Heme/Onc - 2019 Updated! Flashcards
Scenario of a teen with cancer who family now decides to make him palliative. But his 10 yr old brother, with whom they were quite close as brothers, starts ignoring him, and spending not as much time with him. His parents are
concerned. What do you tell them? (1 point)
- it is normal for family members including siblings to experience anxiety, depression, guilt, fear and anger, which might make them pull away
- include in discussions about his brother’s care
- sibs who know about and are involved in the care of their dying sib cope better before and after their death
Sickle cell girl who is obese, RUQ pain, increased liver enzymes, had been treated and recovers from the event. What do you recommend?
a. cholescystectomy
b. low-fat diet
c. ursodiol
a. Cholecystectomy
- Child with Down syndrome. List three associated hematological disorders.
ALL AML Transient Myeloproliferative disorder Polycythemia Leukopenia Macrocytosis Thrombocytosis
Left supraclavicular LN - mgmt?
a. Excisional biopsy
b. monitor
c. EBV serology
a. Excisional biopsy
Management of Acute Chest Syndrome
- Supplemental Oxygen
- Blood Transfusion
- IV ABx: Cefotax and Azithro
- Pain managment
- Bronchodilators + steroids for concomitant asthma
- Incentive spirometry
A girl presents with pallor and fatigue after a viral illness. Her laboratory findings are as follows: Hb 60, WBC 7, Platelets 250, Reticulocytes 359.
1) What is the most likely case of her anemia?
2) What 1 investigation will you do?
3) The blood bank calls to inform you that her plasma reacted to all tested cells. She has now clinically deteriorated and is hypotensive, tachycardic, and less responsive. What will you do with regards to transfusion?
1) Autoimmune hemolytic anemia
2) DAT
3) Transfuse O negative until you find our her blood group and DAT
Newborn has a flank mass. What is the most likely cause:
Wilms tumour
Hydronephrosis
Polycystic kidney
Hydronephrosis
Distinguishing features of congenital red cell aplasia (Diamond Blackfan Anemia) from Transient Erythobastopenia of Childhood
Both - Normochromic, Reticulocytopenia
TEC:
- Usually presents >12 mos (6mos-3years)
- Often follows a virus
- RBC adenosine deaminase (ADA) level is NORMAL
- Normocytic
DBA: - Presents early infancy (2-6mos) -Macrocytic - ADA is abnormal - Other features like craniofacial abs, thumb problems, GU anomalies, Cardiac (ASD, VSD, Coarct), Glaucoma, Cataracts, Short.
A 6-year-old Chinese boy fell off his bike 24 hours ago. He is brought into the ER with right knee swelling and a large yellow bruise on his anterior chest wall. You worry about:
a) hemophilia
b) von Willebrand’s
c) thrombocytopenia
d) child abuse
e) herbal remedies
e) herbal remedies
Super bad ?racist question. But he’s a little old for a first presentation of hemophilia - but could also consider this as an answer.
A thorough history of medication use, including herbal medicines (eg, ginger, feverfew, ginkgo biloba, large amounts of garlic) is crucial. In particular, specific information should be sought about the ingestion of aspirin or NSAIDs.
Which condition is most associated with childhood leukemia?
A. Electromagnetic field exposure during pregnancy
B. NF 1
C. Mom or dad with hx of leukemia
B. NF 1
Standard Risk ALL
Age 1-10
Initial leuks <50
Polycythemic newborn. Hb 240, Hct 0.75. Wt 2000g.
Child requires a partial exchange transfusion.
What fluid do you use as the diluent?
How much blood to you replace to decrease the Hct to 0.5?
- NS
- volume to be exchanged (ml) =
blood volume x (observed-desired hematocrit)/ observed hematocrit
- blood volume = 85ml/kg = 170ml in this case
- therefore tfn volume = 170 x (0.75-0.5)/0.75
= 56.6ml
A VWD patient presents with bleeding. Which is the best treatment:
a. FFP
b. DDAVP
c. Cryoprecipitate
d. Platelets
b. DDAVP
Type 1 vWF (most common) - quantity issue - treated with desmopressin, increases amt of circulating VWF by releasing for storage.
Type 2 - quality issue - treated with vWF containing concentrate
Type 3 - severe quantity issue - treated with vWF containing concentrate
*cryo is best for low fibrinogen but can be used in a pinch for vWD, hemophilia A or B if specific factor concentrate is not available
Description of newborn with petechiae. WBC normal, Hgb normal, plt 9. Rest of exam is normal. List 3 most likely causes aside from sepsis.
- neonatal alloimmune thrombocytopenia
- autoimmune thrombocytopenia (maternal ITP)
- TORCH infection (rubella, toxo, EBV, syphilis)
- Kassabach Merritt
- Renal Vein Thrombosis
- Indwelling catheter
- Type 2B vWB
- Hypersplenism
- Wiskott Aldrich
3 yo with pallor, leg pain and fatigue for three weeks. Exam reveals tired, pale anicteric child with mild hepatosplenomegaly. No effusions or warmth/erythema
of legs. Most likely to confirm dx?
a. Abdominal ultrasound
b. Bone marrow aspirate
b. Bone marrow aspirate
Sounds like ALL
28wk prem now 9wks of age. Mom breast feeding. Do they need iron supplementation and why?
Yes
Prems and LBW infants have smaller fetal iron stores and more rapid growth than term infants, anemia develops earlier
Start when BW doubled at ~4weeks (2-4mg/kg/day)
A child with known hemophilia comes for routine immunizations. What would you advise with immunizations?
a. Delay them
b. give Factor VIII before immunizations
c apply pressure for 10 minutes
c. apply pressure for 10 minutes (and use small needle)
b. give Factor VIII before immunizations (if they are severe)
You are counseling a mother and her young daughter with sickle cell anemia who requires a PRBC transfusion. Which of the following infections is she most at risk of acquiring from a transfusion:
a. Hep B
b. Hep C
c. Parvovirus B19
d. HIV
c. Parvovirus B19
- Hep B: 1 in 1.1-1.7 million
- Hep C: 1 in 5-7 million
- HIV: 1 in 8-12 million
- Parvovirus: 1 in 5000 to 1 in 20000
Most common transfusion related infection is yersinia enterocolitica
All are features of iron deficiency anemia EXCEPT:
a) pica
b) koilonychia
c) cheilosis
d) mild scleral icterus
e) psychomotor retardation
d) mild scleral icterus
koilonychia are thin nails that lose their convexity - become spoon shaped.
Which of the following would most likely be found in the bone marrow?
- Neuroblastoma
- Wilms tumour
- Hepatoblastoma
- Neuroblastoma
Most common sites of mets for Neuroblastoma - regional or distant LN, long bones and skull, bone marrow, liver and skin.
Wilms: lungs, LN and liver
Hepatoblastoma: regional LN and lungs
You are seeing a pregnant woman during her first. Her father has haemophilia. Regarding the risk of her transmitting the disorder to her own children you tell her:
a. None will have it
b. 50% of her sons will have it and all of her daughters will be carriers
c. 50% of her sons will have it and 50% of her daughters will be carriers
c. 50% of her sons will have it and 50% of her daughters will be carriers
X-linked recessive inheritance
A patient is being assessed for a bleeding diathesis. Labs show PT 12 s (normal), aPTT 50 s (prolonged), bleeding time 5 minutes (slightly prolonged), platelets 250,000.
a) hemophilia
b) von Willebrand’s disease
c) vitamin K deficiency
d) factor V deficiency
e) factor X deficiency
b) von Willebrand’s disease
or
a) Hemophilia
(depends if you think 5mins is long or not)
- vWD and hemophilia both have normal INR, long PTT, normal platelets, but vWD has normal or long bleeding time and hemophilia has normal bleeding time
- bleeding time is a bad test - should check vWF levels!
- vWF tethers platelets to damaged endothelium, and acts as a carrier protein for factor 8
14 yo girl with leg pain, radiograph shows lesion in the distal femur with ‘sunburst’ appearance. What is the likely cause?
a. Osteoid osteoma
b. Ewing’s sarcoma
c. Osteogenic sarcoma
d. Aneurismal bone cyst
c. Osteogenic sarcoma (AKA osteosarcoma) - most common primary malignant bone tumour in children and teens. Classic sunburst pattern. Often metaphysical region of long bones, esp. knee.
- note: a. Osteoid osteoma (small benign bone tumour- unremitting, gradual increasing pain at night and relieved by aspirin; Tx- surgical removal - XR shows middle white spot surrounded by dense bone)
An 8 year girl comes with the following skin finding [picture of large café-au-lait macule]. List 4 other skin findings in this condition.
NF1 skin findings:
- axillary or inguinal freckling
- neurofibromas
- Lisch nodules
What is the advantage of using leukoreduced white cells?
- Decreases hemolytic reactions
- Decreases infectious complications
- Decreases febrile transfusion reactions
- Decreases febrile transfusion reactions
The other’s aren’t really wrong either.
3 year old immigrated from West Africa. Appears well, has scleral icterus. Otherwise normal exam. Given CBC - normocytic anemia. Hg electrophoresis HgF50% HgC50% HgA 0%
a. What is the disease?
b. What are 2 complications
c. 2 indications for exchange transfusion in a patient with sickle cell?
a. Hemoglobin C disease
b. Hemolysis, Anemia
?stones
c. Acute emergencies: acute chest, stroke, splenic sequestration, stroke prevention
Kid with tumour lysis syndrome, what additional medication should he be on?
Rasburicase
give potassium
Rasburicase
Do NOT give K+
Hydration +++ Diuresis Allopurinol Rasburicase Fix HyperK+ with kayexalate, Insulin+Glucose, Calcium-Gluconate
Infant (<1y) with distended abdomen and diffuse nonspecific skin lesions. Did have an abdominal ultrasound with a supra-renal mass. The urine was positive for HMA/VMA
a) What are your 3 non-renal differential for abdominal mass in a baby?
b) What additional investigations would you do?
c) What stage would you tell the parents?
d) What is the prognosis?
a) Neuroblastoma, hepatoblastoma, Teratoma, Leukemia (hepatomegaly)
b. Abdominal imaging (CT or MRI)
Tissue Bx or BM
MIBG (to assess extent)
CBCd, serum chemistries, liver and kidney function, LDH
c. 4S: Neuroblastoma in Infants <1 year with dissemination to liver, skin and/or bone marrow without bone involvement
d. Favorable, some regress without treatment
Child with hemihypertrophy.
What condition would you suspect that would require serial follow-up?
Beckwith-Wiedemann
Risk of abdominal tumors including Wilms and hepatoblastoma (get screening q3mos until 8) and neuroblastoma
How to treat macrophage activating syndrome?
High dose IV methylprednisone
Cyclosporine
or Anakinra
You are working in an Emergency department and a mother brings in a 2 year old girl with a petechial rash noted that morning while changing her diaper.
She is otherwise well-appearing. You suspect a diagnosis of ITP.
What are 4 indications for bone marrow aspiration in the setting of a probable diagnosis of ITP?
Basically anything that makes you think maybe it’s actually leukaemia, not ITP
History
Constitutional symptoms *
Bone pain *
Recurrent thrombocytopenia
Poor treatment response
Physical
Generalised LAD *
Hepatomegaly *
Splenomegaly *
Unwell appearing child
Signs of chronic disease
Investigations
Anemia * (reminder that a mild anemia is allowable)
High MCV *
Abnormal WBC/neutrophil count *
Abnormal cellular morphology on smear *
Name four lab findings in tumor lysis syndrome
Hyperkalemia
Hyperuricemia
Hyperphosphatemia
Hypocalcemia
Late Hemorrhagic disease. What is most likely cause?
Breastfeeding
CF
Phenytoin
CF
Late onset > 2 weeks (up to 6 mos) is often chronic malabsorption.
What is the number one cause of complications in beta thalassemia major.
a. Iron overload
b. Megaloblastic anemia
c. Cardiomyopathy
d. Poor hematopoeisis
a. Iron overload
You are consulted on the postpartum ward for a neonate with thrombocytopenia. A CBC reveals a platelet count of 12. Following a platelet transfusion, the platelet count is 14. The baby’s mother has a normal CBC and a platelet count of 250. The baby has a normal newborn exam.
What is the most likely diagnosis?
a) Neonatal alloimmune thrombocytopenia
b) Neonatal autoimmune thrombocytopenia
c) Neonatal lupus
d) Sepsis
a) Neonatal alloimmune thrombocytopenia
Which is the most permanent toxicity of vincristine?
peripheral neuropathy
Seizures
Constipation
Peripheral neuropathy
Cyclosporine at start of treatment and high levels 3d into treatment. Pt started on clarithromycin. Reason?
- renal transplant
- clarithro inhibits renal excretion of cyclosporine
- better compliance
- Clarithromycin something else
Clarithromycin is a CYP3A Inhibitor
Other CYP3A Inhibitors: Clairthromycin Erythromycin Itraconazole Ketoconazole Ritonvir Diltiazem Verapamil Grapefruit
CYP3A help metabolize most drugs in the liver
A 10 year old boy with sickle cell anemia has multiple episodes of acute chest crises. Which of the following complications does this place him at increased risk for:
A. Stroke
B. Gall stones
C. Nephropathy
D. Asthma
A. Stroke
Baby born at home at 38 wks by midwife. Now presents at 7 days with melena. Hb 70, MCV 112, plts normal. What is the most likely diagnosis?
Vitamin K deficiency leading to hemorrhagic disease of the newborn
Newborn with platelets of 12, transfused and post-transfusion platelets were 16. Mom’s CBC was normal. What do you do?
a. transfuse single donor platelets
b. transfuse PLA-1 negative platelets
c. give IVIG
d. give steroids
b. transfuse PLA-1 negative platelets (AKA HPA-1)
- Treatment
o IVIG prenatally to mom
o C/S for delivery
o Transfusion
1. washed maternal platelets
2. cross-matched to mom’s serum
3. PLA-1 negative platelets
4. Random platelets (unlikely to improve as 98% of population PLA1 positive)
List 3 common causes of neutropenia .
- viral suppression
- chronic idiopathic neutropenia
- cyclic neutropenia
- drug induced (penicillins)
- leukemia/lymphoma
Older child who is at home for palliative care for her AML. (4)
What are four principles of treatment with opiods that would guide your treatment
WHO: By the mouth (oral most convenient) By the clock ( regular schedule) By the ladder (stepwise approach) Individualized to the child
- dosing needs to be individualized and titrated for the patient
- the right dose is one that relieves pain and minimizes side effects
- side effects should be anticipated and treated
- ensure good basal level of pain control with prn medication available for breakthrough pain
- administer medication via the simplest, most effective and least distressing route
You saw a 15 year old M with respiratory distress and bilateral wheeze in the ER. This was the first episode of wheeze. Resolved with IV methylprednisone and ventolin in ER and he was sent home. One week later the radiologist is reviewing the film and notices a widened mediastinum. What is the most likely cause?
A. Thymoma
B. Hodgkin’s lymphoma
C. ALL
D. Sarcoidosis
B. Hodgkin’s lymphoma
Hodgkin’s lymphoma is the most common malignancy in adolescence, though T-cell ALL may also have mediastinal mass.
Sarcoid more classicaly with bilateral hilar adenopathy
HB high at birth and now still at 3 months? most likely condition…
Down Syndrome
twin- twin transfusion
premature baby
Down Syndrome
Physiologic nadir is 6-8 weeks, if still high at 3 mos I’d think of something more chronic
Erythrocyte transfusion :
Delayed cord clamping, Intrapartum hypoxia (increased placental transfusion), twin-twin transfusion
Increased Intrauterine Erythropoiesis:
Placental insufficiency and chronic intrauterine hypoxia (SGA, Pre-Eclamplsia, Moms with cardiac or pulmonary disorders, smoking, high altitude)
IDM
LGA - includes Beckwith-Wiedemann
Endocrine Problems - CAH, hypo/hyper thyroid
T21, 19, 13
Newborn with T21 and petechiae, HSM. CBC showed elevated WBC, anemia, thrombocytopenia. What is the most likely diagnosis?
a) ALL
b) Fanconi anemia
c) transient myeloproliferative syndrome
d) sepsis
c) transient myeloproliferative syndrome
Approx. 10% of neonates with Down Syndrome develop a transient leukemia or myeloproliferative disorder → high leukocyte counts, blast cells in peripheral blood, associated anemia, thrombocytopenia and HSM
Features usually resolve within first 3 months of life
20-30% who develop TMD will develop leukemia by age 3
Child macrocytic anemia, bifid thumbs. What else to expect on exam:
craniofacial anomalies
absent radii
Craniofacial anomalies
Diamond Blackfan Anemia (DBA)
- craniofacial (hypertelorism, high arch palate, cleft palates, microcephalic, micrognathia, low ears, low hairline)
- eyes (congenital glaucoma, strabismus, congenital cataract)
- neck (short, webbed)
- thumbs (triphalangeal, duplex or bifid, hypoplastic, absent radial artery)
- GU (hypospadias, absent kidney, horseshoe) - Cardiac (Vsd, ASD, coarct)
- growth retardation - learning problem
Hereditary Spherocytosis:
a) What would you use to diagnose this
b) What would be indications for splenectomy
a)
Can Establish with:
- Positive family history
- Lab/PE Findings:
Splenomegaly, spherocytes on blood smear, reticulocytosis, elevated mean corpuscular hemoglobin concentration
If uncertain:
- Flow cytometry EMA (eosin-5-maleimide) binding test
- osmotic fragility
b) Severe HS: frequent hypo plastic or aplastic crisis, poor growth, cardiomegaly.
Teen on OCP swelling of leg in ED develops chest pain/shortness of breath. Management:
- Coumadin
- Heparin
- TPA
- Surgical removal of clot
- Heparin (or LMWH)
Coumadin - longer term after acute episode
TPA: Use with anticoagulants in hemodynamically significant PE
Surgery - limited to persistent hemodynamic compromise
Long term consequence of fanconi anemia?
- pancytopenia - bone marrow failure
- AML
- squamous cell carcinoma of head, neck and esophagus
Boy with fever, wt loss, splenomegaly, fatigue
a) CT abdo
b) CXR
b) CXR
Splenomegaly Initial diagnostic testing
Complete blood count (CBCd) Reticulocyte count Review of the peripheral blood smear Liver function tests EBV and CMV serologies Chest radiograph Abdominal ultrasound
Teenager has lymphadenopathy (including supraclavicular) and fevers, weight loss. What do you do?
a. abdominal ultrasound
b. chest xray
c. CT chest/abdo
d. bone marrow
b. chest xray
Look for mediastinal mass - lymphoma
A child’s newborn screen was positive, with the following pattern: HbA – None, HbF – 70%, HbS – 20%, HbA2 – 10%.
What is your diagnosis:
a) alpha thalsemia
b) sickle cell trait
c) beta thalasemmia
d) sickle cell disease
d) sickle cell disease
Has NO HbA
FAS = sickle cell trait (HbA > HbS)
FSA = Sickle beta thal (HbS >HbA)
Hemoglobin F: alpha2 gamma2
Hemoglobin A : alpha2 beta2
Hemoglobin A2: alpha2 delta2
Hemoglobin S: alpha2 betaS2 (abnormal beta S globin )
In which of the following situations should you investigate a child with sickle cell anemia for possible stroke:
A. Increasing lethargy
B. Recent poor school performance
C. Acute worsening of anemia
B. Recent poor school performance
- silent cerebral infarct common in kids with sickle cell
** increasing lethargy would be a CRITICAL sign though?
A child with thalassemia major on recurrent transfusions and desfuroxime. What is the most serious complication:
a. cardiac hemosiderosis
b. chronic anemia
c. cognitive impairment
d. liver hemosiderosis
a. cardiac hemosiderosis
- 1 year of tfns = liver hemosiderosis
- 2 years of tfns = endo (hypopara, hypogonadotropic hypogonadism)
- 10 years = cardiac - this is what kills you if you don’t have iron chelation therapy
Which of the following is inherited in an autosomal dominant fashion?
1) G6PD
2) Hereditary Spherocytosis
2) Hereditary Spherocytosis
HS: Most common AD, spectrum of asymptomatic to severe hemolytic anemia
G6PD: X-linked. Presents as neonatal jaundice or chronic intermittent jaundice triggered by infection, drugs, fava beans.
Child with microcytic hypochromic anemia. You believe it is Fe deficiency.
What other features of CBC would be consistent with this? List 3.
- increased RDW
- RBC count decreased
- MCH decreased
- WBC count normal
- thrombocytosis
- low retics
Reason why you can’t get therapeutic heparin for oncology kid with clot:
Antithrombin III Deficiency
Factor V Leiden
Protein C Deficiency
Antithrombin III Deficiency
UFH and LMWH act by catalyzing action of antithrombin.
Asparaginase (used in ALL) reduced AT
What treatment is proven to prevent stroke in SCD?
a. transfusion
b. folate
c. hydroxyurea
d. Iron
a. transfusion
Based on monitoring transcranial dopplers
1 mo baby with FTT and diarrhea. WBC 5 x109 : 80% neutrophils 10% lymphocytes 6% basophils 4% eosinophils
a. ) What does this make you think of? (i.e. interpret the labs)
b. ) List 2 causes:
a)
10% Lymphocytes = 0.5 (low)
Definition of lymphopenia varies according to age
<12 mo: lymph <3
> 12 mo: lymph < 1
b) SCID, DiGeorge, protein losing enteropathy, Wiskott-Aldrich, virus (HIV)
The severity of Rh incompatibility at the time of birth is best predicted by:
a. cord Hb
b. cord bilirubin
c. prematurity
d. splenomegaly
e. maternal anti-D antibody
a. cord Hb
Weekly monitoring is performed with the antibody titer and ultrasonography. Ultrasound is performed for MCA peak systolic velocity (MCA-PSV) and for any evidence of fetal hydrop.
Severity related to level of anemia and degree of reduction in serum albumin.
List 4 indications for blood transfusion in sickle cell disease
Treatment of acute complications:
- acute chest syndrome
- aplastic crisis
- stroke
- splenic sequestration (but be careful with this due to auto transfusion)
Prevention:
- prevent stroke in pt with abnormal intracranial dopplers
- prior to surgery to prevent ACS after
18 month old male (13kg) with history of excessive milk intake presents on routine physical exam with pallor Subsequent bloodwork reveals Hb 45, MCV 56. What would be the best management?
1) Limit milk to 500cc/day
2) Start elemental iron 60mg PO tid
3) Give PRBC transfusion 130cc
4) Change to protein hydrosylate formula
a) Limit milk
You’d also treat him but treatment dose would be less than what they’ve written.
Not symptomatic for transfusion (and I’d probably Tx Iron over pRBCs)
A 3 year old girl with fever, arthralgia and lethargy for 10 days has lymphadenopathy,moderate hepatosplenomegaly, no obvious arthritis but screams in pain with minimal examination. WBC 9.5 Hgb 98 Plts 140, smear Normal. Next test:
a) bone marrow aspirate
b) EBV titers
c) follow
a) bone marrow aspirate
Due to bicytopenia and systemic features.
EBV - rarely apparent in kids <4 but in older kids has the classic triad of : fatigue, pharyngitis, generalized lymphadenopathy.
Neonatal hemophilia – 4 clinical presentation in the neonatal period
- factor VIII and IX do not cross the placenta so symptoms can be present in utero or from birth
- extracranial hemorrhage (cephalohematoma, subgaleal)
- intracranial hemorrhage (subarachnoid, smbgaleal)
- prolonged bleeding with circumcision (30%)
- prolonged bleeding from heel poke/venipuncture (newborn screen)
ALL kid just had chemo. He is sexually active. Now 48 h after has hematuria and mild dysuria. Otherwise well. Plts were 90 before chemo. Dx?
A. Cyclophosphamide induced hemorrhagic cystitis
B. Myelosuppression with thrombocytopenia
C. Chlamydia urethritis
D. UTI
A. Cyclophosphamide induced hemorrhagic cystitis
Usually see 24-48h after chemo and lasts 4-5 days.
Prevention: hydration and mesna
Child with hemihypertrophy. Other than Wilms tumors, what other neoplasm do you need to screen for:
a) Neuroblastoma
b) Hepatoblastoma
c) No other neoplasms
b) Hepatoblastoma
- Neuroblastoma screening has not been incorporated into most screening protocols
Beckwith-Wiedemann:
Risk of Wilms tumor, hepatoblastoma, neuroblastoma, rhabdomyosarcoma
A/W macrosomia, macroglossia, hemihypertrophy, omphalocele
12 year old M with recent change in behaviour, irritability, daily headaches and a change in his vision. What is the most important thing to consider?
A. Brain Tumour
B. Drug use
C. Psychiatric Diagnosis
A. Brain Tumour
4 year old of Mediterranean descent. Lab findings most consistent with Thal minor:
a. Hb 100 MCV 60 RBC 4.8
b. Hb 100 MCV 75 RBC 4.3
c. Hb 80 MCV 75 RBC 3.3
d. Hb 80 MCV 60 RBC 3.3
e. Hb 60 NCV 50 RBC 2.2
ANSWER: a. Hb 100 MCV 60 RBC 4.8 (Mentzer= 12.5)
Alpha or beta thal= Should see: microcytic anemia with high RBC count and low or normal retic
- Mentzer Index: MCV/ RBC count in millions
o > 13= iron deficiency more common; <13 beta thal more likely
Labs in keeping with hemophilia? and effect of mixing studies?
Prolonged PTT - 2-3 x ULN
- when you mix normal plasma with patient plasma the PTT corrects
- platelets and INR are normal (in the lab, INR measures activation of factor X directly by factor VII, BUT in real life factor VII activates factor IX which activates factor X so there is a real life deficiency, but it’s not picked up in the lab)
- specific assays for factor VIII and Ix show deficiency
List 3 treatments for hyperuricemia
- allopurinol (blocks enzyme in pathway that makes uric acid)
- rasburicase (makes uric acid more water soluble and easily excreted)
- dialysis
- IVF
- Treat electrolyte disturbance (will also have hyper K in tumor lysis)
(Alt Q: tumor lysis bloodwork, what do you give?)
