or petechiae in healthy child after viral illness or MMR vaccine. Likely dx?
ITP
| = Idiopathic thrombocytopenia purpura
60
T or F: You can see schistocytes in ITP.
False.
Plt cleared by reticuloendothelial system (spleen, liver).
If RBC break down= hemolysis= HUS, TTP, DIC
61
T or F: Most acute ITP resolve on its own.
True.
70-80% within 3 mo.
```
No therapy
Educate
Counsel if mild/mod dx
- restricted contact sport if < 30
- d/c NSAID, aspirin
- careful w/ head trauma
```
62
T or F: In acute ITP pharma decrease chance of serious bleed or chronic ITP
False.
Note: Chronic ITP= 12 mon.
63
When do you give plt transfusion in ITP?
Life-threatening bleed
i.e. intracranial hemorrhage
> plt, IVIG, high dose steroids (methylpred), prompt consult to neuroSx and Sx
64
T or F: further testing should be done to R/O secondary cause of ITP if chronic (min. 12 month).
True
- Infection (HIV, hep C, CMV, EBV)
- Bone marrow aspirate (aplastic anemia, bone marrow failure, CA)
- AI (ANA, dsDNA)
- Immunodeficiency (immunoglobulin level)
65
Microangiopathic hemolytic anemia + thrombocytopenia + Acute kidney injury. E coli toxin etiology.
HUS
Hemolytic uremic syndrome
CC: abo pain, vomiting, diarrhea
66
Which bacteria strain is most classic for HUS?
E coli O157:H7.
67
T or F: PT test also called INR
True
68
```
List typical pattern for Hemophilia vs. vWD vs. Vitamin K deficient vs. DIC.
_INR (PT)
_ aPTT
_ Bleeding Time
_ Other
```
Hemophilia
** aPTT HIGH. Rest Normal
vWD
**Normal INR/PT.
Rest N/high.
Vitamin K:
**INR/PT HIGH
** apTT HIGH
Normal plt + fibrinogen
DIC
*** INR and apTT high but plt + fibrinogen down
D-dimer UP
69
T or F: you can use intranasal desmopressin for mild hemophilia A (factor 8)?
True
Otherwise factor concentrate, or FFP + cryoprecipitate if acute bleeding dx
70
vWD patient with bleeding. Best treatment:
- FFP
- DDAVP
- Cryoprecipitate
- Plt
DDAVP
Type 1 (# issue) tx with this.
If type 2 or 3= vWF concentrate (plasma derived factor 8 concentrate have vWF, or recombinant vWF)
Cryoprecipitate otherwise reasonable if none above.
71
Most consistent with DIC:
- fibrinogen ++
- PTT ++
- PT/INR down
- Low fibrin split product
PTT +++
DIC: inflammatory response + innate immune response + coat = DYSFN
= consume clotting factors, plt = tissue ischemia + hemolytic anemia.
ABC
+/- pRBC, plt, cryo (fibrinogen, vWF, factor 8C), FPP (replace coag factors + natural inhibitors)
72
Most common inherited bleeding dx?
vWF
73
15 y.o. with menorrhagia + epistaxis. Check for:
- vWF
- hemophilia C
- factor 5 leiden mutation
vWF
Hemophilia C = deep bleeding
Factor 5 Leiden= clots
74
Immunization with kid with hemophilia A:
- defer
- give Factor 8
- pressure over site x 10 min. post-immunization
Pressure x 10 min.
+ clotting factor concentrate if needed
75
List 5 causes of neutropenia
1. Bone marrow suppression= ***post viral infectious, ***drug induced (pen, anticonvulsant), *** Chemo
2. Bone marrow injured= aplastic anaemia (Fanconi), B12 and folate deficiency, ***Malignancy (leuk, lymph, mets)
3. Destroyed= ***Autoimmune (SLE)
4. Sequestered= ***Hypersplenism
```
Full DDX:
....ACQUIRED
> Malignancy
> Infections
- viral (EBV, RSV, Flu etc.)
- Bacterial sepsis
> Autoimm
- primary
- secondary (SLE, IBD, RA, Sjogren)
> Drugs
- Chemo
- antipsychotic, anticonvulsant, antidepressant, Abx (pen, cephalo, vanco)
- gold, antithyroid med destroy neutro
```
Other
> Hypersplenism (sequester)
> Nutritional (vit B12, folic acid def)
... CONGENITAL
> Cyclic neutropenia
> Kostmann Syn (recurrent bacterial infection)
> Shwachman-Diamond (pancreas, metaphyseal dysostosis, neutropenia)
> Bone marrow aplasia (Fanconi, Blackfan-Diamond)
> TAR (lot plt, absent radii)
76
DDX for neonatal thrombocytopenia:
1. Sepsis and DIC, NEC
2. TORCH infection
3. NAIT (mom (-) plt antigen but dad (+))
4. Maternal ITP
5. Genetics (TAR syndrome, Fanconi anemia)
6. Thrombosis
7. IUGR, placental insufficiency
8. Kasabach-Merritt syn
77
What is NAIT?
Neonatal ALLO-immune thrombocytopenia
- mom plt antigen (-)
- dad plt antigen (+); BB (+)
- mom destroy BB plt
can occur in 1st preg
- fall < 50 in days; rise 1-4 wk as antibody decline
- R/O IVH via HUS
- Tx: plt if < 30 (or <50 if prem)
HPA (-) plt ( AKA PLA-1 neg)
Option= IVIG.
Next pregnancy counselling KEY! Wkly IVIG in preg if BB (+) in next preg.
78
What is neonatal autoimmune thrombocytopenia?
No severe low plt
Maternal ITP, SLE, Graves etc.
Mom antibody pass to body.
Recheck in 1 week
Spontaneous resolution
79
Newborn w/ plt 12. Transfused plt and still 16. Mom CBC normal. What do you do?
- Transfuse single donor plt
- transfuse PLA-1 negative (HPA-1)
- IVIG
- Steroids
Transfuse PLA-1 negative
| HPA-1 for NAIT
80
T or F: Neonatal ALLoimmune 1st line is steroids and IVIG.
False.
```
Monitor
Transfuse if < 30
Either...
- washed maternal plt
- cross match for mom's plt
- PLA-1 negative
- random plt
```
81
4 indications for bone marrow aspiration in setting of likely ITP:
1. Abnormal WBC/diff (i.e. neutropenia)
2. Unexplained anemia
3. Hepatosplenomegaly or lymphadenopathy
4. Bone or joint pain
82
T or F: Splenectomy can sometimes be used for ITP
yes.
| If > 4 and > 1 yr without response to tx w/ severe ITP
83
14 y.o. with sickle and dacytlitis. Acute management. Prevention med?
Dactylitis= hand-foot
Pain: tylenol, NSAID, opioids
Hydration: PO or iV
Prevention: Hydroxyurea
84
T or F: hydroxyurea reduce frequency of crisis and acute chest crisis?
True
85
List 4 indications for blood transfusion in sickle cell
Acute Chest Syndrome
Aplastic Crisis
Splenic
Acute stroke
86
Mom healthy. Her father has hemophilia. Risk of transmitting to own child:
- none
- 50% son will have and all daughters carrier
- 50% son will have and 50% daughters carriers
Hemophilia A + B= X linked recessive
```
Maternal GF= XY= affected
Mom= carrier
- Her kid's
50% boy will get X= affected
50% of F will get X= carrier
```
87
Most common complication of beta thal major:
- iron overload
- megaloblastic anemia
- cardiomyopathy
- poor hematopoiesis
Iron overload
88
Sickle. Cough. Tachypnea. R/O which complication
Acute Chest Crisis
89
Sickle cell w/ recurrent chest crisis. What are they at increased risk of?
Recurrent cerebral infarct/ Stroke
| PPT: neurocognitive and behavioural deficits
90
Most common complication of hereditary spherocytosis
gallstones
91
What is hereditary spherocytosis?
- AD
- most common RBC memb abN
- North Europe esp
- asymp -> hemolysis
- gallstone common
- rarely splenic crisis, gout, cardiomyopathy
- spherocyte on smear
- U/S gallstones
- monitor growth, exercise tolerance, spleen size, anticipatory guidance for parvovirus aplastic crisis
92
Severity of Rh incompatibility at birth predicted by:
- cord Hgb
- cord bili
- prematurity
- splenomegaly
- maternal anti-D antibody
Cord Hgb
Note: cord bili can be high and expect rapid raise in bili in first 6hr
Note: high or rapid rise in IgG titre to D antigen suggest hemolytic dx
93
Do IgM or IgG antibodies cross placenta?
IgG (GOOD)
94
Sickle cell. Obese. RUQ pain. LFT up. Recommendation:
- Cholecystectomy
- Low fat diet
- Ursodiol
Cholecystectomy for recurrent chole and gallstones.
High risk of black pigment stones and unconjug. bill. Obesity also RF.
95
What tx proven to prevent stroke in sickle cell:
- Transfusion
- Folate
- Hydroxyurea
- Iron
Transfusion
96
T or F: sickle cell associated strokes are usually subclinical.
True.
Hydroxyurea: reduce painful episodes, ACS and need for transfusions
97
What is sickle cell disease?
AR
HbSS
RF: African, Caribbean, Mediterranean etc.
Deoxygenated = distort into sickle shape = stuck
1st ppt= dactylitis common
98
What is sickle cell prevention protocol?
Pens till min. 5 y.o. (pneumococcal infection)
Folic acid till 5 y.o.
Hydroxyurea after that (increase HbF)
Vaccines + pneumo 23 (at 2 and 5 y.o.) and yearly influenza
99
How do you manage acute stroke in sickle cell?
Apply O2 to keep sat > 96%
pRBC till 100g/L
Exchange to keep HbS < 30%
Peds Neuro
100
What organisms are kids with Sickle at risk for?
Encapsulated:
"YES, Some Killers Have Pretty Nice Capsules"
Yersinia Petis
E coli. (some species)
Strep agalactiae (GBS)
```
Strep pneumoniae
Klebsiella
Haemophilus influnzae
Pseudomonas Aeuroginosa
Neisseria Meningitidis
Cryptococcus Neoformans
```
101
If febrile sickle cell:
> 38.4
= Blood Culture
= IV Abx (ceftriaxone)
= +/- Admit min. 24 h
102
How do you define chest crisis
Fever
New CXR infiltrate
+/- hypoxia/WOB/pain
- 1X IVF
- blood
- O2
- cephalosporin + macrolide
103
List 3 indications for splenectomy:
```
Splenic trauma
Hereditary spherocytosis
Primary hypersplenism
Chronic ITP resistant to tx
Splenic abscess
Sickle Cell Disease w/ recurrent aplastic sequestration
```
104
Teen with B symptoms. Supraclavicular node. Tx:
- AUS
- CXR
- CT chest
- Bone marrow aspirate
CXR
R/O mediastinal mass
105
Name on virus associated with lymphoma
EBV
CMV
HHV-6
106
Node in superclavicular area. Most helpful test:
- Bx
- Bartonella
- Monospot/EBV
- TB skin test
bx
107
List 5 things on ddx for splenomegaly:
1. Structural
- congenital splenic hemangioma
- hamartoma
- cyst
2. *Splenic trauma/ hemm
3. Infectious
* EBV
* Bacterial (typhoid, cat scratch, TB)
* Malaria
4. Heme*
- spherocytosis, G6PD, pyruvate kinase deficiency
- sickle cell sequestration
- thalassemia, osteoporosis
5. *Blood/portal HTN
- HF
- portal HTN
- cirrhosis
6. Storage dx
- Gaucher dx, Niemann-Pick
7. Systemic
- ** Malignancy (leuk, hodgkin)
- Grandulomatous
- Sarcoid
- Amyloid
- SLE, RA
108
VERY white bones on XR. Pale. Hepatosplenoegamly. Likely Dx?
Osteopetrosis
- AR
- +++ Ca2+ bones
- RTA
- anemia, pancytopenia
- compression of cranial nerves possible of skull sclerosed
- no tx.
109
BB with abdo mass. Low Plt. LLSB murmur and murmur over liver. US show cm mass. Next?
- DIC
- Bone marrow
- Pul Imaging
- Liver F'n
DIC
R/O Kasabach-Merritt Syn
110
Child fell off bike. Right knee. Large yellow bruise on chest with R knee swelling.
- Hemophilia
- vWF
- Thrombocytopenia
- Child abuse
- Herbal remedies
Hemophilia
111
4 y.o. new refugee. H/a, dizzy, vague abdo pain and constipated. Short. Microcytic anemia with stifling. Next?
- bone marrow aspirate + bx
- lead level
- Hgb electrophoresis
- iron and ferritin level
Lead level
112
Which is NOT a serious side effect of IVIG:
- acute pancreatitis
- aseptic meningitis
- hemolytic anemia
- transfusion related acute lung injury
- thromboembolism
NOT: acute pancreatitis
113
What do you expect on Hgb electrophoresis for alpha and beta thalassemia?
Alpha Thal
- Normal in adults w/ trait
- Newborn: +/- HbH or Barts
Suspect when: low MCV, normal iron, Normal HPLC/ electrophoresis
Beta Thal:
- Absent or Down HbA
- UP HbA2
- +/- HbF UP
