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BLISS > Pediatric Blood Disorders > Flashcards

Pediatric Blood Disorders Flashcards

1
Q

ITP in children

A

Most common causes of thrombocytopenia in children (1/20,000)
- usually around ages 1-4 and precedes a viral infection

Usually acute but can be chronic

*most common viruses associated with it are Epstein-Barr Virus (EBV) and HIV

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2
Q

ITP pathogenesis in children

A

Target from antibodies against platelets remains undetermined.

Antibodies bound to platelets are recognized by the Fc receptor on splenic macrophages where they are destroyed

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3
Q

ITP clinical manifestations

A

Sudden onset of generalized petechiae and purpura
“Yesterday she was fine and now she is covered in purple dots/bruises”

  • can show bleeding from gums, mucous membranes and profound thrombocytopenia
    (< 10 x 10^9/L)
  • almost always says was sick 1-4 weeks ago with viral infection
  • physical examination is normal outside of petechiae/ purpura
  • rarely will show splenomegaly, lymphadenopathy, be one pain and pallor
    ( suggests possible leukemia as well)
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4
Q

Classification of ITP

A

1: no symptoms
2: mild symptoms: bruising/petechiae and minor epistaxia
3: moderate symptoms: severe skin/mucosal lesions, menorrhagia
4: severe symptoms: extreme bleeding episodes

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5
Q

Treatment of ITP

A

No cure

Mild treatment:
Just monitor and education, no drugs

Moderate-severe:
Treated with (0.8-1g/kg/day IVIG or prednisone 1st line of treatment
- monitor for 48 hrs
- once >20 x 10^9/L stop treatment

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6
Q

Hereditary spherocytosis in children

A

1/ 2000-5000 (most common in Northern European origin)

Defect in RBC skeletal proteins (usually an Anakryin or spectrin)
- RBCs are spherical shaped and burst easily/ cant fit through splenic cords/sinuses and are destroyed early by spleen macrophages

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7
Q

Clinical signs of hereditary spherocytosis

A

Anemia and jaundice

Splenomegaly

Recurrently hemolytic crises and gallstones in severe cases

  • very dangerous if patient was recently affected with parvovirus B19 infection*
  • results often in hematocrit <10% which is super deadly and can result in CV collapse

Possible other dangerous infections are HIV and hep B or C

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8
Q

Diagnosis of hereditary spherocytosis

A

Clinical symptoms hint at i t but the easiest way to diagnosis is a blood smear

  • will show 750% increase of spherocytes and a low normal- low decrease of overall MCV.
  • also show increased MCHC (>35g/dL)
  • the gold standard with hereditary spherocytosis is the osmotic fragility test (will show cells bursting with slight hypotonic solutions)
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9
Q

Treatment of hereditary spherocytosis

A

Only “cure” or correction is splenectomy

Is also 75% a autosomal dominant trait with 25% arising de novo

  • should monitor for hyperbilirubinemia in children that have parents with known hyper spherocytosis (would likely require transfusions and phototherapy)
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10
Q

G6PD in children

A

One of the PPP enzymes are defective, resulting in the inability to reduce Glutathione. This results in a increase chance of damage to RBCs via oxidative stress

  • x-linked disease (more common in males)
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11
Q

G6PD clinical symptoms

A

Severe neonatal jaundice in the eyes out of nowhere cardinal sign

episodic acute hemolytic anemia (usually caused by drugs and rarely favs beans)

dark urine and/or hemoglobinuria

Back/abdominal pain

Hemolytic anemia

  • often times will appear asymptomatic until triggered via some episode of oxidative stress*
  • will show symptoms within 24-48 hrs after this event
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12
Q

G6PD diagnosis

A

Blood smear will show Bite cells and Heinz bodies golden standard

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13
Q

Drugs that cause oxidative stress to G6PD patients

A

Asprin (causes Reyes syndrome in children so shouldn’t be given anyways)

Sulfonamides (Sulfadrugs)

Rasburicase

Antimalarials (primaquine)

Fava beans (due to concentrations of divicine , isouramil, convicine)

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14
Q

G6PD treatment

A

Supportive therapy and blood transfusions

  • No cure*
  • note that males of Central African or Mediterranean descent should always be tested for this when born*
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15
Q

Diamond Blackfan Anemia (congenital hypoplastic anemia)

A

Rare, congenital bone marrow failure

Autosomal dominant disease that is usually diagnosed with 1st year of life

Can make them more susceptible to cancer in the future

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16
Q

Diamond-blackfan anemia clinical signs and diagnosis

A

50% have the following physical abnormalities

  • short stature (<10% percentile)
  • cleft palate and microcephaly (Most common 50%)
  • cardiac/urogential abnormalities
  • thenar eminance flattening
  • super long thumb(looks like a normal finger)

super pale and blue eyes
* no or very poor radial pulse is a key sign*

Lab results Presents with macrocytic and normochromic RBCs, very reticulocytopenia and a very low MCV

17
Q

DIamond- blackfan anemia treatment

A

Corticosteroids are the 1st line treatment
- however watch dose since corticosteroids are known to impair growth and neurocognative development. May chose to wait until 1 yr to start therapy because of this

  • Hematopoietic Stem Cell transplantation is curative in some cases*
  • best route is from sibling donors under age 9yrs
18
Q

Fanconi anemia (FA)

A

Rare multisystem hereditary disorder that results in bone marrow failure

High chance of getting cancer and patients are highly sensitivity to alkylation get agents and radiation

Highrate of carrier (1/200) but very rare expression (1/200000)

19
Q

Fanconi anemia symptoms

A
  • produces a diagnostic triad*
  • bone marrow failure
  • congenital skeletal abnormalities
  • elevated chromosome fragility (no teleomere)
Hearing loss
Absent thumbs 
Microcephaly and microphthalmia 
Endocrine issues 
Hyperpigmentation
20
Q

Treatment for Fanconi anemia

A

Bone marrow transplantation is curative

21
Q

Di-George syndrome

A

22q11.2 micro deletion syndrome

  • classic triad is conotruncal cardinal abnormalities, hypoplastic thymus and hypocalcemia*
  • also almost always produces hypoparathyroidism and hypoplastic thymus as well
22
Q

Di-George syndrome symptoms

A

Mild Skull deformities (cleft palate, prominent nose, narrow bases and ridges, retuned mandible, weak chin)
- 40-50% present with microcephaly

Hypocalcemia tetany/seizures is the most cardinal clinical sign

Signs of congenital heart disease
- ventricular septal defects and right aortic arch are the most common heart symptoms

Short stature

Hearing loss

Decreases motor tone and instability

Learning issues

23
Q

Kawasaki disease (mucocutaneous lymph node syndrome)

A
  • most common cause of acquired heart disease in children*

Idiopathic origin but hypothesized to be caused by bacterial/viral pathogens that turn on the defect

Ultimately will lead to vasculitis of coronary arteries and can cause MIs or aneurisms in coronary arteries
- ALWAYS PRESENTS WITH A RASH

90% of patients are less than 5 years old
- more common in males (1.5:1 ratio)

24
Q

Possible causative agents for Kawasaki disease

A

Viral: measles, adenovirus, EBV

Bacteria: S. Aureus, step pyogenes, rickettsia, Lepto interrogans

Drug reactions: serum sickness and Stevens-Johnson syndrome

Rheumatologist disease: arthritis

Heavy metal toxicity

25
Q

Kawasaki disease diagnosis

A

Fever for at least 5 days and 4/5 of the following

  • polymorphous exanthem (red rash that can be variable in origin and amount)
  • (conjunctival injection) pink eye without exudate
  • strawberry tongue & lips cracking
  • erythema, edema and induration of hands and feet
  • cervical lymphadenopathy (swollen lymph nodes)
  • if they have this it is Kawasaki disease until disproven*
  • also make sure to rule out strep throat*
26
Q

Kawasaki disease laboratory findings

A

Normocytic/normochromic anemia

High inflammatory markers (CRP and ESR)

Low albumin levels

High serum transaminases

High bilirubin in blood

Band neutrophils in blood smears

  • if echocardiogram shows coronary artery dysfunction, only need 3/5 of the cardinal symptoms*
27
Q

Kawasaki disease treatment

A
  • IVIG is the first line treatment*
  • 2g/kg for 1-2 doses

Follow IVIG with asprin for 1-2 months
- 80-100 mg/kg.day until fever is gone for 48 hrs, then stop treatment.

Best prognosis is treatment within 1st 10 days of the fever

28
Q

Henoch- Schonlein purpura (HSP)

A

Idiopathic syndrome where there is large amounts of IgA1 complexes with C3 deposition caused by an environmental trigger
- triggers wide spread inflammatory reactions

Most common in >10 years , with peak at 6 yrs
- 1.5:1 male to female ratio

Possible triggers:

  • Infections: URI, strep and staph most common
  • medications
  • insect bites
  • cold weather
  • trauma
29
Q

Henoch- Schonlein purpura (HSP) symptoms

A

Rash is usually the first clinical manifestation

  • purpura spots
  • glomerulonephritis
  • low grade fever
  • angioedmia in extremities
  • GI symptoms

classic triad that follows the rash is purpura, arthralgia and abdominal pain

30
Q

Possible genetics for HSP

A

HLA-DRB1 is the assumed target gene

Also hypothesized to be affected by HLA-A2, All and B35

  • HLA-A B49 and B50 are suspected to DECREASE likelihood of getting it*
31
Q

Laboratory findings of HSP

A

CBC: low platlet count

Urinalysis: High albumin and creatinine Levels. Also kidney enzymes

32
Q

Intussusception with HSP

A

Possible bowel invaginiation into another part of the bowel “telescope”
- this leads to obstruction

Possible symptoms

  • obstructs lumen
  • abdominal pain and vomiting
  • ischemia of intestine
  • most common site is ileocecal
33
Q

Treatment of HSP

A

Supportive care, avoid exercise

  • 1st line of treatment is NSAIDs (usually acetaminophen and Naproxen)
  • extreme situations require ACE inhibitors or angiotensin blockers

Has a high chance of recurrent disease so need to follow up often especially within 4 months of diagnosis

BLISS (42 decks)

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