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Pediatrics > Hematology > Flashcards

Hematology Flashcards

1
Q

What is the genetic basis behind Sickle Cell Disease?

A

Mutation of a valine to a glutamine resulting in an abnormal beta globin chain.

Sickle Cell Trait - HbSHb
Sickle Cell Anemia - HbSS
Sickle Cell Disease - HbSS or Sickle Hgb C or Sickle B thalessemia

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2
Q

What features would make you most suspicious for langerhans cell histiocytosis?

A
  • bony lucencies
  • exophthalmos
  • diabetes insipidus
  • difficult to treat, scaly, seborrheic dermatitis of the scalp, diaper, axillary or post-auricular regions

may also see chronically draining ears, HSM, growth retardation, weight loss, fever or FTT

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3
Q

How is a diagnosis of LCH confirmed?

A

Biopsy of affected area

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4
Q

How is LCH treated?

A
  • Curettage and bone graft
  • Low dose radiation therapy
  • Corticosteroid injection
  • Systemic multiagent chemotherapy (i.e vinblastine/etoposide)
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5
Q

What are the CPS suggested guidelines for treatment of anemia of prematurity?

A

If on respiratory support:
115, 100, 85 (week 1, week 2, week 3 postnatal)

If not on respiratory support:
100, 85, 75

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6
Q

In children who have had a splenectomy, what are the prophylaxis guidelines as per CPS?

A
  • birth to 5 years of age: 125 mg BID penicillin VK, or 10 mg/kg/dose BID amoxicillin
  • > 5 yoa: 250-300 mg BID penicillin V, or 250 mg BID amoxicillin
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7
Q

What additional vaccines should be considered in a child with sickle cell anemia?

A
  • Hib - 5th dose
  • Polysaccharide pneumococcal-23 - 2 doses
  • Annual influenza
  • Quadrivalent meningococcal vaccine q 5 years
  • Men-B x 2 doses
  • Salmonella typhi if travelling to high risk area
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8
Q

How often should children with sickle cell anemia receive transcranial dopplers?

A

• Yearly between the ages of 2 and 16

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9
Q

What annual screening should be done for children with sickle cell anemia?

A
  • Yearly retinopathy screening starting at age 10
  • Yearly screening for asthma
  • Yearly screening for nephropathy starting at age 10 (UA and urine albumin: creatinine ratio)
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10
Q

What transfusion goals should be set for children with sickle cell anemia?

A

• Should try and maintain Hgb S <30%, total hgb no greater than 125

Remember to also:
• Test for alloantibodies + RBC phenotyping
• If chronic transfusion: annual infection screen (HepB, HepC, HIV)
• Iron overload monitoring if >100 mg/kg of RBC (ferritin)

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11
Q

What organisms are children with SCD at greater risk of falling ill from?

A

Encapsulated bacteria

“Some Nasty Killers Have Some Capsule Protection”

Strep pneumoniae, Neisseria meningitidis, Klebsiella pneumoniae, Haemophilus influenzae, Salmonella typhi, Cryptococcus neoformans, Pseudomonas aeruginosa

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12
Q

What is your management of acute chest crisis?

A
  • Pain control: opioids, Tylenol, NSAIDs
  • Oxygen
  • Bloodwork to assess hematocrit, group and type, and sickling load in preparation for possible transfusion (5 mL/kg)
  • Hydration - to prevent hyperviscosity (10 mL/kg NS bolus)
  • Blood culture and antibiotics (CTX and vanco)
  • Incentive spirometry and SABA as required
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13
Q

What are the 3 most common respiratory organisms in cases of acute chest crisis in SCD?

A
  • streptococcus pneumonia
  • mycoplasma
  • chlamydia
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14
Q

How to determine of a child with SCD has aplastic anemia or splenic sequestration?

A
  • presence or absence of large spleen
  • retics will be low in aplastic anemia, high in splenic sequestration
  • bilirubin will be low in aplastic crisis, high in splenic sequestration
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15
Q

What neurological complications are possible in the setting of sickle cell disease?

A
  • Headaches
  • Cerebral sinovenous thrombosis (CSVT)
  • Posterior reversible leukoencephalopathy syndrome (PRESS)
  • Cognitive Impairment - deficits in intelligence, attention, executive function, memory, language and visual-motor
  • Academic failure, decreased QOL (silent strokes)
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16
Q

What does the CPS suggest with respect to treatment of ITP in children?

A
  • For children without active bleeding
    • Observation as the 1st-line, with PO corticosteroids or IVIG as 2nd-line options
    • Consider the individual’s bleeding risk (e.g., activity level), logistics (e.g., household distance from hospital) and social issues (e.g., reliability of follow-up)
  • For children with moderate bleeding
    • IVIG (0.8 g/kg to 1.0 g/kg) OR corticosteroids
    • IV anti-D Ig only in Rh+ children (NOT first-line)
  • For children with severe bleeding(e.g., prolonged epistaxis, GI bleeding or ICH)
    • Admit, IV steroids and IVIG is indicated
    • Tranexamic acid (25 mg/kg/dose 3 to 4 times per day (to a maximum of 1500 mg per dose or 4500 to 6000 mg per day)
    • Plt tx only for acute, life-threatening bleeds or in children requiring immediate surgery
17
Q

What is the genetic basis behind thalassemia alpha and thalassemia beta?

A
  • Thalassemia alpha: genetic changes that affect the alpha chain (Chromosome 16)
    • silent trait - single mutation
    • cis/trans trait - alpha thal minor
    • 3 mutations - alpha thal major
    • 4 mutations - hemoglobin barts (hydrops)
  • Thalassemia beta: genetic changes that affect the beta chain (Chromosome 11)
    • single mutation B+ or B0 - betal thal minor
    • B+/B0, B+/B+ - beta thal intermedia
    • B0/B0 - beta thal major/Cooley’s anemia (transfusion dependent)
18
Q

What clinical findings are expected in cases of beta thalassemia major?

A
  • expansive extramedullary hematopoesis - chipmunk cheeks, hair-like projections on x-ray of skull, frontal bossing
  • HSM
  • depressed nasal bridge
  • hypothyroidism
  • FTT
  • diarrhea
  • pallor/jaundice
19
Q

What treatment options are available for beta-thalessemia major?

A
  • blood transfusion
  • iron chelation
  • splenectomy
  • folate, iron and B12 supplementation
  • hydroxyurea
20
Q

What are the potential complications of beta-thalessemia major?

A
  • Iron overload (#1 complication) - 2⁰ to hemolysis and repeat transfusions; multi system effects
  • Bone marrow expansion :. May get spinal cord compression, osteoporosis/penia, fractures, AVN
  • Chronic hepatitis, Hemochromocytosis
  • Neurocognitive & learning delays
  • Growth failure
21
Q

What are the measures of severity for hemophilia?

A
  • Severe <1%
  • Moderate 1-5%
  • Mild 5-25%
22
Q

Which children with hemophilia require lifelong prophylaxis?

A
  • Severe forms

* Those who have had their first joint bleed

23
Q

What are the treatment options for hemophilia?

A

• Severe Hemophilia - treat with prophylactic rVIII concentrates, then PRN if bleeds
• Moderate Hemophilia - treat PRN with prophylactic rVIII concentrates
• Mild Hemophilia - DDAVP
- stimulates release of VW protein from endothelial cells and platelets
- VW carries factor VIII so you can boost their levels quite easily with DDAVP for bleeding episodes.
• Antifibrinolytic therapy (TXA and epsilon aminocaproic acid (EACA)) can be used with factor therapy to treat those with a mucosal source of bleeding

24
Q

What are inhibitors and how do you manage their presence?

A

• Inhibitors = antibodies directed against factor VIII or factor IX that block the clotting activity
- Failure of a bleeding episode to respond to appropriate replacement therapy is usually the first sign of an inhibitor.
• Many patients who have an inhibitor lose it with continued regular infusions
• May also treat with immune tolerance induction (= regular (daily or several times weekly) infusions of variable doses of factor VIII or IX administered for a period of weeks to years in an effort to tolerize the immune system to factor VIII or IX)

25
Q

Can immunizations be given to patients with hemophilia?

A

Yes, using a very small gauge needle (at least 23) and applying firm pressure afterwards for at least 5-10 mins

26
Q

What are the classic findings in cases of hemolytic uremic syndrome?

A
  • Thrombocytopenia
  • Renal dysfunction
  • Microangiopathic hemolytic anemia
27
Q

What are potential causes of HUS?

A
  • Infectious: i.e. E coli O157:H7 or neuraminidase streptococcus
  • Genetic: ADAMTS13, complement factor H, I , B abnormality
  • Medication: clopidogrel, calcineurin inhibitors
28
Q

Treatment for HUS?

A
  • Transfusion with washed RBCs (IgM if not washed may worsen course)
  • DO NOT transfuse platelets
  • Support re: renal dysfunction - diuresis, dialysis
  • Management of hypertension
  • Eculizumab
29
Q

What are the different forms of von Willebrand Disease?

A
  • Type 1 - mild, AD, prolonged mucocutaneous bleeding
  • Type 3 - severe, AR, significant mucocutaneous bleeding that can mimic hemophilia
  • Type 2 - difficult to diagnose
30
Q

What is the best test for vWD?

A
  • Ristocetin cofactor assay
  • vWF antigen
  • PT/PTT
  • factor VIII activity
31
Q

What is the most commonly inherited disorder of the RBC membrane?

A

Hereditary Spherocytosis (>Northern Europeans, but present in most populations)

32
Q

What feature on CBC is unique to hereditary spherocytosis?

A

Markedly high MCHC (>350)

33
Q

Which blood factors are not blood products, but recombinant products?

A

Factor VIII and Factor IX

34
Q

What infectious agents does Canadian Blood Services screen for?

A

HIV, HTLV 1/2, Hep B/C, WNV, Bacterial infections by culture, CMV, Syphilis

35
Q

What are the approximate risks of contracting HIV, Hep C and Hep B from donated blood products?

A
  • HIV: 1 in 10 million
  • Hep C: 1 in 5 million
  • Hep B: 1 in 1 million
36
Q

What are the characteristic features of Schwachman-Diamond Syndrome?

A

“Patrick Swayze takes a NAPP”

  • Neutropenia
  • Anemia
  • Pancreatic insufficiency - improves with age
  • Poor growth (FTT, recurrent infections)
37
Q

What are the features of TARS?

A
  • Thrombocytopenia
  • Absent radius
  • Eosinophilia
  • Congenital heart disease i.e. ToF, ASD, VSD
38
Q

What are the features of Diamond-Blackfan?

A
  • Normochromic MACROcytic anemia, presenting in infancy
  • Congenital abnormalities: -fan -> tri-/biphlangic thumbs, snub nose, hyperteloric, high-arched palate
  • Neutropenia
  • Thrombocytopenia
39
Q

What is the main concern in patients with G6PD?

A
  • Genetic abnormality that results in deficient Glucose-6-phosphate dehydrogenase placing the RBCs at greater risk of oxidative stress
  • Results in breakdown of RBCs in times of stress, or exposure to free-radical producing medications or foods i.e. favism

Pediatrics (27 decks)

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